RESUMEN
The etiology of peripartum cardiomyopathy (PPCM) is unknown. Therefore, we evaluated the etiology of patients clinically diagnosed with PPCM using endomyocardial biopsy. We studied five patients diagnosed with PPCM following endomyocardial biopsy (age, 28-42 years; mean age, 35 years). Biopsied samples were evaluated using microscopy, including immunostaining and electron microscopy. The pathological findings were as follows: myocardial hypertrophy, myocardial fibrosis, and cell infiltration. Two patients were diagnosed with lymphocytic myocarditis, one with eosinophilic myocarditis, one with hypertensive heart disease, and one with a combination of hypertension and myocarditis. Endomyocardial biopsy suggested that the causes of PPCM were varied and related to myocarditis and myocardial overload due to hypertension.
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Cardiomiopatías , Hipertensión , Miocarditis , Humanos , Adulto , Miocarditis/diagnóstico , Miocarditis/patología , Periodo Periparto , Cardiomiopatías/diagnóstico , Miocardio/patología , Biopsia , Hipertensión/patologíaRESUMEN
Restrictive cardiomyopathy (RCM) is a rare primary myocardial disease, and its pathological features are yet to be determined. Restrictive cardiomyopathy with MHY7 mutation was diagnosed in a 65-year-old Japanese woman. Electron microscopy of a myocardial biopsy revealed electron-dense materials resulting from focal myocyte degeneration and necrosis as well as tubular structures and pseudo-inclusion bodies in some nuclei. These features may be associated with the pathogenesis of RCM.
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Miosinas Cardíacas/genética , Cardiomiopatía Restrictiva/patología , Células Musculares/patología , Mutación Missense , Cadenas Pesadas de Miosina/genética , Anciano , Biopsia , Cardiomiopatía Restrictiva/genética , Cardiomiopatía Restrictiva/metabolismo , Femenino , Humanos , Células Musculares/ultraestructura , LinajeRESUMEN
BACKGROUND: Lymphovascular invasion (LVI), which includes vascular or lymphatic invasions, is a representative prognostic factor even in patients with resected stage IA non-small cell lung cancer (NSCLC). Because tegafur-uracil is effective on cancers with LVI, we conducted a multi-center single-arm phase II study to estimate the efficacy of adjuvant tegafur-uracil in patients with LVI-positive stage IA NSCLC. METHODS: Patients with completely resected LVI-positive stage IA NSCLC were registered. LVI was diagnosed by consensus of two of three pathologists. Adjuvant chemotherapy consisted of 2 years of oral tegafur-uracil at 250 mg/m2/day. Fifty-five patients from 7 institutions were enrolled from June 2007 to September 2012. RESULTS: Among the 52 eligible patients, 36 (69.2%) completed the treatment course. There were 39 male and 13 female patients. The observation period was calculated as 562 to 3107 days using the reverse Kaplan-Meier method. The 5-year overall and relapse free survival rates were 94.2 and 88.5% respectively, which were significantly better than that of any other studies conducted on patients with LVI-positive stage IA NSCLC. Notably, the overall survival rate was 15% better than that of our prior retrospective study. The retrospective analysis of stage IA NSCLC patients who had received an operation in the same period revealed that the 5-year overall survival rate of the LVI positive group was 73.6% when adjuvant chemotherapy was not applied. Among 55 safety analysis sets, 4 cases of grade 3 hepatic function disorder (9.1%) and 5 cases of grade 2 anorexia (10.9%) were most frequently observed. No grade 4 adverse effects were encountered. CONCLUSION: A 2-year course of oral tegafur-uracil administration is feasible and might have a significant benefit in the adjuvant treatment of LVI-positive stage IA NSCLC. TRIAL REGISTRATION: UMIN identifier: UMIN000005921 ; Date of enrolment of the first participant to the trial: 19 June 2007; Date of registration: 5 July 2011 (retrospectively registered).
