Disfunciones del metabolismo del surfactante pulmonar: caso clínico pediátrico / Pulmonary surfactant metabolism dysfunction: A pediatric clinical case report

Las enfermedades pulmonares intersticiales son patologías poco frecuentes en pediatría. Dentro de ellas, se incluyen las disfunciones del metabolismo del surfactante pulmonar, molécula anfipática cuya función es disminuir la tensión superficial y evitar el colapso alveolar. Se presenta el caso de un lactante de 6 meses, en seguimiento por bajo peso, que presentó dificultad respiratoria aguda y cianosis; la radiografía de tórax evidenció infiltrado intersticial, neumomediastino y neumotórax bilateral. Al interrogatorio, surgió antecedente materno de internación al año de vida, con requerimiento de oxigenoterapia prolongada y diagnóstico desconocido; presenta signos de hipoxia crónica. El paciente cursó internación con requerimiento de oxigenoterapia. Se realizaron estudios complementarios en búsqueda de etiología, sin resultados positivos. La tomografía de tórax evidenció opacidades en vidrio esmerilado, engrosamiento del intersticio septal y áreas de atrapamiento aéreo; con resultado de biopsia pulmonar y estudio genético se llegó al diagnóstico de disfunción del metabolismo del surfactante pulmonar.

Interstitial lung diseases are rare in pediatrics. They include dysfunctions in the metabolism of pulmonary surfactant, an amphipathic molecule that reduces surface tension and prevents alveolar collapse. Here we describe the case of a 6-month-old infant controlled for low weight, who presented with acute respiratory distress and cyanosis; his chest X-ray showed interstitial infiltrate, pneumomediastinum, and bilateral pneumothorax. During history-taking, it was noted that his mother had a history of hospitalization at 1 year old with unknown diagnosis, requiring prolonged oxygen therapy; she now shows signs of chronic hypoxia. The patient was hospitalized and required oxygen therapy. Ancillary tests were done to look for the etiology of the condition, with no positive results. A chest computed tomography showed groundglass opacities, thickening of the septal interstitium, and areas of air trapping; based on the results of a lung biopsy and a genetic study, pulmonary surfactant metabolism dysfunction was diagnosed.

Anti-neutrophil cytoplasmic antibodies associated vasculitis with interstitial lung disease: a case report

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis is a necrotizing vasculitis that primarily affect small blood vessels of the airway and kidneys. The major clinicopathologic feature include microscopic polyangiitis and granulomatosis with polyangiitis. There are several studies done that have shown the association of interstitial lung disease (ILD) and ANCA vasculitis. Majority of the studies showed that the prognosis was bad in patients with AAV and ILD, then those without it, and patients with pulmonary fibrosis and ANCA had as low a prognosis as patients with IPF without ANCA. We hereby report a case of a female patient in her 70s who was been treated as a case of infection at different hospital, only to be diagnosed later as ANCA associated vasculitis with interstitial lung disease. Methylenetetrahydrofolate reductase (MTHFR) enzyme is one of the key enzymes involved in the metabolism of folate.

Clinical analysis of lung transplantation for lung chronic graft-versus-host disease after hematopoietic stem cell transplantation / 器官移植

<b>Objective</b> To evaluate clinical efficacy of lung transplantation for lung chronic graft-versus-host disease (cGVHD) after hematopoietic stem cell transplantation (HSCT). <b>Methods</b> Clinical data of 12 patients undergoing lung transplantation for lung cGVHD were retrospectively analyzed. Preoperative clinical manifestations and involved organs of patients were analyzed. The lung function before and after lung transplantation was compared, and the survival of patients after lung transplantation was analyzed. <b>Results</b> Eleven patients underwent HSCT due to primary hematological malignancies, including 9 cases of leukemia, 1 case of myelodysplastic syndrome, 1 case of lymphoma. And 1 case underwent HSCT for systemic lupus erythematosus. Among 12 cGVHD patients, skin involvement was found in 8 cases, oral cavity involvement in 5 cases, gastrointestinal tract involvement in 4 cases and liver involvement in 3 cases. All 12 patients developed severe respiratory failure caused by cGVHD before lung transplantation, including 9 cases of typeⅡ respiratory failure and 3 cases of type Ⅰ respiratory failure. Two patients underwent right lung transplantation, 2 cases of left lung transplantation and 8 cases of bilateral lung transplantation. The interval from HSCT to lung transplantation was 75 (19-187) months. Upon the date of submission, postoperative follow-up time was 18 (7-74) months. Ten patients survived, 1 died from severe hepatitis at postoperative 22 months, and 1 died from gastrointestinal bleeding at postoperative 6 months. No recurrence of primary diseases was reported in surviving patients. <b>Conclusions</b> Lung transplantation is an efficacious treatment for lung cGVHD after HSCT, which may prolong the survival time and improve the quality of life of the recipients.

