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BACKGROUND: Patients with Cushing's disease (CD) with hypercortisolism have an increased risk of opportunistic infection. However, most CD patients exposed to infections are diagnostic latency, leading to a poor prognosis. METHODS: Six patients in our hospital and an additional six patients in the literature were included in this study. Clinical information of CD patients with pulmonary Cryptococcus neoformans are reviewed. RESULTS: The average baseline total cortisol and ACTH in serum at 8 am of all the patients was 44.85 µg/dL (normal range 4.0-22.3 µg/dL) and 200.3 pg/mL (normal range 0-46 pg/mL), respectively. Lymphopenia was found in 2 out of 6 patients in our hospital. The pulmonary radiologic findings included nodules (4/12), masses with or without a cavity (5/12), infiltration (5/12), and consolidation (4/12). The diagnosis of C.neoformans was established by lung pathology results (7/12), microorganism culture (3/12), and serum cryptococcal polysaccharide antigen (4/12). Lung lobectomy was performed in two patients who had a nodule in one lung lobe. Antifungal drugs were administered, including amphotericin-B (7/12), fluconazole (4/12), flucytosine (2/12) and liposomal amphotericin (1/12). Additional therapies for CD included trans-sphenoidal pituitary adenoma surgery (9/12), adrenalectomy (1/12) and ketoconazole (2/12). Seven patients survived, and five patients died. CONCLUSION: Pulmonary C.neoformans is an uncommon but fatal opportunistic infection in CD patients. Pulmonary nodules or masses should be aggressively investigated to exclude the C.neoformans among CD patients. The infiltration lesions in chest CT scan and lymphopenia are associated with poor prognosis.
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Antifúngicos/uso terapéutico , Criptococosis/complicaciones , Criptococosis/tratamiento farmacológico , Hipersecreción de la Hormona Adrenocorticotrópica Pituitaria (HACT)/complicaciones , Adulto , Beijing , Criptococosis/mortalidad , Cryptococcus neoformans/aislamiento & purificación , Femenino , Humanos , Pulmón/patología , Masculino , Persona de Mediana Edad , Infecciones Oportunistas/microbiología , Infecciones Oportunistas/mortalidad , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: To study the serum lipid panels in consecutive autoimmune pulmonary alveolar proteinosis(APAP)patients and analyze their relationship with anti-granulocyte macrophage-colony stimulating factor(GM-CSF)antibody and other markers. METHODS: Thirty-two non-diabetic APAP patients were enrolled in the study. Serum lipids of these patients and 100 healthy volunteers were tested after an overnight fasting. Anti-GM-CSF antibody levels were measured with enzyme-linked immunosorbent assay. The correlation of serum lipids with lactate dehydrogenase,carcinoembryonic antigen,pulmonary function,and artery blood gas parameters were analyzed. RESULTS: Total cholesterol and low-density lipoprotein cholesterol levels [(5.54±0.99)and(3.73±0.83)mmol/L respectively] were significantly higher in APAP patients than in healthy volunteers [(5.05±0.97)and(3.17±0.89)mmol/L respectively](all P<0.05). High-density lipoprotein cholesterol(HDL-C)level of the APAP group [(1.10±0.18)mmol/L ]was significantly lower than that of the healthy group(P<0.05). Low-density lipoprotein/HDL and total cholesterol/HDL ratios in the APAP group(3.47±0.90 and 5.14±1.12 respectively)were significantly higher than those in the healthy group[(2.63±0.87)and(4.18±1.12)](all P<0.05). There was no significant difference in triglyceride level between the two groups(P>0.05). HDL-C level was negatively correlated with alveolar-arterial oxygen pressure difference(r=-0.436,P<0.05)and positively correlated with arterial oxygen saturation(r=0.459,P<0.05). None of the lipid markers correlated with serum anti-GM-CSF antibody levels(all P>0.05). CONCLUSIONS: APAP patients were likely to suffer from disturbed lipid metabolism,which was correlated with disease severity to some degree. Lipid markers deserved more attention in the management of APAP patients.
