Disease-associated Interstitial Lung Research Articles

Characteristics of patients with systemic sclerosis who develop interstitial lung disease

Systemic sclerosis associated-interstitial lung disease (SSc-ILD) show a high mortality. The factors associated SSc-ILD have shown variability in different populations. There are few studies in Mexican mestizos. To analyze the epidemiological, clinical and paraclinical factors associated with SSc-ILD. Cross-sectional study, where patients > 18 years of age with a diagnosis of SSc according to EULAR/ACR 2013 criteria and diagnosis of ILD by forced vital capacity (FVC) < 70% and > 5% of affected lung area on tomography were included. The strength of association of the factors for ILD was measured by odds ratio (OR) with 95% confidence intervals (95% CI). The significant variables were analyzed by multiple logistic regression with adjustment. Of the 80 patients with SSc, 38 (47.5%) had ILD. Risk factors such as smoking, higher activity index, lower FVC, anti-topoisomerase I antibodies, and protective factors such as a limited SSc, early capillaroscopic pattern, and anti-centromere antibodies (ACA) were associated with ILD. In the multivariate analysis, a higher activity index with OR 4.17, (95% CI 2.01-8.65) persisted as a risk factor, while ACA with OR 0.17, (95% CI 0.03-0.85) persisted as a protective factor associated with SSc-ILD. A higher index of activity and ACA persisted as factors associated with SSc-ILD.

Serum antibodies to peptidylarginine deiminase-4 in rheumatoid arthritis associated-interstitial lung disease are associated with decreased lung fibrosis and improved survival

ObjectiveInterstitial lung disease (ILD) is a leading cause of mortality in rheumatoid arthritis (RA), particularly in those with the usual interstitial pneumonia subtype (RA-UIP). Serum antibodies to peptidylarginine deiminase type 4 (anti-PAD4), particularly a subset that cross-react with PAD3 (PAD3/4XR), have been associated with imaging evidence of ILD. We aimed to determine the specificity of anti-PAD4 antibodies in RA-ILD and to examine associations with markers of ILD severity. Methods48 RA-ILD and 31 idiopathic pulmonary fibrosis (IPF) patients were identified from the National Jewish Health Biobank. RA-ILD subtype was defined by imaging pattern on high-resolution chest computed tomography (CT), and serum was tested for anti-PAD4 and anti-PAD3/4XR antibodies. Antibody prevalence, measures of ILD severity (% predicted forced vital capacity, FVC; % predicted diffusion capacity carbon monoxide, DLCO; quantitative CT fibrosis) and mortality were compared between groups. ResultsAnti-PAD4 antibodies were present in 9/48 (19%) subjects with RA-ILD and no subjects with IPF. Within RA-ILD, anti-PAD4 antibodies were found almost exclusively in RA-UIP (89%). Within RA-UIP subjects, % predicted FVC was higher in anti-PAD4+ subjects, and this finding was most strongly associated with anti-PAD3/4XR antibodies. In addition, quantitative CT fibrosis score was lower in anti-PAD4+ RA-UIP subjects, including those with mono-reactive anti-PAD4 antibodies and anti-PAD3/4XR antibodies. Anti-PAD4+ RA-UIP subjects also exhibited decreased mortality. ConclusionsWe demonstrate the presence of serum anti-PAD4 antibodies in a subset of patients with RA-UIP that were notably associated with better lung function, less fibrosis and decreased mortality.

Clinical features and outcomes of patients with myositis associated-interstitial lung disease.