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Vasos Sanguíneos/patología , Carcinoma de Pulmón de Células no Pequeñas/tratamiento farmacológico , Neoplasias Pulmonares/tratamiento farmacológico , Vasos Linfáticos/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Carcinoma de Pulmón de Células no Pequeñas/patología , Carcinoma de Pulmón de Células no Pequeñas/cirugía , Quimioterapia Adyuvante/efectos adversos , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Enfermedades Gastrointestinales/inducido químicamente , Humanos , Estimación de Kaplan-Meier , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Invasividad Neoplásica , Neutropenia/inducido químicamente , Cooperación del Paciente , Neumonectomía , Profármacos/administración & dosificación , Profármacos/efectos adversos , Estudios Prospectivos , Tegafur/administración & dosificación , Tegafur/efectos adversos , Uracilo/administración & dosificación , Uracilo/efectos adversosRESUMEN
Background It is occasionally difficult to distinguish progressive massive fibrosis (PMF) from lung cancer on computed tomography (CT) in patients with pneumoconiosis. Purpose To evaluate the magnetic resonance imaging (MRI) features of PMF and to assess its ability to differentiate PMF from lung cancer. Material and Methods Between 2000 and 2014, 40 pulmonary lesions suspected to be lung cancer on the basis of CT in 28 patients with known pneumoconiosis were evaluated. Twenty-four of the 40 lesions were pathologically or clinically diagnosed as PMF. The signal pattern on T2-weighted (T2W) images, post-contrast enhancement pattern on T1-weighted (T1W) images, and the pattern of the time intensity curve (TIC) on contrast-enhanced dynamic studies were evaluated. All images were analyzed independently by two chest radiologists. Results All 24 PMF lesions showed low signal intensity (SI) on T2W images (sensitivity, 100%), while 15 of 16 lung cancer lesions showed intermediate or high SI on T2W images (specificity, 94%) when PMF was regarded as a positive result. Six of 17 PMF lesions showed a homogeneous enhancement pattern (sensitivity, 35%), and 4/9 lung cancer lesions showed an inhomogeneous or a ring-like enhancement pattern (specificity, 44%). Six of 16 PMF lesions showed a gradually increasing enhancement pattern (sensitivity, 38%), and 7/9 lung cancer lesions showed rapid enhancement pattern (specificity, 78%). Conclusion When differentiation between PMF and lung cancer in patients with pneumoconiosis is difficult on CT, an additional MRI study, particularly the T2W imaging sequence, may help differentiate between the two.
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Neoplasias Pulmonares/diagnóstico por imagen , Pulmón/patología , Imagen por Resonancia Magnética/métodos , Neumoconiosis/diagnóstico por imagen , Anciano , Anciano de 80 o más Años , Diagnóstico Diferencial , Fibrosis/diagnóstico por imagen , Humanos , Pulmón/diagnóstico por imagen , Neoplasias Pulmonares/patología , Masculino , Persona de Mediana Edad , Neumoconiosis/patología , Reproducibilidad de los Resultados , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVES: To retrospectively review the clinical, radiological and pathological data in patients who underwent surgical resection for pulmonary pleomorphic carcinoma (PC), and to analyse the prognostic predictors of survival. METHODS: The data were retrospectively examined for 33 consecutive patients (28 males and five females) who had undergone surgical resection for pulmonary PC. Cox's proportional-hazards model was used to analyse the prognostic predictors of survival. RESULTS: The size of the tumours ranged from 1.1 to 12.0 cm (mean 5.4 cm). The majority (26) of the tumours were located at the lung periphery, five tumours had cavitation, two had calcification and 14 had peritumoral ground-glass opacity. Most of the tumours showed heterogeneous enhancement and contained a low-density area (LDA) within the tumour. The 5-year overall survival of surgically resected PC was 36 % (standard error = 0.093). A multivariate analysis revealed the LDA grade [hazard ratio (HR), 2.019], pathological stage (HR, 7.552) and pathological N factor (HR, 0.370) to be significant predictors of a poorer prognosis. CONCLUSIONS: A greater component of LDA within the tumour on contrast-enhanced CT is associated with a poorer prognosis in patients with PC. KEY POINTS: PC has a poorer prognosis than other conventional NSCLC. The five-year OS of surgically resected PC was 36 %. A greater component of LDA on contrast-enhanced CT suggests a poorer prognosis.