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Enfermedades Autoinmunes/metabolismo , Metabolismo de los Lípidos , Proteinosis Alveolar Pulmonar/metabolismo , Anticuerpos/sangre , Enfermedades Autoinmunes/epidemiología , Biomarcadores/sangre , Colesterol/sangre , Ensayo de Inmunoadsorción Enzimática , Factor Estimulante de Colonias de Granulocitos y Macrófagos/metabolismo , Humanos , Lípidos/sangre , Lipoproteínas LDL/sangre , Pulmón/fisiopatología , Proteinosis Alveolar Pulmonar/epidemiologíaRESUMEN
BACKGROUND: Sirolimus is increasingly utilized in treating diseases associated with mTOR pathway overactivation. Despite its potential, the lack of evidence regarding its long-term safety across all age groups, particularly in pediatric patients, has limited its further application. This study aims to assess the long-term safety of sirolimus, with a specific focus on its impact on growth patterns in pediatric patients. METHODS: This pooled analysis inlcudes two prospective cohort studies spanning 10 years, including 1,738 participants (aged 5 days to 69 years) diagnosed with tuberous sclerosis and/or lymphangioleiomyomatosis. All participants were mTOR inhibitor-naive and received 1 mg/m²/day of sirolimus, with dose adjustments during a two-week titration period to maintain trough blood concentrations between 5 and 10 ng/ml (maximum dose 2 mg). Indicators of physical growth, hematopoietic, liver, renal function, and blood lipid levels were all primary outcomes and were analyzed. The adverse events and related management were also recorded. RESULTS: Sirolimus administration did not lead to deviations from normal growth ranges, but higher doses exhibited a positive association with Z-scores exceeding 2 SD in height, weight, and BMI. Transient elevations in red blood cell and white blood cell counts, along with hyperlipidemia, were primarily observed within the first year of treatment. Other measured parameters remained largely unchanged, displaying only weak correlations with drug use. Stomatitis is the most common adverse event (920/1738, 52.9%). In adult females, menstrual disorders were observed in 48.5% (112/217). CONCLUSIONS: Sirolimus's long-term administration is not associated with adverse effects on children's physical growth pattern, nor significant alterations in hematopoietic, liver, renal function, or lipid levels. A potential dose-dependent influence on growth merits further exploration. TRIAL REGISTRATION: Pediatric patients: Chinese clinical trial registry, No. ChiCTR-OOB-15,006,535. Adult patients: ClinicalTrials, No. NCT03193892.
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Sirolimus , Humanos , Sirolimus/efectos adversos , Sirolimus/uso terapéutico , Niño , Femenino , Adolescente , Preescolar , Adulto , Masculino , Lactante , Adulto Joven , Persona de Mediana Edad , Recién Nacido , Anciano , Esclerosis Tuberosa/tratamiento farmacológico , Linfangioleiomiomatosis/tratamiento farmacológico , Estudios ProspectivosRESUMEN
OBJECTIVE: To improve understanding of the clinical characteristics and diagnosis of hypersensitivity pneumonitis (HP). METHODS: We retrospectively analyzed the clinical data, including clinical symptoms, laboratory tests, exposure, pulmonary function tests, chest CT imaging and cytological classification of bronchoalveolar lavage (BAL) of 96 patients with HP from Jan 2001 to Jun 2011 in Peking Union Medical College Hospital. We divided the patients into 2 groups: a pathologically-confirmed group and a clinically-suspected group. RESULTS: There were 58 females and 41 males. The median age at the diagnosis was 53 years. The most common exposures were low-molecular-weight chemicals (42.7%) and animal proteins (37.5%). Common clinical symptoms included dyspnea on exertion (90.6%) and cough (76.0%). Pulmonary function test showed diffusion abnormality (73.5%) and restrictive ventilatory impairment (59.7%). Chest CT scan revealed patchy or diffuse bilateral ground-glass opacities (64.6%), centrilobular nodules (21.9%), and air trapping (15.6%). Reticulation (45.8%), traction bronchiectasis (21.9%) and honeycombing(9.4%) were present in chronic HP. BAL lymphocyte counts > 0.2 and CD4/CD8 < 0.9 were more commonly seen in patients with a disease course of less than 1 year. The pathologically-confirmed group and the clinically-suspected group shared many similar characteristics including age at diagnosis, gender, clinical manifestation, pulmonary function impairments and imaging findings, but significant differences existed in certain parameters. In the pathologically- confirmed group, the duration of disease was longer (24 months vs 6 months, Z = -2.492, P = 0.013) and clubbed fingers were more common (23.4% vs 8.2%, χ(2) = 4.227, P = 0.040). Diffusion abnormality was present in more patients of this group (90.7% vs 44.0%, χ(2) = 35.219, P < 0.01). By CT scan, reticulation, traction bronchiectasis and honeycombing (57.5% vs 26.5%, χ(2) = 9.434, P < 0.01) were more evident as compared to the clinically-suspected group. The value of transbronchial lung biopsy for diagnosing HP was limited, with a positive result of only 8.2%. Surgical lung biopsy was needed in uncertain cases. CONCLUSION: The diagnosis of HP was difficult. In some cases a clinical diagnosis can be made by combination of history of exposure, CT manifestations and cell classification of BAL. For atypical cases a multi-disciplinary approach including pathologists, radiologists and pulmonologists is needed.