A unicenter real-world study of the correlation factors for complete clinical response in idiopathic inflammatory myopathies / 北京大学学报(医学版)

Objective:To investigate the correlation factors of complete clinical response in idiopathic inflammatory myopathies(IIMs)patients receiving conventional treatment.Methods:Patients diagnosed with IIMs hospitalized in Peking University People's Hospital from January 2000 to June 2023 were in-cluded.The correlation factors of complete clinical response to conventional treatment were identified by analyzing the clinical characteristics,laboratory features,peripheral blood lymphocytes,immunological indicators,and therapeutic drugs.Results:Among the 635 patients included,518 patients finished the follow-up,with an average time of 36.8 months.The total complete clinical response rate of IIMs was 50.0％(259/518).The complete clinical response rate of dermatomyositis(DM),anti-synthetase syn-drome(ASS)and immune-mediated necrotizing myopathy(IMNM)were 53.5％,48.9％and 39.0％,respectively.Fever(P=0.002)and rapid progressive interstitial lung disease(RP-ILD)(P=0.014)were observed much more frequently in non-complete clinical response group than in complete clinical re-sponse group.The aspartate transaminase(AST),lactate dehydrogenase(LDH),D-dimer,erythrocyte sedimentation rate(ESR),C-reaction protein(CRP)and serum ferritin were significantly higher in non-complete clinical response group as compared with complete clinical response group.As for the treat-ment,the percentage of glucocorticoid received and intravenous immunoglobin(IVIG)were significantly higher in non-complete clinical response group than in complete clinical response group.Risk factor analysis showed that IMNM subtype(P=0.007),interstitial lung disease(ILD)(P=0.001),eleva-ted AST(P=0.012),elevated serum ferritin(P=0.016)and decreased count of CD4+T cells in peripheral blood(P=0.004)might be the risk factors for IIMs non-complete clinical response.Conclu-sion:The total complete clinical response rate of IIMs is low,especially for IMNM subtype.More effec-tive intervention should be administered to patients with ILD,elevated AST,elevated serum ferritin or decreased count of CD4+T cells at disease onset.

Diagnosis, treatment and research progress of hypersensitivity pneumonitis in children / 中华实用儿科临床杂志

Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD). It is a disease with highly heterogeneous clinical manifestations, severity and outcomes, which are associated with individual sensitivity, as well as property, dosage, duration and frequency of exposure to the antigens.The 2020 adult HP guideline reclassifies it and describes its radiographic features in detail.HP often occurs in adults, also affects the pediatric population and is one of the most common ILDs in children.The most common factors causing HP in children are avian and fungal antigens in the home environment.The diagnosis of HP is based on clear antigens, typical symptoms and characteristic radiological manifestations.The serum-specific IgG antibody, bronchoalveolar lavage fluid, and pulmonary function tests can help diagnose HP clearly, and lung histopathology is required for children whose diagnosis cannot be confirmed.Early diagnosis and adequate avoidance of antigen exposure are the keys to its treatment and prognosis.

Pharmaceutical care for a case of tumor patient with interstitial lung disease due to EGFR-TKI and AIDS opportunistic infection / 中国药房

OBJECTIVE To provide a reference for drug treatment and pharmaceutical care in AIDS patients with tumor. METHODS For a case of AIDS complicated with pulmonary adenocarcinoma， interstitial lung disease occurred repeatedly in the course of targeted therapy， and bacterial and fungal infections could not be ruled out. Clinical pharmacists provided pharmaceutical care such as medication monitoring， drug reconciliation， and adverse reaction monitoring for the patient. RESULTS The patient’s use of Amivantamab is “highly likely related” to adverse reactions such as interstitial lung disease， and it is recommended by the clinical pharmacist that the targeted therapy drugs should be suspended， and hormone medication monitoring plans should be formulated. For the possible pathogens of AIDS opportunistic infection， it was recommended to stop ertapenem and foscarnet sodium， monitor voriconazole concentration in blood and follow up on the safety and antifungal course of voriconazole. According to the drug-drug interaction and the patient’s condition， the anti-AIDS drug was adjusted to bictegravir sodium， emtricitabine and tenofovir alafenamide. For the possibility of Pneumocystis carinii pneumonia， thrombosis and gastric mucosal injury， preventive drugs such as Compound sulfamethoxazole， nadroparin calcium and esomeprazole were recommended. Physicians followed the advice of the clinical pharmacists. The patient made a good outcome after drug treatment without any significant adverse reactions or drug-drug interactions， and was discharged smoothly. CONCLUSIONS AIDS patients with tumor have complex disease condition and use many therapeutic drugs. Clinical pharmacists should conduct drug treatment management as drug reconciliation and medication monitoring and provide individual pharmaceutical care for these patients to guarantee the safety of drug use.

