Pulmonary Manifestations in Systemic Sclerosis: Hospital-Based Descriptive Study

Ravindrachari Mulkoju; Vinod Kumar Saka; Manju Rajaram; Rashmi Kumari; Vir S Negi; Madhusmita Mohanty Mohapatra; Vishnukanth Govindaraj; Dharm Prakash Dwivedi; Vemuri Mahesh Babu

doi:10.7759/cureus.8649

Pulmonary Manifestations in Systemic Sclerosis: Hospital-Based Descriptive Study

Cureus. 2020 Jun 16;12(6):e8649. doi: 10.7759/cureus.8649.

Authors

Ravindrachari Mulkoju¹, Vinod Kumar Saka¹, Manju Rajaram¹, Rashmi Kumari², Vir S Negi³, Madhusmita Mohanty Mohapatra⁴, Vishnukanth Govindaraj¹, Dharm Prakash Dwivedi¹, Vemuri Mahesh Babu⁴

Affiliations

¹ Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND.
² Dermatology, Jawaharlal Institute of Post Graduate Medical Education and Research, Puducherry, IND.
³ Internal Medicine: Rheumatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND.
⁴ Internal Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, IND.

Abstract

Introduction Prevalence of systemic sclerosis (SSc)-related organ injury is difficult to estimate as it occurs early in SSc, even though patients are often asymptomatic. As the patients with organ damage have a poor prognosis, all the patients should be carefully evaluated and followed‑up in the initial periods. This facilitates the early identification and initiation of appropriate therapy. This study emphasizes on different clinical manifestations and early predictors of lung involvement by using clinical, radiological, and pulmonary function tests in a tertiary care centre. Materials and methods A total of 53 SSc cases, who satisfied American College of Rheumatology (ACR) 2013 criteria, without any overlap syndromes were included in the study. All patients underwent thorough clinical examination along with Modified Rodnan Scoring (MRS) assessment, nailfold capillaroscopy (NFC), chest X-ray (CXR), HRCT thorax, 2D-echocardiography, spirometry and diffusion lung study by carbon monoxide (DLco). Results Out of 53 patients, four were male and 49 were female. Twenty-one patients had limited SSc (lcSSc) and 32 had diffuse SSc (dcSSc). Eighty-three per cent of subjects presented with skin manifestations and 34% with respiratory complaints. Reticulonodular opacities and ground glassing were the predominant radiological abnormalities suggestive of non-specific interstitial pneumonia (NSIP) followed by usual interstitial pneumonia (UIP). Pulmonary hypertension was predominant in patients with lcSSc. Thirty-eight patients had a restrictive pattern of spirometry. Forty-four patients showed deranged DLco, among which two patients showed an isolated decrease in DLco. Thirty-seven patients had abnormal NFC among which dropout pattern was predominant. MRS was significantly correlated with pulmonary involvement by DLco and HRCT. Conclusions SSc can affect the lungs even before developing obvious clinical pulmonary manifestations. DLco and HRCT play a critical role in detecting early lung involvement and predicting the outcomes in SSc. Higher modified Rodnan's score, which has a significant correlation with DLco and HRCT can be used to predict early visceral involvement in resource-limited settings.

Keywords: carbon monoxide diffusion capacity; high resolution computed tomography; interstitial lung disease; modified rodnan scoring; nailfold capillaroscopy; pulmonary involvement; systemic sclerosis.