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Adenoma Pleomórfico/patología , Carcinoma de Pulmón de Células no Pequeñas/patología , Neoplasias Pulmonares/patología , Adenoma Pleomórfico/mortalidad , Adenoma Pleomórfico/cirugía , Anciano , Carcinoma de Pulmón de Células no Pequeñas/mortalidad , Carcinoma de Pulmón de Células no Pequeñas/cirugía , Femenino , Humanos , Pulmón/diagnóstico por imagen , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Análisis Multivariante , Estadificación de Neoplasias , Pronóstico , Modelos de Riesgos Proporcionales , Radiografía , Estudios RetrospectivosAsunto(s)
Tumor Carcinoide , Metástasis de la Neoplasia , Anciano , Tumor Carcinoide/patología , Corazón , Humanos , Masculino , TóraxAsunto(s)
Neoplasias Cardíacas , Hemangioma , Hemangiosarcoma , Neoplasias Hepáticas , Neoplasias Cardíacas/diagnóstico por imagen , Hemangioma/diagnóstico por imagen , Hemangiosarcoma/diagnóstico por imagen , Humanos , Neoplasias Hepáticas/diagnóstico por imagen , Masculino , Persona de Mediana EdadRESUMEN
OBJECTIVES: The aim of this study was to clarify the molecular mechanisms elicited by toll-like receptor (TLR)3 in salivary gland cell death in patients with SS. METHODS: Expression of TLR3 and its downstream molecules was examined by immunohistochemical analysis, immunofluorescence, Western blot (WB), and antibody dot-blot array in labial salivary glands (LSGs), and cultured primary salivary gland epithelial cells (SGECs) obtained from patients with SS. We also investigated the difference of expression between ducts/alveoli of LSGs and cultured SGECs. RESULTS: Phosphorylated Fas-associated protein with death domain (p-FADD) or caspase-8 was not found in ducts or alveoli of LSGs from SS patients and controls. Weak expression of receptor-interacting serine/threonine-protein kinase 3 (RIPK3) was found in SS patients, whereas no staining was observed in LSGs of controls. In contrast to LSGs, stimulation of SGECs with polyinosinic:cytidylic acid (poly I:C) significantly induced the expression of RIPK3, p-FADD, and cleaved caspase-8 by immunofluorescence and RIPK3, p-FADD, and cleaved caspase-3 by WB. However, it was counteracted by epidermal growth factor (EGF). Co-localization of anti-apoptotic molecules hemeoxygenase-2, heat shock protein 27, and p-protein kinase B or p-Akt was induced in EGF-stimulated SGECs. CONCLUSIONS: We observed that poly I:C induced apoptosis of SGECs in vitro compared with a relatively low prevalence of apoptosis found in the ducts and alveoli of LSGs in vivo. Thus, we speculate that other counter-regulatory mechanisms, including those induced by EGF, might exist to protect against TLR3-mediated apoptosis of ductal and acinar epithelial cells in vivo.