Myositis associated interstitial lung disease (ILD) seems to be an under-recognized entity. In this multicenter, retrospective study, we recorded between 9/12/2019 and 30/9/2021 consecutive patients who presented in five different ILD centers from two European countries (Greece, France) and received a multidisciplinary diagnosis of myositis associated-ILD. The primary outcome was all-cause mortality over 1 year in specific subgroups of patients. Secondary outcomes included comparison of disease characteristics between patients diagnosed with the amyopathic subtype and patients with evidence of myopathy at diagnosis. We identified 75 patients with myositis associated-ILD. Median age (95% CI) at the time of diagnosis was 64.0 (61.0-65.0) years. Antinuclear antibody testing was positive in 40% of the cohort (n = 30/75). Myopathy onset occurred first in 40.0% of cases (n = 30), ILD without evidence of myopathy occurred in 29 patients (38.7%), while 16 patients (21.3%) were diagnosed concomitantly with ILD and myopathy. The commonest radiographic pattern was cellular non-specific interstitial pneumonia (NSIP) and was observed in 29 patients (38.7%). The radiographic pattern of organizing pneumonia was significantly more common in patients diagnosed with the amyopathic subtype compared to patients that presented with myopathy [24.1% (n = 7/29) vs. 6.5% (n = 3/46), p = 0.03]. One year survival was 86.7% in the overall population. Kaplan-Meier analysis demonstrated significantly higher all-cause 1-year mortality in patients with the amyopathic subtype compared to patients with evidence of myopathy [H R 4.24 (95% CI: 1.16-15.54), p = 0.03]. Patients diagnosed following hospitalization due to acute respiratory failure experienced increased risk of 1-year all-cause mortality compared to patients diagnosed in outpatient setting [HR 6.70 (95% CI: 1.19-37.81), p = 0.03]. Finally, patients with positive anti-MDA5 presented with higher 1-year all-cause mortality compared to anti-MDA5 negative patients [HR 28.37 (95% CI: 5.13-157.01), p = 0.0001]. Specific ILD radiographic patterns such as NSIP and organizing pneumonia may herald underlying inflammatory myopathies. Hospitalized patients presenting with bilateral organizing pneumonia refractory to antibiotics should be meticulously evaluated for myositis associated-ILD even if there is no overt muscular involvement. Incorporation of ILD radiological patterns in the diagnostic criteria of inflammatory myopathies may lead to timely therapeutic interventions and positively impact patients' survival.

Does avian antigen-induced chronic hypersensitivity pneumonitis lead to more severe PH than other causes of the same?

Sir, Chronic hypersensitivity pneumonitis (CHP) is a prominent aetiology of diffuse parenchymal lung disease (DPLD), contributing to over 47% of the inclusions in the ILD-India registry.[1] The diagnosis of CHP is difficult because the pathological description needs lung biopsy that remains mostly undone. The high-resolution computed tomography (HRCT) chest, however, can give some definite clues for the possibility.[2] In our series, the HRCT diagnosis of HP was done by a pulmonologist and a radiologist on an agreement for the probability of HP in their independent interpretation of HRCT chest pictures. The radiologically possible-HP diagnosis was supported by positive IgG precipitin titre for avian antigen by immunoCAP method.[3] A precipitin titre value >30 mg mgA/L was taken as significant.[3] The clinico-radio-serological positivity was further supported by history of exposure to pigeons/birds to build up a nearly definite avian antigen–induced diagnosis of CHP. However, the possible CHP cases from other causes could not be evaluated serologically as the panel with common antigens was not available to us at that point of time. The study did not essentially include subjects with features of CHP without any etiological confirmation and exclude other causes like connective tissue disease associated-interstitial lung disease (CTD-ILD) and sarcoidosis. The latter was done by a thorough clinical exercise (noting the American Rheumatology Association [ARA]) criteria for the common CTDs supported by the relevant serological tests whenever possible in the real-world situation. Sarcoidosis was diagnosed with demonstration of non-ceasing granuloma with exclusion of tuberculosis. All the patients underwent Doppler echo-cardiography by a single dedicated non-invasive cardiologist who looked systematically for evidence of pulmonary hypertension (PH) in addition to other parameters. Furthermore, we looked for the presence of clinical and radiological XRay -posterio-anterior (PA) view (chest X-ray [PA view] and HRCT) features of PH to satisfy the clinico-radio-echocardiographic screening criteria of the institute for the presence of PH.[4] We had 95 cases of suspected HP included in the evaluation from 2018 to 2019. These patients were sub-divided into two groups - CHP caused due to exposure to avian antigen and CHP likely from other possible etiologies. The above two groups were thus compared on the basis of demographic, spirometric, functional health status and echo-cardiographic variables [Table 1].Table 1: Demographic, spirometric, and functional health status and echocardiographic parameters between the two groups of chronic hypersensitivity pneumonitis (CHP) patientsThe results reflect that the two groups were similar on demographical and spirometric lung function variables, but the patients with CHP from avian antigen had significantly higher systolic pulmonary artery pressure (PAP). To appreciate the relative contribution of the variables for the difference between the two groups, we used the VIP plot that suggested the presence of PAP as the most effective discriminant between the two groups [Figure 1a]. However, the overlap between the clusters was very high, suggesting limited applicability of the information [Figure 1b]. In one of our previous observations, we found a very high frequency HRCT-chest that suggested PH in CHP patients induced by chronic exposure to avian antigen.[4] Such exposure of avian antigen has been found to be one of the common causes of CHP. Avian exposure was present in 21.4% of 513 HP patients in the ILD-India registry,[1] with the adjusted odds ratio of 3.52 (P < 0.001) for developing HP after exposure.[2] Pulmonary hypertension is found quite frequently in CHP[5] and is associated with poor survival prospect.[5] The subjects having CHP with history of exposure to avian antigen had a lesser but statistically insignificant mean duration of illness compared to the other group. The standard deviation in both the groups was very wide for the illness duration, making it unsuitable for interpretation through VIP plot [Figure 2]. Despite the weakness of having no biopsy-proven diagnosis of HP, a non-stringent exclusion of other aetiologies, non-inclusion of treatment history, and dearth of haemodynamic confirmation by right heart catheterisation, our observation demands attention. Furthermore, objective validation of our observation is, thus, warranted.Figure 1: (a) The VIP plot with the variables represents the relative contribution of the parameters to the variance between the study groups, (b) 2D score scatter plot of principal component analysis (PCA) to distinguish the two study groups [avian exposure related and other causes of CHP (chronic hypersensitivity pneumonitis)]showing a significant overlapFigure 2: Comparison of the disease duration between the two study groups [avian exposure related and other causes of CHP (chronic hypersensitivity pneumonitis)]Financial support and sponsorship The research was funded by the organization itself. Conflicts of interest There are no conflicts of interest.