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Alveolitis Alérgica Extrínseca/diagnóstico , Adolescente , Adulto , Anciano , Alveolitis Alérgica Extrínseca/patología , Líquido del Lavado Bronquioalveolar/citología , Femenino , Humanos , Pulmón/patología , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria , Estudios Retrospectivos , Adulto JovenRESUMEN
Background: The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient. Methods: We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months. Results: The HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO2) than extent scores. The mild patients accounted for 61.9% based on DSS 1-2, 20.3% based on SPSP 1-3, and 20.8% based on SPSPII 1-3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO2 differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO2 (P[A-a]O2), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months. Conclusion: The HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO2, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis. Trial registration: ClinicalTrial.gov, identifier: NCT04516577.
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OBJECTIVES: To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP. METHODS: Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin. Sections were cut for HE and immunohistochemical stain. According to the diagnostic criteria for IgG4-related disease, 4 cases were confirmed to be IgG4-related NSIP. The clinicopathological features including clinical history, laboratory examination, and pathologic evaluation were studied. RESULTS: The 4 patients with IgG4-related NSIP included 1 man and 3 women, with a median age of 48 years (range, 44 - 56 years). The presenting symptoms were dry cough or shortness of breath. One patient (1/4, 25.0%) was found to have a positive autoantibody but no cases showed positive RF in serum. The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis, and 1 case showed obliterative arteritis. The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf, 22/hpf, 11/hpf, and 33/hpf respectively, while the percentages of IgG4-positive to IgG-positive plasma cells were 70%, 71%, 57%, 43% respectively. CONCLUSIONS: IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern. The pathological features of IgG4-related NSIP include infiltration of lympho-plasmacytes and eosinophils in interstitium with fibrosis, and lymphoid follicles are frequently identified in the area of lymphocyte aggregation, but obliterative arteritis is infrequently identified in the lesion. Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.
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Inmunoglobulina G/sangre , Enfermedades Pulmonares Intersticiales/patología , Adulto , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/clasificación , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
OBJECTIVE: This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD). METHODS: A total of 481 patients admitted in Peking Union Medical College Hospital from January 2000 to December 2008 underwent CT-guided percutaneous cutting needle lung biopsy. The patients were evaluated by clinical history, physical examination and lung HRCT. Those with localized opacity or lesions in a single lung in the CT scan were excluded. Finally, 248 patients with DPLD in HRCT were enrolled for this study. RESULTS: The study patients included 114 males and 134 females, and the mean (± SD) age at diagnosis was 50 ± 16 (range from 13 - 78) years. Confirmed diagnosis by percutaneous needle lung biopsy was obtained in 130 patients (52.4%), including pulmonary infection (35.4%, 46/130), pulmonary malignant diseases (25.4%, 33/130), bronchiolitis obliterans organizing pneumonia/organizing pneumonia (22.3%, 29/130), pulmonary vasculitis (6.2%, 8/130), granulomatous lesions (4.6%, 6/130), pulmonary sarcoidosis (2.3%, 3/130), acute interstitial pneumonia (1.5%, 2/130), pulmonary amyloidosis (1.5%, 2/130), and pulmonary alveolar proteinosis (0.8%, 1/130). Open lung biopsy/video-assisted thoracoscopic surgery was performed in 37 out of 118 cases for which the diagnosis was undetermined by percutaneous lung biopsy. Confirmed diagnosis was obtained in 36 patients, including non-specific interstitial pneumonia (NSIP, 33.3%, 12/36), usual interstitial pneumonia (UIP, 8.3%, 3/36), pulmonary infection (16.7%, 6/36), neoplasm (8.3%, 3/36), lymphoid interstitial pneumonia, pulmonary vasculitis (5.6% 2/36), hypersensitivity pneumonitis (5.6%, 2/36), and pulmonary sarcoidosis, allergic bronchopulmonary aspergillosis, pulmonary hyalinizing granuloma, pneumoconiosis, Castleman's disease, and lymphoproliferative disorder (1 case respectively). CONCLUSION: CT-guided percutaneous cutting needle lung biopsy can provide confirmed diagnosis in half of patients with DPLD, and has a high diagnostic yield in patients with infectious or neoplastic diseases, but it is not a good method for diagnosis of interstitial lung diseases such as NSIP and UIP.