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Apoptosis/inmunología , Glándulas Salivales Menores/metabolismo , Síndrome de Sjögren/metabolismo , Receptor Toll-Like 3/metabolismo , Adulto , Anciano , Línea Celular , Células Epiteliales/inmunología , Células Epiteliales/metabolismo , Proteína de Dominio de Muerte Asociada a Fas/metabolismo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fosforilación , Proteína Serina-Treonina Quinasas de Interacción con Receptores , Transducción de Señal/inmunología , Síndrome de Sjögren/inmunologíaRESUMEN
OBJECTIVE: To investigate whether aquaporins (AQPs) are involved in salivary gland dysfunction in patients with neuromyelitis optica (NMO) complicated with Sjögren's syndrome (SS). METHODS: Eight primary SS (pSS) patients, four NMO spectrum disorder (NMOsd) patients complicated with SS (NMOsd-SS), and three control subjects were enrolled. Immunohistochemistry of labial salivary glands (LSGs) was performed to determine the expressions of AQP4, AQP5, and tumor necrosis factor-alpha (TNF-α). In vitro expression of AQP5 was examined by Western blotting in cultured primary salivary gland epithelial cells (SGECs). RESULTS: No expression of AQP4 was shown in all LSGs. AQP5 was clearly expressed in the all acini, but the predominant localization of AQP5 in the apical side was diminished in the patients with pSS or NMOsd-SS compared with the controls and tended to be even lower in NMOsd-SS than pSS. The abnormal localization of AQP5 was associated with poor saliva secretion. No difference was found in TNF-α expression in the LSGs between patients with pSS and NMOsd-SS. AQP5 expression of SGECs in vitro was not changed by TNF-α or interleukin-10. CONCLUSIONS: Our results suggest that AQP5 but not AQP4 contributes to salivary secretion in patients with SS including those with NMO complicated with SS.
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Acuaporina 5/metabolismo , Neuromielitis Óptica/metabolismo , Saliva/metabolismo , Glándulas Salivales Menores/metabolismo , Síndrome de Sjögren/metabolismo , Adulto , Anciano , Acuaporina 4/metabolismo , Células Epiteliales/efectos de los fármacos , Células Epiteliales/metabolismo , Femenino , Humanos , Inmunohistoquímica , Interleucina-10/farmacología , Masculino , Persona de Mediana Edad , Neuromielitis Óptica/complicaciones , Neuromielitis Óptica/patología , Glándulas Salivales Menores/efectos de los fármacos , Glándulas Salivales Menores/patología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/patología , Factor de Necrosis Tumoral alfa/metabolismo , Factor de Necrosis Tumoral alfa/farmacologíaAsunto(s)
Cardiomiopatías/patología , Miocardio/ultraestructura , Estimulación Cardíaca Artificial , Cardiomiopatías/genética , Cardiomiopatías/terapia , Muerte Súbita Cardíaca/patología , Muerte Súbita Cardíaca/prevención & control , Femenino , Predisposición Genética a la Enfermedad , Humanos , Lamina Tipo A/genética , Persona de Mediana Edad , Mutación , Marcapaso Artificial , Fenotipo , Resultado del TratamientoRESUMEN
Reduced expressions of plakoglobin and connexin 43 have been reported in the myocardium of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). However, the relationships between these expression abnormalities and the clinical features of ARVC remain unknown.The expressions of plakoglobin and connexin 43 in myocardial biopsy specimens from 10 patients with confirmed ARVC, and 13 control patients without ARVC (non-ARVC; hypertrophic cardiomyopathy, n = 7; dilated cardiomyopathy, n = 6), were examined by immunostaining to evaluate the relationships between these expressions and the clinical characteristics of ARVC. The ratios of plakoglobin/N-cadherin and of plakoglobin/connexin 43 expressions were significantly lower in the ARVC group than in the control group. Significantly more patients had decreased plakoglobin expression in the ARVC group than in the control group (9/10 versus 7/13; P = 0.0376). Sustained ventricular tachycardia occurred more frequently in patients with ARVC and with decreased expressions of both plakoglobin and connexin 43 than in those with decreased expression of plakoglobin alone (5/5 versus 1/4, P = 0.048).Decreased expressions of both connexin 43 and plakoglobin in the myocardium might be associated with the development of arrhythmia in ARVC.
