Clinical profile of patients with interstitial lung disease in connective tissue disorders – An original study

Abhishek Nuchin, Girija Nair, Balaji Tuppekar, Shahid Patel, Abhay Uppe

Abstract


Background and Objectives: Interstitial Lung Diseases (ILD’s) are a heterogeneous collection of more than one distinct lung disorder which is grouped together owing to their clinical, radiographic, and pathologic features they share in common. Connective tissue disease may be an underlying cause of
Interstitial Lung Disease, and often patients may not present with a pre-existing diagnosis. The aim of this study is to study the clinical profile of these patients including physiologic testing and have a better understanding of CTD-ILD in the Indian scenario.
Material and Methods: This was a prospective, observational study conducted at D.Y.Patil Hospital and Medical Research Center, Navi Mumbai, India, between a two year period from December 2017-December 2019. A total of 50 patients of age 18 and above with clinical diagnosis of connective tissue disease and interstitial lung disease were included in the study. Clinical profile of the study participants was studied based on presenting symptomatology and history, chest x-ray and HRCT findings, microbiological aspirates in sputum, pulmonary function testing, 6MWT and 2D-Echo findings. The convenient sampling
method was used for data collection and distribution of responses was examined using frequencies and percentages. Further analysis was done using appropriate statistical tests (Chi-square test, t-test) were used to compare responses between various subgroups. 
Results: Majority of the study subjects were females (56%) as compared to males (44%). Mean age of the study participants was 58.08 11.92 years, ranging from 27 years to 81 yrs. Majority were of the age group 61-70 years(21%), followed by 51-60 years(16%) respectively. All the participants (100%) presented with dyspnea, more than half of them 28(56%) had cough and over a quarter of them had joint pain 15(30%).17(34%) symptomatic patients of CTD were also diagnosed as ILD. 33(66%) of patients with ILD had underlying CTD. 66% patients of ILD had underlying Connective Tissue Disease. UIP pattern was the predominant in 88% of RA-ILD and NSIP pattern was predominant in other CTD-ILD. Restrictive abnormality on PFT with
reduced DLCO was observed in all patients with raised pulmonary artery pressure in 70% patients. During the 6-minute walk test, 64% of patients walked <350 meters and significantly reduced SpO2 levels were observed after exercise P<0.05.
Conclusion: Our study showed a high frequency of lung involvement in the form of respiratory symptoms, radiological patterns, echocardiography, and changes in pulmonary function and exercise testing in patients with ILD diagnosed to have underlying CTD. Since lung involvement in CTD is associated with significant morbidity and mortality, a high index of suspicion in ILD patients with underlying connective tissue disease will help in early diagnosis and treatment of CTD-ILD which is associated with a better prognosis compared
to other ILD’s. Further studies with a larger sample size and regular patient follow up are required for a better understanding of CTD-ILD in the Indian scenario.

Keywords: Interstitial Lung Diseases (ILD’s). Declaration of Helsinki (DoH).


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