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Biopsia con Aguja/métodos , Pulmón/patología , Fibrosis Pulmonar/patología , Adolescente , Adulto , Anciano , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/patología , Masculino , Persona de Mediana Edad , Fibrosis Pulmonar/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
CASE PRESENTATION: A previously healthy 47-year-old nonsmoking woman was admitted to our hospital with an 8-month history of progressive exertional dyspnea and fatigue. Chest high-resolution CT (HRCT) on admission showed diffuse, bilateral, patchy ground-glass opacity (GGO) (Fig 1A). She was diagnosed with interstitial lung disease, and corticosteroid therapy with 8 weeks prednisone taper was completed, with initial good response. Eight months later, she was readmitted because of worsening of the dyspnea, with no fever, wheeze, dry cough, chest pain, weight loss, or hemoptysis. She denied a history of hair loss, skin rash, oral ulcers, or arthralgia. She denied a history of allergy or taking other drugs. She had no occupational or environmental exposures. There was no family history of respiratory diseases or hematologic diseases.
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Crioglobulinemia/diagnóstico por imagen , Enfermedades Pulmonares/diagnóstico por imagen , Crioglobulinemia/tratamiento farmacológico , Diagnóstico Diferencial , Quimioterapia Combinada , Disnea , Fatiga , Femenino , Humanos , Enfermedades Pulmonares/tratamiento farmacológico , Persona de Mediana Edad , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: To analyze the clinical characteristics of chylous effusion and boost its diagnostic and therapeutic level. METHODS: A retrospective analysis was conducted for 123 cases of chylous effusion at our hospital between January 1990 and December 2009. RESULTS: The main clinical manifestations of chylous effusion included dyspnea (55.3%), edema (26.8%), abdominal distention (22.8%) and loss of weight (17.1%). Hypoalbuminemia was common (45.5%) and it was even more so in patients with idiopathic lymphopathies or of unknown causes (95.2%). The positive rates of identifying chylous effusion by Sudan III test, high triglyceride levels (> 1.25 mmol/L), lymphangiography or lymphangioscintigraphy were 89.1%, 80.6%, 70.6% and 89.5% respectively. Its common etiologies included injury (14.6%), malignancy (9.0%), infections (7.3%), lymphatic disorders or idiopathic lymphopathies (40%), drug-associated (2.4%) or associated with underlying disorders (16.3%). Efficacy was achieved in 63.6% of the patients and 21.8% died. CONCLUSIONS: Chylous effusion is a special type of serous effusion with multiple causes. Its clinical manifestations are often nonspecific. But malnutrition is common. Its causes are identified after a systematic evaluation. Treatment modalities include dietary modification, management of underlying causes and surgical approaches.
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Ascitis Quilosa , Derrame Pleural , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Preescolar , Quilotórax , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Derrame Pericárdico , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: To examine the correlation between the health-related quality of life measured by the St. George's Respiratory Questionnaire (SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis (LAM). METHODS: This study retrospectively analyzed the SGRQ scores and other measures (the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function) of patients diagnosed and confirmed with LAM. Altogether 38 patients between June 2007 and November 2009 were included. RESULTS: The mean values of the SGRQ three components (symptoms, activity, and impacts) and total scores in the LAM patients were 46.95 +/- 28.90, 58.47 +/- 25.41, 47.89 +/- 29.66, and 51.11 +/- 26.35, respectively. The SGRQ total or component scores were correlated well with the Borg scale of breathlessness, 6-minute walking distance, partial pressure of oxygen in arterial blood, spirometry and diffusion capacity of lung. There were poor correlations between SGRQ score and residual volume or total lung capacity. In our preliminary observation, sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment (n = 6). CONCLUSIONS: The SGRQ score in LAM is correlated well with physiological measures (Borg scale of breathlessness, 6-minute walking distance, blood oxygen levels, and pulmonary function tests). The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM. Treatment with sirolimus, an inhibitor of mammalian target of rapamycin, may improve the quality of life and patient's perception of breathlessness in LAM.