Research progress on role of cytokines in pathogenesis of connective tissue disease-associated interstitial lung disease / 中国免疫学杂志

Interstitial lung disease(ILD)is one of common pulmonary complications of connective tissue disease(CTD),which seriously affects quality of life and significantly increases risk of death of patients with CTD.However,related immune mecha-nism of CTD-ILD is not clear yet.This paper systematically reviews involvement of various cytokines in pathogenesis of CTD-ILD.Pathogenesis of CTD-ILD and correlation between cytokines and clinical indicators were discussed from cell level,animal model and clinical trials,to investigate whether cytokines can be targeted for treatment of CTD-ILD to help guide future research and clinical practice.

Effect of gender difference on the model of rheumatoid arthritis induced by bovine type Ⅱ collagen / 中国比较医学杂志

Objective To establish female and male bovine collagen-induced arthritis(CIA)models and compare the effects of gender differences on joint and extra-articular manifestations of the CIA model.Methods The CIA model was induced by injection of bovine type Ⅱ collagen and Freund's complete adjuvant into female and male SD rats.The general condition,arthritis index,foot swelling,serum proinflammatory factors and plasminogen activator inhibitor levels,spleen index,knee and ankle joint pathologies,right rear paw bone destruction,and pulmonary interstitial lesions were evaluated.Results The arthritis index of female CIA rats was significantly higher than that of male CIA rats on day 21 after initial immunization(P＜0.05),but no significant difference was found in the degree of foot swelling between the two groups at any time point(P＞0.05).Serum tumor necrosis factor α,interleukin-1β,and the spleen index of female CIA rats were significantly higher than those of male CIA rats(P＜0.05,P＜0.001).No significant difference was found in plasminogen activator inhibitor levels(P＞0.05).The scores of inflammatory cell infiltration and synovial hyperplasia in the knee and ankle pathologies of female CIA rats were significantly higher than those of male CIA rats(P＜0.05),and cartilage damage of the knee joint and bone damage of the right rear paw of female CIA rats were significantly higher than that of male rats(P＜0.05).Both male and female CIA rats showed pulmonary interstitial inflammatory cell infiltration and mild fibrosis,but the pulmonary interstitial lesions in females were more severe than those in males.Conclusions Female and male CIA models established in SD rats have arthritis and pulmonary interstitial lesions,but the lesion degree in female CIA rats is more serious.When using CIA models for RA-related research,attention should be focused on the effect of gender differences.

The impact of hospitalization on mortality in patients with connective tissue disease-associated interstitial lung disease: a medical records review study

Abstract Background Interstitial lung disease (ILD) remains one of the most important causes of morbidity and mortality in patients with Connective Tissue Diseases (CTD). This study evaluated the impact of hospitalization on mortality in an ethnically and racially diverse cohort of CTD-ILD patients. Methods We conducted a medical records review study at Montefiore Medical Center, Bronx, NY. We included 96 patients and collected data on demographic characteristics, reasons for hospitalization, length of stay, immunosuppressant therapy use, and mortality. We stratified our patients into two cohorts: hospitalized and nonhospitalized. The hospitalized cohort was further subdivided into cardiopulmonary and non-cardiopulmonary admissions. Two-sample tests or Wilcoxon's rank sum tests for continuous variables and Chi-square or Fisher's exact tests for categorical variables were used for analyses as deemed appropriate. Results We identified 213 patients with CTD-ILD. Out of them, 96 patients met the study's inclusion criteria. The majority of patients were females (79%), and self-identified as Hispanic (54%) and Black (40%). The most common CTDs were rheumatoid arthritis (RA) (29%), inflammatory myositis (22%), and systemic sclerosis (15%). The majority (76%) of patients required at least one hospitalization. In the non-hospitalized group, no deaths were observed, however we noted significant increase of mortality risk in hospitalized group (p = 0.02). We also observed that prolonged hospital stay (> 7 days) as well as older age and male sex were associated with increased mortality. Conclusion Prolonged (> 7 days) hospital stay and hospitalization for cardiopulmonary causes, as well as older age and male sex were associated with an increased mortality risk in our cohort of CTD-ILD patients.