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Arritmias Cardíacas , Displasia Ventricular Derecha Arritmogénica , Conexina 43/metabolismo , Miocardio , gamma Catenina/metabolismo , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/metabolismo , Displasia Ventricular Derecha Arritmogénica/patología , Biomarcadores/metabolismo , Biopsia , Femenino , Humanos , Inmunohistoquímica , Japón , Masculino , Persona de Mediana Edad , Miocardio/metabolismo , Miocardio/patología , Valor Predictivo de las Pruebas , Estudios RetrospectivosRESUMEN
A 63-year-old man with enlargement of the bilateral submandibular glands visited with elevated serum IgG4. A biopsy specimen showed plasma cell infiltration with more than 50% IgG4/IgG staining, suggesting the existence of IgG4-related disease (IgG4-RD). Positive anti-SS-A/Ro antibody and the labial salivary gland's biopsy suggested existence of primary Sjögren's syndrome (pSS). Administration of glucocorticoid improved the serum IgG4 level while reducing the submandibular gland lesions. Positive TUNEL staining suggested the coexistence of IgG4-RD and pSS.
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Enfermedades Autoinmunes/complicaciones , Inmunoglobulina G/sangre , Síndrome de Sjögren/complicaciones , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/patología , Humanos , Masculino , Persona de Mediana Edad , Síndrome de Sjögren/inmunología , Síndrome de Sjögren/patología , Glándula Submandibular/patologíaRESUMEN
We developed an easy, quick and cost-effective detection method for lymph node metastasis called the semi-dry dot-blot (SDB) method, which visualizes the presence of cancer cells with washing of sectioned lymph nodes by anti-pancytokeratin antibody, modifying dot-blot technology. We evaluated the validity and efficacy of the SDB method for the diagnosis of lymph node metastasis in a clinical setting (Trial 1). To evaluate the validity of the SDB method in clinical specimens, 180 dissected lymph nodes from 29 cases, including breast, gastric and colorectal cancer, were examined. Each lymph node was sliced at the maximum diameter and the sensitivity, specificity and accuracy of the SDB method were determined and compared with the final pathology report. Metastasis was detected in 32 lymph nodes (17.8%), and the sensitivity, specificity and accuracy of the SDB method were 100, 98.0 and 98.3%, respectively (Trial 2). To evaluate the efficacy of the SDB method in sentinel lymph node (SLN) biopsy, 174 SLNs from 100 cases of clinically node-negative breast cancer were analyzed. Each SLN was longitudinally sliced at 2-mm intervals and the sensitivity, specificity, accuracy and time required for the SDB method were determined and compared with the intraoperative pathology report. Metastasis was detected in 15 SLNs (8.6%), and the sensitivity, specificity, accuracy and mean required time of the SDB method were 93.3, 96.9, 96.6 and 43.3 min, respectively. The SDB method is a novel and reliable modality for the intraoperative diagnosis of SLN metastasis.