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Linfangioleiomiomatosis/psicología , Calidad de Vida , Encuestas y Cuestionarios , Adulto , Volumen Espiratorio Forzado , Humanos , Linfangioleiomiomatosis/fisiopatología , Persona de Mediana Edad , Volumen Residual , Capacidad VitalRESUMEN
OBJECTIVE: To observe the function of gamma delta T lymphocytes and the polymorphism of T cell receptor V delta chain in the lungs of asthmatic patients and explore the role of gamma delta T cells in airway inflammation. METHODS: Bronchoalveolar lavage fluid BALF was obtained from 7 asthmatic patients and 7 healthy control individuals. The percentage of gamma delta T cell in BALF was measured by flow cytometry. The gamma delta T cell in BALF was purified by magnetic labeled beads. Proliferous activity was examined by MTT assay. Cytokines secreted by gamma delta T cells in medium was assessed by enzyme-linked immunosorbent assay. Polymorphism of T cell receptor V delta chain was detected by RT-PCR and gene scan analysis. RESULTS: The proportion of gamma delta T cell in the BALF of asthmatic patients [(6.39+/-0.71)%] was significantly higher than that in control subjects [(2.62+/-0.37)%] (P<0.01). The proportion of macrophage in the BALF of asthmatic patients [(81+/-4)] was significantly lower than that in control subjects [(86+/-2)] (P<0.05). The proliferation rate of asthmatic patients [(284.2+/-43.6)%] was significantly higher than that of control subjects [(217.5+/-59.5)%] (P<0.05). Interleukin-4 secreted by gamma delta T cells of asthmatic patients [(18.9+/-3.1) pg/ml)] significantly increased when compared with the control subjects [(14.1+/-3.0) pg/ml] (P<0.05). The polymorphism of T cell receptor V delta chain was not significantly different between these two groups. CONCLUSIONS: The increase of gamma delta T cells in the lung of asthmatic patients further exacerbates Th1/Th2 disturbance and airway inflammation. Antigen recognition by gamma delta T cells is non-specific.
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Asma/inmunología , Genes Codificadores de la Cadena delta de los Receptores de Linfocito T/genética , Genes Codificadores de la Cadena gamma de los Receptores de Linfocito T/genética , Subgrupos de Linfocitos T/inmunología , Adulto , Asma/genética , Líquido del Lavado Bronquioalveolar/citología , Estudios de Casos y Controles , Proliferación Celular , Citocinas/metabolismo , Femenino , Humanos , Región Variable de Inmunoglobulina/genética , Pulmón/inmunología , Masculino , Persona de Mediana Edad , Polimorfismo Genético , Subgrupos de Linfocitos T/metabolismo , Balance Th1 - Th2RESUMEN
OBJECTIVE: To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome. METHODS: We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography (HRCT) since 2006 to 2009 at Peking Union Medical College Hospital. RESULTS: All patients were male, aged 65 (59 - 75) years, and 7 of them were smokers. Dyspnea on exertion was presented in 7 patients. Basal crackles were heard in 6 patients and finger clubbing was observed in 4 patients. Pulmonary function demonstrated that forced expiratory volume in one second/forced vital capacity (FEV1/FVC) was slightly lower with a median of 76% (range 60% to 86%), forced vital capacity (FVC) was 73% (51% - 92%), and total lung capacity (TLC) was 80% (59% - 114%). However, carbon monoxide diffusion capacity (D(L)CO) was significantly impaired (44%, 16% - 65%). HRCT findings included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. Pulmonary hypertension was seen in 6 patients. CONCLUSIONS: The co-existence of lower lung fibrosis and upper lung emphysema was found in some smokers. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange. A high prevalence of pulmonary hypertension is seen in CPFE syndrome. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.