A new proposal for phenotypic classification and outcome assessment of dermatomyositis based on clinical manifestations and serological testing

Abstract Background Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging. Objectives Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes. Methods This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University. 162 DM patients were enrolled for unsupervised hierarchical cluster analysis. In addition, we divided the clinical outcomes of DM patients into four subgroups: withdrawal, stabilization, aggravation, and death, and compared the clinical profiles amongst the subgroups. Results Out of 162 DM patients, three clusters were defined. Cluster 1 (n = 40) was mainly grouped by patients with prominent muscular involvement and mild Interstitial Lung Disease (ILD). Cluster 2 (n = 72) grouped patients with skin rash, anti-Melanoma Differentiation Associated protein 5 positive (anti-MDA5+), and Rapid Progressive Interstitial Lung Disease (RP-ILD). Cluster 3 (n = 50) grouped patients with the mildest symptoms. The proportion of death increased across the three clusters (cluster 3 < cluster 1 < cluster 2). Study limitations The number of cases was limited for the subsequent construction and validation of predictive models. We did not review all skin symptoms or pathological changes in detail. Conclusions We reclassified DM into three clusters with different risks for poor outcome based on diverse clinical profiles. Clinical serological testing and cluster analysis are necessary to help clinicians evaluate patients during follow-up and conduct phenotype-based personalized care in DM.

Enfermedad pulmonar intersticial en colangitis biliar primaria / Interstitial Lung Disease in Primary Biliary Cholangitis

Durante el transcurso de la colangitis biliar primaria se puede desarrollar compromiso intersticial pulmonar: neumonía organizada, fibrosis intersticial, neumonía intersticial linfoide, neumonía intersticial no específica. A pesar de que el diagnóstico de colangitis biliar primaria usualmente precede a las manifestaciones pulmonares, puede ocurrir lo inverso. La frecuencia de enfermedad intersticial en pacientes con colangitis biliar primaria no es conocida con exactitud. Puede estar o no asociada a otras enfermedades del tejido conectivo; por lo tanto, es necesario realizar una búsqueda sistemática de estas y de las manifestaciones pulmonares de dicha entidad. Presentamos el caso de una paciente con diagnóstico previo de colangitis biliar primaria, la cual desarrolla durante el curso de su enfermedad, afectación pulmonar intersticial.

During the course of PBC, interstitial lung involvement may develop: organizing pneu monia, interstitial fibrosis, lymphoid interstitial pneumonia, or non-specific interstitial pneumonia. Although the diagnosis of PBC usually precedes pulmonary manifestations, the opposite can occur. The frequency of interstitial disease in patients with PBC is not exactly known. It may or may not be associated with other connective tissue diseases; therefore, it is necessary to carry out a systematic search of these diseases and the pulmonary manifestations of this entity. We present the case of a patient with a previ ous diagnosis of PBC, who developed interstitial lung involvement during the course of the disease.

Rendimiento diagnóstico y morbimortalidad de las biopsias pulmonares quirúrgicas en enfermedad pulmonar intersticial difusa

Introducción: Se denomina Enfermedad Pulmonar Intersticial Difusa (EPID) a un conjunto heterogéneo de patologías caracterizadas por inflamación y fibrosis pulmonar. El diagnóstico basado en patrones clínicos o radiológicos puede, ocasionalmente, ser insuficiente para iniciar un tratamiento. La biopsia pulmonar quirúrgica es una alternativa cuando se requiere aumentar la precisión diagnóstica luego de discusión multidisciplinaria. Objetivo: Describir el rendimiento diagnóstico, morbilidad y mortalidad de las biopsias quirúrgicas pulmonares en un hospital público chileno. Pacientes y Método: Cohorte retrospectiva de todos los pacientes a quienes se realizó biopsia quirúrgica por diagnóstico de EPID entre los años 2010 y 2020, indicada por un comité multidisciplinario. Se excluyen procedimientos similares o biopsias con diagnóstico de EPID como hallazgo incidental. Resultados: 38 pacientes intervenidos, mediana de edad de 63 años, 47% femenino. Solo 1 (2,6%) paciente operado de urgencia, y 34 (89,5%) por videotoracoscopía. 5 (13,1%) pacientes presentaron morbilidad, en 4 de ellos fuga aérea, ninguno requiriendo intervención adicional. No hubo rehospitalización, reoperación ni mortalidad a 90 días. En el 95% de los casos se alcanzó un diagnóstico preciso de la EPID tras discusión multidisciplinaria. Discusión: Se observa un alto rendimiento diagnóstico y una baja morbimortalidad en los pacientes estudiados. La baja frecuencia de procedimientos de urgencia y la adecuada indicación en comité multidisciplinario puede haber contribuido a la baja morbilidad. Conclusión: La biopsia pulmonar quirúrgica en un hospital general tiene un alto rendimiento diagnóstico cuando se discute en comité multidisciplinario para precisar el diagnostico en EPID, con una baja morbimortalidad si se seleccionan adecuadamente los pacientes.