Progressive fibrosing interstitial lung disease in rheumatoid arthritis: A retrospective study.

Rheumatoid arthritis associated-interstitial lung disease (RA-ILD) is the most common pulmonary manifestation of rheumatoid arthritis (RA) and an important cause of mortality. In patients suffering from interstitial lung diseases (ILD) from different etiologies (including RA-ILD), a significant proportion is exhibiting a fibrotic progression despite immunosuppressive therapies, defined as progressive fibrosing interstitial lung disease (PF-ILD). Here, we report the frequency of RA-ILD and PF-ILD in all RA patients' cohort at University Hospital of Liège and compare their characteristics and outcomes. Patients were retrospectively recruited from 2010 to 2020. PF-ILD was defined based on functional, clinical and/or iconographic progression criteria within 24 months despite specific anti-RA treatment. Out of 1,500 RA patients, about one third had high-resolution computed tomography (HRCT) performed, 89 showed RA-ILD and 48 PF-ILD. RA-ILD patients were significantly older than other RA patients (71 old of median age vs. 65, p < 0.0001), with a greater proportion of men (46.1 vs. 27.7%, p < 0.0001) and of smoking history. Non-specific interstitial pneumonia pattern was more frequent than usual interstitial pneumonia among RA-ILD (60.7 vs. 27.0%) and PF-ILD groups (60.4 vs. 31.2%). The risk of death was 2 times higher in RA-ILD patients [hazard ratio 2.03 (95% confidence interval 1.15-3.57), p < 0.01] compared to RA. We identified a prevalence of PF-ILD of 3% in a general RA population. The PF-ILD cohort did not seem to be different in terms of demographic characteristics and mortality compared to RA-ILD patients who did not exhibit the progressive phenotype yet.

An update on interstitial lung disease.

Interstitial lung diseases are a complex group of conditions that cause inflammation and scarring of the lung interstitium. This article discusses the diagnosis and management of common interstitial lung diseases including idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, connective tissue disease associated-interstitial lung disease, sarcoidosis and drug-induced interstitial lung disease. A multidisciplinary approach to diagnosis of interstitial lung disease is the gold standard; key history and examination features, blood panel, pulmonary function tests, high resolution computed tomography imaging, and when required bronchoalveolar lavage and lung biopsy results are discussed to reach a multidisciplinary consensus diagnosis. Advances, including the development of the disease-modifying anti-fibrotic medications nintedanib and pirfenidone, continue to shape the future management of interstitial lung disease. A holistic approach to the care of patients with interstitial lung disease is paramount, as they often have a high symptom burden and considerable palliative care needs.