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Enfisema Pulmonar/complicaciones , Fibrosis Pulmonar/complicaciones , Fumar , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfisema Pulmonar/diagnóstico por imagen , Enfisema Pulmonar/fisiopatología , Fibrosis Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar/fisiopatología , Radiografía , Pruebas de Función RespiratoriaRESUMEN
OBJECTIVE: To study the clinical characteristics and diagnosis of primary ciliary dyskinesia (PCD). METHODS: Four cases diagnosed as PCD by cilia electron microscopy examination from Jan. 2007 to Aug. 2009 in this hospital were retrospectively analyzed, and the related literature was reviewed. RESULTS: In the 4 patients, there were 1 male and 3 females. The age at disease onset was 0 - 10 years, and the age at diagnosis was 15 - 53 years. The most common symptoms were productive cough (4/4), sinusitis (3/4), and shortness of breath (3/4). Other manifestations included situs inversus (2/4), infertility (1/4), and tympanitis (1/4). Hypoxemia was found in 3 cases. Obstructive ventilatory impairment accompanied with diffusion dysfunction was observed in 2 cases, while the pulmonary function tests were normal in the other 2 cases. All 4 cases received chest CT scan, and bronchiectasis was present in all of them. Bilateral diffuse micronodules and patchy infiltrates were found in 3 and 2 cases respectively. Electron microscopic examination of the endobronchial biopsy specimen showed lack of dynein arms in 4, lack of muco-cilia in 2, and abnormal arrangement of microtubules in 2 cases. CONCLUSIONS: The Kartagener syndrome is relatively easy to be diagnosed, because it is characterized by the triad of sinusitis, bronchiectasis and situs inversus. However, PCD without situs inversus often goes unrecognized. PCD should be considered in patients with childhood onset disease, bronchiectasis, centrilobular micronodules or tree-in-bud signs in CT scan. Examination of the ciliary ultrastructure is essential to the confirmation of the diagnosis.
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Síndrome de Kartagener , Adolescente , Adulto , Femenino , Humanos , Síndrome de Kartagener/diagnóstico , Síndrome de Kartagener/patología , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
BACKGROUND: Primary Sjögren's syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. OBJECTIVES: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. METHODS: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. RESULTS: The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. CONCLUSIONS: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.
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Enfermedades Pulmonares Intersticiales/etiología , Pulmón/patología , Síndrome de Sjögren/complicaciones , Adulto , Antirreumáticos/uso terapéutico , Ciclofosfamida/uso terapéutico , Femenino , Glucocorticoides/uso terapéutico , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Prednisolona/uso terapéutico , Pruebas de Función Respiratoria , Estudios Retrospectivos , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/tratamiento farmacológico , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis (EAA). METHODS: Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases (acute, subacute, and chronic) according to clinical presentations. Follow-up data regarding treatment response, subsequent radiological and pulmonary function studies, and clinical outcomes were collected. RESULTS: A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds (6 cases, 28.6%). The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity (12 cases, 63.2%). The most common findings on high-resolution computed tomography (HRCT) were ground-glass opacities (13 cases, 68.4%) and centrilobular nodules (8 cases, 42.1%). Airway obstruction in pulmonary function test, emphysema, lung cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid (BALF) showed lymphocytosis. The total cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients (P<0.05). Nonnecrotizing granulomas were seen in 8 (47.1%) cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with available follow-up data. Five patients recurred. CONCLUSIONS: The characteristic abnormalities of pulmonary function, findings on HRCT, and pathology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.
