A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

Neha T. Solanki, Sahana P. Raju, Deepmala Budhrani, Bharti K. Patel


Background: The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.

Methods: The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.

Results: The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc)  and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.

Conclusions: AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.


Auto-immune connective tissue diseases, High-resolution computed tomography, Pulmonary function

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Schwartz MI. Pulmonary manifestations of the collagen vascular diseases. In: Fishman AP, Elias JA, Fishman JA, Grippi MA, Kaisar LR, Senior RM, editors. Fishman’s Pulmonary Diseases and Disorders. New York: McGraw-Hill; 1998: 1115-1132.

Memet B, Ginzler EM. Pulmonary manifestations of systemic lupus erythematosus. Semin Respir Crit Care Med. 2007;28(4):441-50.

Lamblin C, Bergoin C, Saelens J, Wallaert B. Interstitial lung disease in collagen vascular diseases. Eur Respir J. 2001;18(Suppl.32):695-805.

Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of systemic lupus international collaborating clinics (SLICC) classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64:2677-86.

Alarcón-Segovia D, Villarreal M. Classification and diagnostic criteria for mixed connective tissue disease. In: Kasukawa R, Sharp GC, eds. Mixed connective tissue disease and anti-nuclear antibodies. Amsterdam: Elsvier Science Publishers B.V. (Biomedical Division); 1987: 33-40.

Arakkal G, Chintagunta S. Cardio-pulmonary involvement in systemic sclerosis: a study at a tertiary care center. Indian J Dermatol Venerol Leprol. 2017;83:677-82.

Yeison S, Julian S. Acute respiratory involvement in Colombian patients with systemic lupus erythematosus undergoing chest computed tomography. Int J Rheumatol. 2019;22(10):1825-31.

Assayag D, Ryerson CJ. Determining respiratory impairment in connective tissue disease-associated interstitial lung disease. Rheum Dis Clin North Am. 2015;41:213-23.

Ferri C, Manfredi A, Sebastiani M, Colaci M, Giuggioli D, Vacchi C, et al. Interstitial pneumonia with autoimmune features and undifferentiated connective tissue disease: our interdisciplinary rheumatology-pneumology experience, and review of the literature. Autoimmun Rev. 2016;15:61-70.

Ryerson CJ, Collard HR. Update on the diagnosis and classification of ILD. Curr Opin Pulm Med. 2013;19:453-9.

Wells AU, Steen V, Valentini G. Pulmonary complications: one of the most challenging complications of systemic sclerosis. Rheumatol. 2009;48(suppl 3):iii40-iii44.

Patkar NM, Teng GG, Curtis JR, Saag KG. Association of infections and tuberculosis with antitumor necrosis factor alpha therapy. Curr Opin Rheumatol. 2008;20:320-6.