Interstitial Lung Disease: A Rare Association with Adult-Onset Still’s Disease

Pulmonary lung involvement in adult-onset Still’s disease (AOSD) can be classified into two categories: with or without acute respiratory distress syndrome. Interstitial fibrosis in AOSD is rare, occurring in less than 5% of cases. Here, the authors present a case of a 40-year-old male of Asian descent with a past history of hyperthyroidism who presented with fever, shortness of breath associated with cough, sore throat, diffuse arthralgias, pink-coloured rash, and hepatomegaly. Laboratory investigations revealed leukocytosis, abnormal liver function tests, negative antinuclear antibodies, and negative rheumatoid factor. Chest X-ray showed bilateral basal infiltrates, while high-resolution CT chest scan confirmed the diagnosis of interstitial lung disease in association with ASOD. This case suggested a direct association of AOSD and interstitial lung disease, but autoimmune pathogenesis is the only link synchronising both diseases and the exact mechanism of direct involvement is ambiguous.

Idiopathic Interstitial Pneumonias (IIPs): Review of Clinical, Radiographic and High-Resolution Computed Tomography (HRCT)

Making a confident diagnosis is a complex task for a specific form of interstitial lung disease and providing appropriate management in an attempt to achieve normalization of the disease can put up an alarming process for the clinicians. A set of diffuse and restrictive lung diseases incorporate with idiopathic interstitial pneumonias, showing inflammation and fibrosis of the interstitium due to parenchymal damage. High-resolution computed tomography (HRCT) has magnified the diagnostic standpoint in stepwise identification and classified various patterns in the evaluation of interstitial lung disease. The aim of our review is to elaborate clinical, radiographic and typical and atypical HRCT findings of idiopathic interstitial pneumonias by correlating with its differential diagnosis. Idiopathic pulmonary fibrosis is the most predominant idiopathic interstitial pneumonias and its diagnosis needs to omit all other well-known causes of interstitial lung diseases. According to the 2011 evidence-based guidelines, usual interstitial pneumonia can be diagnosed by HRCT when all criteria are fulfilled. Non-specific interstitial pneumonia is distinguished by bilateral patchy ground-glass opacities and irregular linear/reticular opacities. Respiratory bronchiolitis associated-interstitial lung disease and desquamative interstitial pneumonia show centrolobular nodules and ground-glass opacities as imaging patterns. Cryptogenic organizing pneumonia consists of patchy peripheral or peribronchial consolidations, while ground-glass opacities with tendency for migration, which is evolving to fibrosis, in acute interstitial pneumonia. Lymphoid interstitial pneumonia and idiopathic pleuro-parenchymal fibroelastosis are classified under rare idiopathic interstitial pneumonias. HRCT images help radiologists in diagnosis and mapping specific patterns of idiopathic interstitial pneumonias. This article reviews the stages of evolution in HRCT features for idiopathic interstitial pneumonias.

Clinical and radiological features of idiopathic interstitial pneumonias (IIPs): a pictorial review.

ObjectivesTo illustrate the clinical and radiological features of idiopathic interstitial pneumonias (IIPs), according to the American Thoracic Society (ATS)/European Respiratory Society (ERS) classification updated in 2013.MethodsIIPs include a subset of diffuse and restrictive lung diseases, resulting from damage to the parenchyma characterised by inflammation and fibrosis of the interstitium. Classification into major and rare IIPs is based on the 2013 ATS/ERS committee.ResultsThe diagnosis of idiopathic pulmonary fibrosis (IPF) needs to exclude other well-known causes of interstitial lung diseases. According to the 2011 evidence-based guidelines, usual interstitial pneumonia (UIP) can be diagnosed by HRCT when all criteria are fulfilled. Non-specific interstitial pneumonia (NSIP) is characterised by patchy ground-glass opacities and irregular linear/reticular opacities. The imaging patterns of respiratory bronchiolitis associated-interstitial lung disease (RB-ILD) and desquamative interstitial pneumonia (DIP) show centrolobular nodules and ground-glass opacities. Cryptogenic organising pneumonia (COP) consists of patchy peripheral or peribronchial consolidations, while ground-glass opacities are typically associated with diffuse lung consolidation, evolving to fibrosis, in acute interstitial pneumonia (AIP). Rare IIPs include lymphoid interstitial pneumonia and idiopathic pleuro-parenchymal fibroelastosis (IPPFE).ConclusionsThe knowledge of IIP imaging features on HRCT images help radiologists in diagnosis. Moreover, the overlap of imaging features needs a multidisciplinary approach.Teaching Points• UIP findings are reticulations, bronchiectasis, honeycombing and absence of inconsistent features.• Bilateral patchy ground-glass areas represent the most encountered features in NSIP.• Poorly defined centrilobular nodules are typical of RB-ILD, whereas a ground-glass appearance is typical of DIP.• HRCT features of COP include characteristic peripheral or peribronchial patchy consolidations.• Rare IIPs include idiopathic LIP and idiopathic pleuro-parenchymal fibroelastosis (PPFE).