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Alveolitis Alérgica Extrínseca/etiología , Adolescente , Adulto , Anciano , Alveolitis Alérgica Extrínseca/diagnóstico , Alveolitis Alérgica Extrínseca/diagnóstico por imagen , Alveolitis Alérgica Extrínseca/patología , Líquido del Lavado Bronquioalveolar/inmunología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , RadiografíaRESUMEN
OBJECTIVE: To study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis. METHODS: The HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed. RESULTS: There were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.2 years). Clinically, the patients presented with chronic cough and shortness of breath for 4 months to 6 years. Histologically, 7 cases showed usual interstitial pneumonitis (UIP)-like fibrosis. Patchy fibrosis was observed under the pleura, adjacent to interlobular septa and around bronchioles. In all of the 7 cases, foci of fibroblastic proliferation, as well as bronchiolar metaplasia of peribronchiolar alveoli and mild bronchiolitis, were noted. Three cases presented with mild honeycomb changes of lung and 3 cases showed non-specific interstitial pneumonitis (NSIP)-like fibrosis, in which the alveolar septa were expanded by fibrous tissue and collagen, with relative preservation of alveolar architecture. Bronchiolitis and lymphocytic infiltrates in alveolar septa were seen. Schaumann bodies were identified in 1 case. In general, patients with chronic hypersensitivity pneumonitis were younger than patients with idiopathic UIP. Computed tomography often showed upper and middle lobar involvement and mosaic attenuation. Compared with idiopathic UIP, the UIP-like fibrosis of chronic hypersensitivity pneumonitis often occurred not only under the pleura and adjacent to interlobular septa, but also around bronchioles and was accompanied by bronchiolar metaplasia. CONCLUSIONS: Chronic hypersensitivity pneumonitis can mimic other types of lung conditions with interstitial fibrosis, especially UIP and NSIP. As a result, some cases of chronic hypersensitivity pneumonitis may be misdiagnosed as such.
Asunto(s)
Alveolitis Alérgica Extrínseca/complicaciones , Fibrosis Pulmonar Idiopática/patología , Alveolos Pulmonares/patología , Adulto , Alveolitis Alérgica Extrínseca/patología , Enfermedad Crónica , Errores Diagnósticos , Femenino , Humanos , Fibrosis Pulmonar Idiopática/etiología , Enfermedades Pulmonares Intersticiales/patología , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
OBJECTIVE: The purpose of this paper was to investigate the clinical and radiological features of pulmonary tuberculosis presenting as interstitial lung diseases (ILD). METHODS: We analyzed the data of cases suspected of diffuse parenchyma lung diseases at this hospital between October 2003 and October 2007. The diagnosis of active pulmonary tuberculosis was based on epithelioid granuloma or positive acid-fast bacilli in lung biopsy and changes on serial radiographs obtained during treatment. RESULTS: The data of a series of 230 consecutive patients with suspected ILD were retrospectively analyzed. The diagnosis was confirmed by lung biopsy. Twelve patients were confirmed to have pulmonary tuberculosis. There were 5 males and 7 females with a mean age of 38 +/- 11 years (range, 17 - 68). The median course of disease in these patients was 3 months (range, 0.5 - 18 months). Patients with pulmonary tuberculosis presented with fever (11/12), cough (9/12), weight loss (7/12), dyspnea (7/12), lymphadenopathy (4/12), and splenohepatomegaly (2/12). On chest CT scan, ground-glass attenuation was identified in 4, bilateral patchy infiltration in 5, tree-in-bud appearance 1, and centrilobular lesions in 2 of the 12 patients. During the follow-up period (median, 9 month, range from 3 to 12 month), 11 patients improved, but 1 died of diabetic ketoacidosis. CONCLUSION: The diagnosis of pulmonary tuberculosis should be considered in suspected ILD patients presenting with fever, splenohepatomegaly and lymphadenopathy.
Asunto(s)
Enfermedades Pulmonares Intersticiales , Tuberculosis Pulmonar , Biopsia , Humanos , Pulmón , TuberculosisRESUMEN
OBJECTIVE: To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements. METHODS: A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed. RESULTS: We described a case of T cell intravascular lymphomatosis, who was a 36 year old man, complained of fever and multiple pulmonary shadows on chest radiograph and admitted to Peking Union Medical College Hospital in march, 2008. Open lung biopsy showed features characteristic of intravascular lymphomatosis. He received CHOP chemotherapy, but died 20 days after diagnosis. Most cases of intravascular lymphomatosis primarily manifested in the lung occurred in older patients, ranging from 36 to 79 years of age (mean 59 years), with a male predominance (M : F = 11 : 4). The chief complaints were dyspnoea (10/15), cough (9/15), fever (5/15) and weight loss (5/15). Pulmonary function tests usually revealed some degree of decreased diffusing capacity. Eight cases had high serum lactate dehydrogenase levels. Chest CT scan showed multiple reticular or/and nodular density. Immunophenotypically, 10 cases were B cell lineage, 3 cases were T cell lineage. Six cases of B cell intravascular lymphomatosis were followed, of whom 4 and were alive, and 2 died of respiratory failure. Three cases of T cell intravascular lymphomatosis showed poor prognosis, both of whom died of respiratory failure. CONCLUSIONS: Intravascular lymphomatosis primarily manifested in the lung is a rare malignant disease. Its pulmonary symptoms were nonspecific, and pulmonary function tests and chest CT scan manifested as those of interstitial pneumonia or pulmonary infection. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.