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Biomarcadores de Tumor/metabolismo , Neoplasias de la Mama/patología , Carcinoma Ductal de Mama/secundario , Carcinoma Intraductal no Infiltrante/patología , Carcinoma Lobular/secundario , Neoplasias del Colon/patología , Biopsia del Ganglio Linfático Centinela , Neoplasias Gástricas/patología , Axila , Neoplasias de la Mama/metabolismo , Neoplasias de la Mama/cirugía , Carcinoma Ductal de Mama/metabolismo , Carcinoma Ductal de Mama/cirugía , Carcinoma Intraductal no Infiltrante/metabolismo , Carcinoma Intraductal no Infiltrante/cirugía , Carcinoma Lobular/metabolismo , Carcinoma Lobular/cirugía , Neoplasias del Colon/metabolismo , Neoplasias del Colon/cirugía , Femenino , Estudios de Seguimiento , Humanos , Immunoblotting , Técnicas para Inmunoenzimas , Escisión del Ganglio Linfático , Metástasis Linfática , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Pronóstico , Estudios Prospectivos , Sensibilidad y Especificidad , Neoplasias Gástricas/metabolismo , Neoplasias Gástricas/cirugíaAsunto(s)
Cardiomiopatías/patología , Miocarditis/patología , Miocardio/patología , Trastornos Puerperales/patología , Cardiomiopatías/diagnóstico por imagen , Cardiomiopatías/fisiopatología , Femenino , Humanos , Persona de Mediana Edad , Miocarditis/diagnóstico por imagen , Miocarditis/fisiopatología , Embarazo , Trastornos Puerperales/diagnóstico por imagen , Trastornos Puerperales/fisiopatología , Volumen Sistólico , Factores de Tiempo , Función Ventricular IzquierdaRESUMEN
BACKGROUND: The pathogenesis of chronic pulmonary aspergillosis (CPA) including chronic necrotizing pulmonary aspergillosis (CNPA), chronic cavitary pulmonary aspergillosis (CCPA), and simple aspergilloma (SA) has been poorly investigated. We examined all types of CPA cases with histopathological evidence to clarify the differences in pathogenesis and clinical features. METHOD: We searched for cases diagnosed as pulmonary aspergillosis by histopathological examination in Nagasaki University Hospital between 1964 and September 2010. All available clinical information including radiological findings were collected and analyzed. RESULT: We found 7, 5, 8, and 7 cases of proven CNPA, probable CNPA, CCPA, and SA, respectively. The radiograph of proven and probable CNPA was initially infiltrates or nodules that progress to form cavities with or without aspergilloma, whereas the radiograph of CCPA showed pre-existed cavities and peri-cavitary infiltrates with or without aspergilloma. The patients with proven and probable CNPA exhibited not only respiratory symptoms but also systemic symptoms and malnutrition. Aspergillus fumigatus was the most frequently isolated Aspergillus species (n = 14), however, Aspergillus niger was the predominant isolated species in proven CNPA cases (n = 4). CONCLUSION: Our data indicate that the cases with chronic infiltration, progressive cavitation, and subsequent aspergilloma formation should be diagnosed as CNPA, and the cases with pre-existed cavities showing peri-cavitary infiltrates with or without aspergilloma would mean CCPA. However, it may be difficult to distinguish the two subtypes if a series of adequate radiography films are not available. We propose the term "chronic progressive pulmonary aspergillosis (CPPA)" for the clinical syndrome including both CNPA and CCPA.
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Aspergilosis Pulmonar Invasiva/clasificación , Aspergilosis Pulmonar Invasiva/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Cohortes , Diagnóstico Diferencial , Femenino , Humanos , Aspergilosis Pulmonar Invasiva/diagnóstico por imagen , Aspergilosis Pulmonar Invasiva/patología , Masculino , Persona de Mediana Edad , Radiografía TorácicaRESUMEN
BACKGROUND: Ki-67 expression is a well-established prognostic marker in various cancers. However, Ki-67 expression is also known as being heterogeneous. We investigated the prognostic significance of Ki-67 from the view of staining heterogeneity by the technique of Spiral Array. METHODS: 100 cases of resected lung cancer from Toyama university hospital archive were collected. Spiral Array blocks were generated out of 100 cases using 100 µm thick paraffin sections. Four µm thick sections of the Array block were stained for Ki-67. Staining results in each reel were scored for areas with lowest (LS), highest (HS), and average (AS) expression, exclusively in the cancer cells. Heterogeneity score (HeS) was designed as the difference between HS and LS. The scores were divided into four grades (0-3). Clinical information was collected, and the prognostic significance of Ki-67 was analyzed. RESULTS: Pathological stage was available for 91 patients (43 stage IA, 22 stage IB, 2 stage IIA, 9 stage IIB, 13 stage IIIA, 1 stage IIIB, and 1 stage IV). The HS of Ki-67 score in non-small cell lung cancer was 3 in 17 cases, 2 in 27 cases, 1 in 28 cases, 0 in 21 cases, and 4 reels were lost. 78 cases had clinical follow up. 74 cases had all the information available and were analyzed for correlation between Ki-67 expression and survival. Cases with score 2 and 3 of HS and HeS showed significant poorer prognosis (both P < 0.001), whereas LS or AS did not show significance. The results were identical when analyzing adenocarcinoma and squamous cell carcinoma, separately. Cox multivariate analysis of Ki-67 showed that HS was an independent risk factor affecting overall survival. CONCLUSIONS: Ki-67 is a strong prognostic marker for non-small cell lung cancer when the degree of highest staining frequency or heterogeneity is considered.