Revista del año de Archivos de Neumología en neumología intervencionista, enfermedades intersticiales y trasplante pulmonar

A review has been made of original articles on invasive pneumology techniques, interstitial diseases and lung transplantation, published in the Archivos de Bronconeumología during the year 2008. We have selected the publication by Martínez-Olondrins et al on the mediastinal staging of bronchogenic carcinoma by «blind» transbronchial needle aspiration to highlight the role of this simple, safe and cost-effective technique at a time when aspiration by ultrasound-guided bronchoscopy is profiled as an alternative to staging by mediastinoscopy. Besides its usefulness in the study of lymph nodes, transbronchial needle aspiration increases the overall performance of bronchoscopy by 20%, which means that it should be considered as a basic tool in the study of lung cancer. We also comment on the work by Galvis-Caravajal et al, who describe percutaneous radiofrequency as an alternative to radiotherapy in small lung or metastasic tumours. In diffuse interstitial disease, Morell et al analysed the diagnostic methods in 500 patients with this clinical-radiological presentation in which a definitive diagnosis was achieved in 85%, with 25% of them being obtained by non-invasively. Baloira et al analysed the characteristics of 19 patients with desquamative interstitial pneumonia and respiratory bronchiolitis associated-interstitial lung disease obtained from the National register of Interstitial Diseases.

Statins and Interstitial Lung Disease: A Systematic Review of the Literature and of Food and Drug Administration Adverse Event Reports

Objective To systematically review all published case reports and the US Food and Drug Administration adverse event reporting (FDA-AER) database to examine the relationship between statins and interstitial lung disease (ILD). Data sources PubMed (1987 to September 2007) and the FDA-AER database (as of June 2007) were searched for reports of ILD in which a statin was listed as a causative suspect. Review methods Two authors (one author for Pub Med cases and one for FDA-AER cases) independently abstracted patient data. Given the paucity of information, all case reports and case series in English and French were included. All adverse event reports from the FDA-AER database in which a statin was listed as causative suspect were included. Results The literature search using PubMed yielded eight articles describing a total of 14 case reports of ILD in association with statin use. The FDA-AER system database contained 162 cases of reported statin-induced ILD as of June 2007. For every 10,000 reports of a statin-associated adverse event, approximately 1 to 40 reports were for ILD. Conclusions Statin-induced ILD is a possible newly recognized side effect of statin therapy. The mechanism of lung injury is not defined. The current review provides novel information from the FDA-AER that supports a possible, although unusual, pulmonary class effect of statins. To systematically review all published case reports and the US Food and Drug Administration adverse event reporting (FDA-AER) database to examine the relationship between statins and interstitial lung disease (ILD). PubMed (1987 to September 2007) and the FDA-AER database (as of June 2007) were searched for reports of ILD in which a statin was listed as a causative suspect. Two authors (one author for Pub Med cases and one for FDA-AER cases) independently abstracted patient data. Given the paucity of information, all case reports and case series in English and French were included. All adverse event reports from the FDA-AER database in which a statin was listed as causative suspect were included. The literature search using PubMed yielded eight articles describing a total of 14 case reports of ILD in association with statin use. The FDA-AER system database contained 162 cases of reported statin-induced ILD as of June 2007. For every 10,000 reports of a statin-associated adverse event, approximately 1 to 40 reports were for ILD. Statin-induced ILD is a possible newly recognized side effect of statin therapy. The mechanism of lung injury is not defined. The current review provides novel information from the FDA-AER that supports a possible, although unusual, pulmonary class effect of statins.

Síndrome antisintetasa sin afectación muscular

Antisynthetase syndrome is a well defined syndrome characterized by the presence of interstitial lung disease in association with arthritis, miositis, mechanic's hands and Ruynaud's phenomenon in the presence of antisynthetase antibodies, especially Ac anti-Jo1. We described the case of a 68-year-old man with this syndrome in the absence of inflammatory muscle disease.