Background: Interstitial Lung Disease (ILD) is a heterogeneous group of diseases characterized by inflammation and fibrosis of the lung. Diagnosis based exclusively on clinical or radiologic patterns may be inaccurate, and if a reliable diagnosis cannot be made, surgical lung biopsy can be strongly considered to increase the diagnostic yield after multidisciplinary committee. Objective: To review the diagnostic results, morbidity, and mortality of surgical biopsies in a chilean public health institution. Patients and Method: Retrospective cohort of patients operated for diagnostic purposes for ILD between 2010 - 2020. Surgical biopsies done for other diagnoses were excluded. Results: 38 patients were included, with a median age of 63 years, 47% were female. Only 1 patient (2.6%) underwent emergency surgery and 89.5% underwent minimally invasive surgery techniques. 5 patients had some morbidity (13.1%), 4 of them being air leak. All complications were successfully managed conservatively. We had no readmission, reoperations, or 90-day mortality in this cohort. In 95% of the cases an accurate diagnosis of ILD was reached after multidisciplinary discussion. Discussion: In our experience surgical lung biopsy has a high diagnostic yield and a low morbidity and mortality. A low number of emergency procedures and accurate surgical indication by an expert committee could explain the low morbidity. Conclusion: Surgical lung biopsy in a general hospital reach a high diagnostic performance when discussed in a multidisciplinary committee to specify the diagnosis in ILD, with low morbidity and mortality if patients are properly selected.

Risk factors for clinically significant diffuse parenchymal lung abnormalities persisting after severe COVID-19 pneumonia

Background & objectives: The risk factors for clinically significant diffuse parenchymal lung abnormalities (CS-DPLA) persisting after severe coronavirus disease 2019 (COVID-19) pneumonia remain unclear. The present study was conducted to assess whether COVID-19 severity and other parameters are associated with CS-DPLA. Methods: The study participants included patients who recovered after acute severe COVID-19 and presented with CS-DPLA at two or six month follow up and control group (without CS-DPLA). Adults volunteers without any acute illness, chronic respiratory illness and without a history of severe COVID-19 were included as healthy controls for the biomarker study. The CS-DPLA was identified as a multidimensional entity involving clinical, radiological and physiological pulmonary abnormalities. The primary exposure was the neutrophil-lymphocyte ratio (NLR). Recorded confounders included age, sex, peak lactate dehydrogenase (LDH), advanced respiratory support (ARS), length of hospital stay (LOS) and others; associations were analyzed using logistic regression. The baseline serum levels of surfactant protein D, cancer antigen 15-3 and transforming growth factor-? (TGF-?) were also compared among cases, controls and healthy volunteers. Results: We identified 91/160 (56.9%) and 42/144 (29.2%) participants with CS-DPLA at two and six months, respectively. Univariate analyses revealed associations of NLR, peak LDH, ARS and LOS with CS-DPLA at two months and of NLR and LOS at six months. The NLR was not independently associated with CS-DPLA at either visit. Only LOS independently predicted CS-DPLA at two months [adjusted odds ratios (aOR) (95% confidence interval [CI]), 1.16 (1.07-1.25); P<0.001] and six months [aOR (95% CI) and 1.07 (1.01-1.12); P=0.01]. Participants with CS-DPLA at six months had higher baseline serum TGF-? levels than healthy volunteers. Interpretation and conclusions: Longer hospital stay was observed to be the only independent predictor of CS-DPLA six months after severe COVID-19. Serum TGF-? should be evaluated further as a biomarker.

Caracterización de la enfermedad pulmonar intersticial en los pacientes con esclerosis sistémica / Description of Interstitial Lung Disease in Systemic Sclerosis Patients

Introducción: La esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por vasculopatía, activación del sistema inmune y aumento de depósitos de matriz extracelular. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, ha pasado a ser la primera causa de muerte en estos pacientes. La afección pulmonar puede ocurrir como hipertensión o enfermedad pulmonar intersticial. La meta del tratamiento es detener el deterioro de la función pulmonar. Objetivo: Caracterizar las manifestaciones clínicas, imagenológicas y la función respiratoria en pacientes con esclerosis sistémica y enfermedad pulmonar intersticial. Métodos: Se realizó un estudio observacional, descriptivo de corte transversal en el período comprendido entre diciembre de 2018 y diciembre de 2019. En el Servicio de Reumatología para caracterizar la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. El universo estuvo constituido por 168 pacientes, diagnosticados con esa enfermedad y la muestra se conformó por 55 pacientes que cumplieron los criterios de inclusión establecidos. Resultados: La enfermedad pulmonar intersticial fue más frecuente en pacientes mayores de 40 años, del sexo femenino, piel mestiza, predominó la forma clínica difusa, el síntoma más frecuente fue la disnea de esfuerzo, la mayoría tuvo ANA positivo y el patrón tomográfico en panal de abejas. La capacidad vital forzada estaba disminuida con mayor frecuencia, se asoció a un comportamiento autoinmune positivo para anti-ScL-70. Conclusiones: Se caracterizó las manifestaciones clínicas y radiográficas de la enfermedad pulmonar intersticial fueron comprobadas por la utilidad de la tomografía computarizada y la espirometría para identificar la presencia de fibrosis pulmonar(AU)