RESUMEN
BACKGROUND: Heat shock protein (HSP) 47, a collagen-specific molecular chaperone, is involved in the processing and/or secretion of procollagen. We hypothesized that HSP47 could be a useful marker for fibrotic lung disease. The aim of this study was to evaluate serum levels of HSP47 in patients with various idiopathic interstitial pneumonias (IIPs). METHODS: Subjects comprised 9 patients with acute interstitial pneumonia (AIP), 12 with cryptogenic organizing pneumonia (COP), 16 with nonspecific interstitial pneumonia (NSIP), 19 with idiopathic pulmonary fibrosis (IPF), and 19 healthy adult volunteers. RESULTS: Patients with AIP had serum HSP47 levels that were significantly higher than those of COP, NSIP or IPF patients and those of healthy volunteers. In contrast, serum levels of HSP47 among patients with COP, NSIP, IPF, and healthy volunteers did not differ significantly. Receiver operating characteristic curves revealed that the cut-off level for HSP47 that resulted in the highest diagnostic accuracy for discriminating between AIP and COP, NSIP, IPF, and healthy controls was 859.3 pg/mL. The sensitivity, specificity, and diagnostic accuracy were 100.0%, 98.5%, and 98.7%, respectively. CONCLUSION: The present results demonstrate that, among patients with various IIPs, serum levels of HSP47 were elevated specifically in patients with AIP.
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Proteínas del Choque Térmico HSP47/sangre , Neumonías Intersticiales Idiopáticas/sangre , Enfermedad Aguda , Adulto , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
Sarcoidosis is a granulomatous disorder of unknown etiology, with several clinical manifestations. Löfgren's syndrome is an acute type of sarcoidosis, characterized by the triad of arthritis, erythema nodosum, and bilateral hilar lymphadenopathy (BHL), which spontaneously resolve within about 2 years. Löfgren's syndrome is common among young white women from Nordic countries and Ireland, but it is very rare in Japan. Because the incidence of Löfgren's syndrome varies according to race, most studies on Löfgren's syndrome, including HLA typing, have been reported in Western countries. Indeed, HLA-DR3 has been reported to be associated with Löfgren's syndrome in Western countries, although the association between HLA typing and Japanese Löfgren's syndrome remains unclear. Here we present a Japanese patient with Löfgren's syndrome. A 34-year-old female patient was hospitalized with arthritis and erythema nodosum. Chest computed tomography revealed mediastinal and BHL. Endobronchial ultrasound-guided transbronchial needle aspiration showed non-caseating epithelioid cell granulomas. Löfgren's syndrome was thus diagnosed. Her ankle arthralgia and bilateral ankle swelling recovered without steroid treatment within two months, and the BHL almost completely diminished one year after admission. Her HLA genotype contains DR12. We also reviewed the literature on 11 Japanese patients with Löfgren's syndrome, showing that HLA-DR12 is present in five out of nine patients (55.6%). The relevant data were unavailable in the remaining three patients. Importantly, only 5.4% of registered donors in the Japan Marrow Donor Program are positive for this allele. We suggest the potential link between HLA-DR12 and the pathogenesis of Löfgren's syndrome in Japanese patients.