A Kindred of Children With Interstitial Lung Disease

Childhood interstitial lung disease (ILD) is a spectrum of diseases including many different rare lung conditions. We present a family with an unusual presentation of ILD in association with rheumatologic and immunologic abnormalities. Eight children with a common father were evaluated for evidence of lung disease in association with rheumatologic findings. All underwent routine history and physical examination, hematologic evaluation, and chest radiography and/or CT scan of the chest. Seven children underwent a more extensive immunologic evaluation. Those who were able underwent pulmonary function testing, and four children underwent lung biopsy. Six of eight children with a common father were found to have radiographic findings consistent with ILD. These children also had evidence of autoimmune disease with joint symptoms, alopecia, rheumatoid factor production, and hypergammaglobulinemia. Open-lung biopsy in four children revealed a spectrum of pulmonary lymphoid proliferations ranging from reactive lymphoid hyperplasia to lymphoid interstitial pneumonia. The findings of ILD and autoimmunity in a kindred of children suggest a novel genetic disorder of autosomal dominant pattern and variable penetrance. Although the precise pathogenesis remains unclear, these cases provide valuable insight into childhood ILD.

Antisynthetase Syndrome Without Muscle Involvement

Antisynthetase syndrome is a well defined syndrome characterized by the presence of interstitial lung disease in association with arthritis, miositis, mechanic's hands and Ruynaud's phenomenon in the presence of antisynthetase antibodies, especially Ac anti-Jo1. We described the case of a 68-year-old man with this syndrome in the absence of inflammatory muscle disease.

Interstitial lung disease in association with polymyositis-dermatomyositis: long-term follow-up CT evaluation in seven patients.

To determine the long-term follow-up computed tomographic (CT) findings of interstitial lung disease associated with polymyositis-dermatomyositis. CT scans in seven patients with interstitial lung disease and associated polymyositis-dermatomyositis were evaluated retrospectively. Six patients underwent sequential CT (follow-up range, 2-8 years; mean, 4.3 years). Histologic confirmation of pulmonary involvement was available in five patients. The predominant finding on the initial CT scans in four patients was subpleural consolidation, which corresponded to bronchiolitis obliterans organizing pneumonia with or without coexistent chronic eosinophilic pneumonia. In most cases, consolidation improved with use of corticosteroid and/or immunosuppressive therapy; in two patients, however, consolidation evolved into honeycombing. In one patient, diffuse areas of ground-glass opacity and consolidation appeared rapidly during illness; this patient died of sudden, rapid deterioration. In one patient with subpleural linear opacities, parenchymal abnormalities slowly progressed, and linear opacities had evolved into honeycombing at 8-year follow-up. In one patient with histologically proved organizing diffuse alveolar damage, bilateral patchy areas of ground-glass opacity and consolidation were seen. In one patient, subpleural bands changed to subpleural lines on sequential CT scans. CT provides an excellent demonstration of the lung changes in patients with interstitial lung disease and associated polymyositis-dermatomyositis.

Interstitial lung disease associated with polymyositis:response to cyclophosphamide and prednisolone combination treatment.

Polymyositis (PM) is a inflammatory connective tissue disease involving predominantly skeletal muscles, characterized by symmetrical, proximal muscle weakness, inflammation, and frequently, degeneration. Interstitial lung disease in association with PM occurs in 5~10% of cases and carries an especially grave prognosis. Although the cause of lung involvement in PM is not known, the underlying pathologic process in the lung is an immune mediated inflammation of alveolar structures, alveolitis. It is of interest, therefore, that cyclophosphamide, an immune modulating agent, has been reported to be effective in the treatment of PM. We report a case of corticosteroid resistant PM associated with interstitial lung disease, successfully treated with cyclophosphamide. A 37-year-old female was presented with 8 months duration of cough, exertional dyspnea, and muscle weakness. She had typical symptoms, physical findings, and elevated muscle enzyme levels in serum with characteristic findings of muscle biopsy. She also had typical interstitial lung disease pattern on chest X-ray and high resolution CT with restrictive pattern on pulmonary function test. The findings of transbronchial lung biopsy was compatible with interstitial lung disease. She failed to respond to corticosteroid initially. Subsequently steroids and cyclophosphamide were given with excellent clinical improvement.