Clinical and Autoimmune Profile of Scleroderma Patients from Western India

Abstract

Background. Systemic sclerosis (SSc, scleroderma) is a disorder characterized by fibrosis of skin and visceral organs. Pathogenesis of scleroderma is complex and is incompletely understood as yet. Autoantibodies in SSc represent a serologic hallmark which have clinical relevance, with diagnostic and prognostic potential. Objectives. To study distribution of clinical manifestations and to identify frequency of autoantibodies among subtypes of scleroderma patients from Western India. Methodology. One hundred and ten scleroderma patients were clinically classified according to the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria. All these patients were in active stage of disease. Clinical manifestations were recorded at the time of presentation. Autoantibodies were tested in them by indirect immunofluorescence test and ELISA. Immunoglobulin levels were estimated by nephelometer. These parameters were further correlated with clinical presentation of the disease. Results. Scleroderma patients had M : F ratio of 1 : 10 where mean age at evaluation was 34.7 ± 10.7 years and a mean disease duration was 43.7 ± 35 months. Clinical subtypes showed that 45 patients (40.9%) had diffused cutaneous (dcSSc) lesions, 32 patients (29.1%) had limited cutaneous (lcSSc) lesions, and 33 patients (30%) had other autoimmune overlaps. The overall frequency of ANA in SSc patients studied was 85.5%. The frequency of anti-Scl70, anti-centromere, anti-endothelial cell antibodies (AECA), and anti-keratinocyte antibodies (AKA) was 62.7%, 22.7%, 30%, and 40.9%, respectively. Anti-Scl70 antibodies were significantly high (75.6% versus 46.9%) among dcSSc patients (P < 0.0115) whereas anti-centromere antibodies were significantly high (9% versus 38%) among lcSSc patients when these two subtypes were compared (P < 0.0044). Conclusion. This study supports that there are geoepidemiological variations among scleroderma patients for their clinical presentation, autoantibody profile, and immune parameters across the country.

Characteristics of patients with systemic sclerosis living in Qatar

“…Such a high frequency of Raynaud's phenomenon has been similarly reported in other cohorts, e.g., in 97.3% of Egyptian patients, 95% of Iranian patients, and 76.5% of Indian patients. 15–17…”

Systemic Sclerosis Patients With Negative Antinuclear Antibodies Have Distinctive Clinical Manifestations: a Multicenter CRDC&nbsp;Cohort in China

“…Detection of SSc-speci c ANAs is not only bene cial for diagnosis but is also clinically useful for classifying SSc subtypes that are exclusively associated with characteristic clinical phenotypes [11]. According to the study of Cristinane et al on an African-Brazilian population [12], anti-Scl-70 was present in 38.1% of patients with dcSSc and 25.0% of patients with lcSSc; in another cohort study, ACA was present in 9% of dcSSc patients and 38% of lcSSc patients [3]. Bardoni and colleagues [13] indicated that 7.8% of SSc patients had anti-RNP III antibodies.…”

“…The occurrence of anti-Scl-70 antibodies is a marker of more extensive skin brosis and clinically signi cant pulmonary brosis, which predicts a poor prognosis. Conversely, anti-centromere antibodies are typically associated with localized cutaneous systemic sclerosis (lcSSc), infrequent lung, heart and kidney involvement, and late onset of pulmonary arterial hypertension (PAH), representing an overall good prognosis [3].…”

New Classification Criteria for Systemic Sclerosis (Scleroderma)

“…25 This finding is also likely true for other regions where the distribution between lcSSc and dcSSc and autoantibodies may be different from North America and Europe. [26][27][28][29][30][31] Abbreviations: 1, positive; Ab, antibody; ANA, antinuclear antibody; ILD, interstitial lung disease; PAH, pulmonary arterial hypertension; WBC, white blood cell count. a A score of 9 or more is necessary to classify SSc.…”