Introduction: Systemic sclerosis is a chronic autoimmune disease, characterized by vasculopathy, activation of the immune system and increased extracellular matrix deposits. In recent years, lung involvement has gained great importance, it has become the first cause of death in these patients. Lung involvement can occur as hypertension or interstitial lung disease. The goal of treatment is to stop the decline in lung function. Objective: To characterize the clinical and imaging manifestations and respiratory function in patients with systemic sclerosis and interstitial lung disease. Methods: An observational, descriptive, cross-sectional study was carried out from December 2018 to December 2019 in the rheumatology service to characterize interstitial lung disease in patients with systemic sclerosis. The universe consisted of 168 patients diagnosed with this disease and the sample was made up of 55 patients who met the established inclusion criteria. Results: Interstitial lung disease was more frequent in patients older than 40 years, female, mixed-race skin color, the diffuse clinical form predominated, the most frequent symptom was exertional dyspnea, the majority had positive ANA and the pattern honeycomb tomography. Forced vital capacity was more frequently decreased, associated with positive autoimmune behavior for Anti-ScL-70. Conclusions: The radiographic and clinical manifestations of PID were verified by the usefulness of computed tomography and spirometry to identify the presence of pulmonary fibrosis(AU)

Spirometry assessment of interstitial lung disease patients and correlation with its clinical and radiological profile

Background: Interstitial lung diseases (ILD) are a diverse set of lower respiratory tract illnesses that are defined by both abrupt and persistent inflammation as well as a largely irreversible and continuous progression of fibrosis in the interstitium and the walls of the alveoli. This study focuses on non-invasive techniques for clinically and radiographically confirmed situations. Since most patients refuse surgical or transbronchial lung biopsies and are in respiratory difficulty, an alternate method is preferable. Spirometry tests are often used as diagnostic aids. This study compares spirometry in ILD patients with their radiological and clinical features. Methods: In this prospective observational study, 50 ILD patients who were diagnosed on the clinical and radiological grounds included. A detailed history of illness was obtained and noted. All patients were examined clinically and underwent basic investigations. All patients were performed 6 MWT O2 saturation and spirometry. Correlation between spirometry findings and clinical and radiological profile was done. Results: The study group of 50 patients with ILD, idiopathic pulmonary fibrosis was the most common cause of ILD consists of 32 patients performing 64% of the study group. Average duration of symptoms in ILD patients in this study was 5.46±5.49 months. The mean age of the patients was 61.58±12.92 years ranging from 27 to 88 years with 27 (54%) male and 23 (46%) female. Cough and dyspnoea were the most common features at presentation in our study group, present in almost all the patients. Crepitations were present in 41 (82%) patients. Most common chest X-ray feature was reticular opacities which was present in 24 (48%) patients. Ground glass opacity in high-resolution computed tomography (HRCT) was seen in 33 (66%) patients. Most common spirometry pattern seen in our study was Restrictive pattern which was present in 42 patients. In ILD patients, mean values of FEV1% was 59.2±20.33, FVC% was 60.76±25.45, FEV1/FVC was 100.86±20.12. Conclusions: Idiopathic pulmonary fibrosis is the most common and chronic hypersensitivity pneumonitis along with cryptogenic organizing pneumonia are the second common ILD in our study. The underdiagnosis of interstitial lung disease is due to a lack of knowledge among doctors. Therefore, spirometry and 6MWT O2 saturation should be performed in all patients presented with complaints of chronic cough and breathlessness as screening tool and then HRCT chest and biopsy can be done for confirmation of diagnosis. So early diagnosis and treatment of ILD patients is possible with use of spirometry.

Treatment of Connective Tissue Diseases-associated Interstitial Lung Disease by the Origin-restoring and Bi （痹）-alleviating Method / 中医杂志

It is believed that the fundamental pathogenesis of the connective tissue diseases-associated interstitial lung disease （CTD-ILD） is kidney essence deficiency， with lung collateral obstruction throughout the disease， and environmental toxin pathogen is the important causative factors for the development of CTD-ILD. This article proposed to restore origin and alleviate bi （痹） for CTD-ILD， for which restoring origin means tonifying the lungs， spleen and kidneys to bank up the roots and consolidate the original qi， with modified Erxian Decoction （二仙汤） plus Liu Junzi Decoction （六君子汤）； alleviating bi means expelling wind and dredging collaterals， and eliminating the mass to restore the smoothness of the lung collaterals， with paired medicines of Chuanshanlong （Dioscorea nipponica）-Dilong （Kalanchoe pinnata）， Vinegar-processed Sanleng （Sparganium stoloniferum）-Vinegar-processed Ezhu （Curcuma zedoaria）， and stem-type medicines， and emphasized on removing the environmental toxin pathogens to facilitate the recovery of healthy qi.