Asunto(s)
Neoplasias Pulmonares/metabolismo , Pulmón/patología , Linfoma no Hodgkin/patología , Neoplasias Vasculares/patología , Adulto , Humanos , Neoplasias Pulmonares/patología , MasculinoRESUMEN
OBJECTIVE: To study the clinicopathologic features, immunohistochemical findings and immunoglobulin heavy chain (IgH) gene rearrangement results of primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) and reactive lymphoid hyperplasia. METHODS: Twenty cases, included 13 cases of pulmonary MALToma and 7 cases of pulmonary lymphoid hyperplasia, encountered during the period from 1989 to 2007, were retrospectively analyzed. The samples were paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study and semi-nested polymerase chain reaction for IgH gene rearrangement were performed. RESULTS: The 13 cases of primary pulmonary MALToma were composed of a spectrum of lymphoid cells, including lymphocyte-like cells, centrocyte-like cells and mononuclear B cells with plasmacytoid differentiation. They often had diffuse or marginal zone growth patterns. Lymphoid follicles with neoplastic colonization were apparent. The lymphoma cells spread along alveolar septa and bronchovascular bundles. Vascular invasion was noted in 9 cases, pleura involvement in 6 cases and nodal involvement in 2 cases. Lymphoepithelial lesions (LEL) were identified in 9 cases of pulmonary MALToma. Immunohistochemically, the lymphocytes in LEL were CD20-positive and CD3-negative. On the other hand, LEL was also present in 2 of the 7 cases of lymphoid hyperplasia studied, with a mixture of CD20-positive B cells and CD3-negative T cells. Eight of the 9 cases of primary pulmonary MALToma were positive for IgH gene rearrangement, while all of the 7 cases of lymphoid hyperplasia were negative. CONCLUSIONS: Histologically, the cell population of primary pulmonary MALToma is similar to that of extranodal MALToma occurring in other organs. LEL, though commonly observed in pulmonary MALToma, are not specific and can also be seen in cases of reactive lymphoid hyperplasia. The immunophenotype of intraepithelial lymphocytes in pulmonary MALToma and reactive lymphoid hyperplasia is different. The presence of a monotonous population of CD20-positive intraepithelial lymphocytes supports a diagnosis of MALToma. IgH gene rearrangement study is also useful in differentiating both entities.
Asunto(s)
Neoplasias Pulmonares/patología , Linfoma de Células B/patología , Seudolinfoma/patología , Adulto , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Inmunoquímica/métodos , Inmunofenotipificación/métodos , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
OBJECTIVE: Polymyositis-dermatomyositis has been associated with various histological patterns of interstitial lung disease (ILD). This study was to investigate the clinico-pathologic features of interstitial lung diseases associated with polymyositis-dermatomyositis. METHODS: We retrospectively identified 26 patients with polymyositis-dermatomyositis associated ILD who underwent lung biopsies (6 autopsies, 5 surgical lung biopsies and 15 percutaneous lung biopsies) at our hospital during a 27-year period from January 1980 through October 2007. Histopathologic findings were analyzed and correlated with radiological features and outcome. RESULTS: Median age was 48 years (range, 19 to 65 years), and 16 patients were women. Chest X-ray imaging revealed bilateral infiltrates including ground-glass attenuation, patchy infiltration, and reticular opacities. The major histopathologic patterns included diffuse alveolar damage (DAD) (n = 5), lymphocytic interstitial pneumonia (n = 2), nonspecific interstitial pneumonia (NSIP) (cellular pattern, n = 6; mixed pattern, n = 8), organizing pneumonia (n = 4), usual interstitial pneumonia (UIP) (n = 1). Treatment commonly included prednisone with immunosuppressive agent. During the follow-up period (median, 15 month,range from 6 to 108 month), 18 patients improved or remained stable. Eight patients died, including 5 deaths from DAD, 2 from NSIP mixed pattern, 1 from UIP. CONCLUSIONS: A variety of histological patterns can be seen in patients with polymyositis-dermatomyositis-associated ILD. Those with DAD tended to have poor prognosis.