Spectrum of Clinical Manifestations in Patients of Scleroderma and Correlation between Cutaneous and Pulmonary Involvement.

Abstract

Purpose: Our research aimed at characterizing the range of signs and symptoms observed in scleroderma patients at a tertiary care hospital and investigating potential correlations between pulmonary and cutaneous involvement.

Materials and methods: We obtained informed consent from scleroderma patients for conducting a comprehensive clinical assessment, including detailed medical histories and physical examinations. Standard diagnostic investigations like complete blood counts, erythrocyte sedimentation rate, renal function, liver function, electrocardiograms (ECG), high-resolution computed tomography (HRCT) scans, and pulmonary function tests were performed. The modified Rodnan skin score (mRSS) was used to assess skin involvement.

Results: About 74 (71.2%) patients had diffuse cutaneous systemic scleroderma (dcSSc), while 30 (28.8%) patients had limited cutaneous scleroderma (lcSSc). 96 (92.3%) patients had Raynaud's phenomenon. About 68 (65.4%) patients had mild mRSS, while 26 (25%) and 10 (9.6%) patients had moderate and severe mRSS, respectively. Skin tightening (88.4%) and sclerodactyly (88.4%) were the most common cutaneous manifestations, followed by digital ulcers and pits (57.7%), diffuse edema of hands and feet (38.4%), salt-and-pepper skin (38.4%), calcinosis (30.8%), telangiectasia (25%), and contractures (19.2%). Pulmonary manifestations showed interstitial lung disease (ILD) in 62 (59.6%) patients and pulmonary hypertension (PH) in 14 (13.5%) patients. 8 (7.7%) patients had ILD with PH. 48 (46.2%) and 18 (17.3%) patients with dcSSc had mild and moderate mRSS, respectively, while 8 (7.7%) patients had severe mRSS. About 20 (19.2%) and 8 (7.7%) patients with lcSSc had mild and moderate mRSS, respectively, while 2 (1.9%) patients had severe mRSS. There was no significant correlation of mRSS and subtypes of scleroderma patients. The mean mRSS score in ILD was low in comparison to the mRSS score in patients with PH (25.7 ± 8.90 vs 28.9 ± 7.62). There was a significant correlation of mRSS and pulmonary involvement as indicated by the Student's t-test (p < 0.05).

Conclusion: In systemic sclerosis (SSc) patients, the emergence of severe systemic complications such as pulmonary arterial hypertension and ILD can manifest regardless of disease duration or subtype. Symptoms might not consistently align with disease advancement. Therefore, a thorough evaluation that incorporates symptoms and specialized diagnostic tests, such as pulmonary function tests, echocardiography (ECHO), and HRCT, is crucial for early identification, timely treatment, and better prognosis. The mRSS serves as a valuable clinical instrument for monitoring scleroderma progression.