A concise review of diagnosis and evaluation of interstitial lung abnormalities / 中华劳动卫生职业病杂志

Interstitial lung abnormalities (ILAs) refer to the subtle or mild signs of ILAs pulmonary parenchyma on chest HRCT scans, which are not yet sufficient to diagnose a certain interstitial lung disease, may be potentially compatible an early stage of the diseases. The signs of ILAs usually includes ground-glass opacities, reticular abnormakicies, honeycombing, traction bronchiectasis or non-emphysematous cysts. This article reviews the research progreses in the definition and classification, risk factors, prognosis, comorbidities and management of ILAs in combination with domestic and foreign literatures.

Genome-wide DNA methylation and transcriptome expression profiles of peripheral blood mononuclear cells in patients with systemic sclerosis with interstitial lung disease / 中南大学学报(医学版)

OBJECTIVES@#This study aims to investigate the genome-wide DNA methylation and transcriptome expression profiles of peripheral blood mononuclear cells (PBMCs) in patients with systemic sclerosis (SSc) with interstitial lung disease (ILD), and to analyze the effects of DNA methylation on Wnt/β-catenin and chemokine signaling pathways.@*METHODS@#PBMCs were collected from 19 patients with SSc (SSc group) and 18 healthy persons (control group). Among SSc patients, there were 10 patients with ILD (SSc with ILD subgroup) and 9 patients without ILD (SSc without ILD subgroup). The genome-wide DNA methylation and gene expression level were analyzed by using Illumina 450K methylation chip and Illumina HT-12 v4.0 gene expression profiling chip. The effect of DNA methylation on Wnt/β-catenin and chemokine signal pathways was investigated.@*RESULTS@#Genome-wide DNA methylation analysis identified 71 hypermethylated CpG sites and 98 hypomethylated CpG sites in the SSc with ILD subgroup compared with the SSc without ILD subgroup. Transcriptome analysis distinguished 164 upregulated genes and 191 downregulated genes in the SSc with ILD subgroup as compared with the SSc without ILD subgroup. In PBMCs of the SSc group, 35 genes in Wnt/β-catenin signaling pathway were hypomethylated, while frizzled-1 (FZD1), mitogen-activated protein kinase 9 (MAPK9), mothers against DPP homolog 2 (SMAD2), transcription factor 7-like 2 (TCF7L2), and wingless-type MMTV integration site family, member 5B (WNT5B) mRNA expressions were upregulated as compared with the control group (all P<0.05). Compared with the SSc without ILD subgroup, the mRNA expressions of dickkopf homolog 2 (DKK2), FZD1, MAPK9 were upregulated in the SSc with ILD subgroup, but the differences were not statistically significant (all P>0.05). In PBMCs of the SSc group, 38 genes in chemokine signaling pathway were hypomethylated, while β-arrestin 1 (ARRB1), C-X-C motif chemokine ligand 10 (CXCL10), C-X-C motif chemokine ligand 16 (CXCL16), FGR, and neutrophil cytosolic factor 1C (NCF1C) mRNA expressions were upregulated as compared with the control group (all P<0.05). Compared with the SSc without ILD subgroup, the mRNA expressions of ARRB1, CXCL10, CXCL16 were upregulated in the SSc with ILD subgroup, but the differences were not statistically significant (all P>0.05).@*CONCLUSIONS@#There are differences in DNA methylation and transcriptome profiles between SSc with ILD and SSc without ILD. The expression levels of multiple genes in Wnt/β- catenin and chemokine signaling pathways are upregulated, which might be associatea with the pathogenesis of SSc.

Traditional Chinese Medicine Syndrome of Primary Sjögren's Syndrome Patients with Interstitial Lung Disease / 中国实验方剂学杂志

ObjectiveTo summarize the characteristics of traditional Chinese medicine （TCM） syndrome in primary Sjögren's syndrome （pSS） patients with interstitial lung disease （ILD） and to explore associated factors. MethodA survey was conducted and pSS patients who were treated in TCM department of rheumatism at China-Japan Friendship Hospital from December 2018 to April 2022 were included. Tongue manifestations and syndromes of patients were recorded. pSS patients with ILD were classified into the pSS-ILD group and those without the ILD were included in the pSS-non-ILD group. The tongue manifestations， syndromes， and laboratory indexes were compared between the two groups， and logistic regression was used to explore the factors associated with pSS-ILD. ResultA total of 200 pSS patients were included， with 186 （93.0%） females， median age of 57 years， and median disease course of 60 months， of which 44 （22%） had pSS-ILD. In terms of tongue manifestations， pSS-ILD patients generally had dark/purple/stasis tongue， fissured tongue， and tongue with little fluid， thick coating， yellow coating， and greasy coating. The proportion patients with yellow coating was higher in pSS-ILD group than in the pSS-non-ILD group （χ2=4.799，P<0.05）. In terms of syndrome， more than 40% of pSS-ILD patients had Qi deficiency， Yin deficiency， phlegm-dampness， Qi stagnation， and/or blood stasis syndrome. As for Yin deficiency， liver-kidney Yin deficiency syndrome ranked the first. For Qi deficiency， lung Qi deficiency syndrome was most commonly seen. The proportion of patients with lung Qi deficiency was higher in the pSS-ILD group than in the pSS-non-ILD group （χ2=18.667，P<0.01）. As to laboratory indexes， compared with the pSS-non-ILD group， pSS-ILD group had high proportion of anti-SSA-positive patients （P<0.05） and high levels of C-reactive protein （CRP） （P<0.01）， complement C3 （χ2=4.332，P<0.05）， and complement C4 （P<0.05）. Logistic regression analysis showed that pSS with ILD was positively associated with lung Qi deficiency ［odds ratio （OR）=6.079， 95% confidence interval （CI） 2.585-14.298， P<0.01）］ and yellow coating （OR=5.260， 95% CI 1.337-20.692， P<0.05） and negatively associated with low C4 （OR=0.199， 95% CI 0.070-0.564， P<0.01）. ConclusionAbout 22% of pSS patients had ILD， and patients with pSS-ILD generally have Qi deficiency， Yin deficiency， phlegm-dampness， Qi stagnation， and/or blood stasis syndrome. Yellow coating， lung Qi deficiency and C4 level are factors associated with pSS combined with ILD.

Correlation between dyslipidemia and rheumatoid arthritis associated interstitial lung disease / 北京大学学报(医学版)

OBJECTIVE@#To study the correlation between dyslipidemia and rheumatoid arthritis associa-ted interstitial lung disease (RA-ILD) by retrospective analysis of the clinical data.@*METHODS@#The clinical data of patients with rheumatoid arthritis (RA), who were hospitalized in the Department of Rheumatism and Immunology of Peking University Shenzhen Hospital from January 2015 to July 2020 and fulfilled the criteria of the 2010 Rheumatoid Arthritis Classification Criteria established by American College of Rheumatology/European League Against Rheumatism collaborative initiative, were collected and analyzed.@*RESULTS@#There were 737 RA patients included, of whom 282(38.26%)were with interstitial lung disease (ILD). The median time from the onset of the first RA-related clinical symptoms to the onset of ILD was 13 years (95%CI 11.33-14.67). By multivariate Logistic regression analysis, we found that low-density lipoprotein cholesterol (LDL-C) was an independent risk factor for RA-ILD (OR 1.452, 95%CI 1.099-1.918, P=0.009), whereas high-density lipoprotein cholesterol (HDL-C) was a protective factor for RA-ILD (OR 0.056, 95%CI 0.025-0.125, P < 0.001). The RA patients with high LDL-C or low HDL-C had higher incidence of ILD than that of the RA patients with normal LDL-C or HDL-C(57.45% vs. 36.96%, P < 0.001; 47.33% vs. 33.81%, P < 0.001, respectively). The median time of ILD onset in the RA patients with low HDL-C was shorter than that of the RA patients with normal HDL-C [10.0(95%CI 9.33-10.67)years vs.17.0 (95%CI 14.58-19.42) years, P < 0.001]. HDL-C level was negatively correlated with disease activity. Among the RA-ILD patients, the patients with low HDL-C had higher percentage of usual interstitial pneumonia (UIP) then that of the patients with normal HDL-C (60.00% vs. 53.29%, P=0.002). The RA-ILD patients with high LDL-C had higher incidence rate of decrease in forced vital capacity (FVC) than that of the RA-ILD patients with normal LDL-C (50.00% vs. 21.52%, P=0.015). The RA-ILD patients with low HDL-C had higher incidence rate of decrease in FVC (26.92% vs. 16.18%, P=0.003) and carbon monoxide diffusion (80.76% vs. 50.00%, P=0.010) than that of RA-ILD patients with normal HDL-C.@*CONCLUSION@#LDL-C was possibly a potential independent risk factor for RA-ILD. HDL-C was possibly a potential protective factor for RA-ILD. HDL-C level was negatively correlated with disease activity of RA. The median time of ILD onset in the RA patients with low HDL-C was significantly shorter than that of the RA patients with normal HDL-C.