Unique clinical and autoantibody profile of a large Asian Indian cohort of scleroderma—do South Asians have a more aggressive disease?
Aim and methods
A single-centre retrospective study was conducted using electronic medical records (EMR) of inpatients and outpatients with the diagnosis of “scleroderma” or “systemic sclerosis” visiting our clinic over the preceding 5 years.
A total of 327 patients’ charts met our selection criteria; 301 were females. The median (IQR (inter quartile range)) age at onset of first non-Raynaud’s symptom was 34.67 (27–43) years and median (IQR) disease duration prior to presentation to our department was 2.5 (1–5) years. Of these, 310 (94.8%) belonged to diffuse systemic sclerosis variety, 13 (4%) had limited systemic sclerosis, and 4 (1.2%) were of sine scleroderma type. A total of 289/302 (95.7%) patients were positive for ANA; of them, 245/327 (74.9%) were Scl-70 antibody-positive and 4% were CENP antibody-positive. Interstitial lung disease (ILD) was present in 288/327 (88.1%) patients. Among patients with available baseline forced vital capacity (FVC) data, 20% had a normal lung function and 28.4% had severe restriction. Pulmonary hypertension as assessed by echocardiography was present in 8.1% of patients. A significant association of Scl-70 antibody positivity with the presence of interstitial lung disease (ILD) (p = 0.000) and pulmonary hypertension (p = 0.035) was seen. On the other hand, presence of CENP antibody showed a protective trend against muscle weakness and/or muscle enzyme elevation (p = 0.052). Presence of arthritis was protective against development of digital ulceration (p = 0.021) and PAH (0.004). Patients younger than 40 years of age had significantly higher frequency of Scl-70 positivity (p = 0.038), whereas CENP antibody positivity was more likely in those aged > 40 years (p = 0.002).
Younger age of onset and high prevalence of Scl-70 antibody are unique South Asian features common with large Indian, Thai, and Chinese series. High prevalence of ILD is a feature common to Indian and Chinese series. Strong correlation of Scl-70 antibody with younger age and pulmonary hypertension were unique features of our cohort.
• Asian Indian scleroderma patients are younger by 2 decades compared to Caucasian series.
• Higher prevalence of Scl-70 antibody, its association with young age, interstitial lung disease and pulmonary hypertension are features of our cohort.
• High prevalence of interstitial lung disease (88.1%) was noted ; among those with baseline spirometry data (141/327), two thirds(66%) had moderate to severe restriction.
• Younger age at onset, higher prevalence of Scl-70 antibody are features common to other Indian, Thai and Chinese series.
KeywordsAnti-Scl-70 antibody Interstitial lung disease Pulmonary arterial hypertension Scleroderma Younger
Technical team involved in serology, echocardiography, radiology, and pulmonary function lab.
Compliance with ethical standards
- 2.Pradhan V, Rajadhyaksha A, Nadkar M, Pandit P, Surve P, Lecerf M, Bayry J, Kaveri S, Ghosh K (2014) Clinical and autoimmune profile of scleroderma patients from Western India. Int J Rheumatol 2014(983781):6 https://doi.org/10.1155/2014/983781
- 8.Nishioka K, Katayama I, Kondo H, Shinkai H, Ueki H, Tamaki K et al (1996) Epidemiological analysis of prognosis of 496 Japanese patients with progressive systemic sclerosis (SSc). Scleroderma Research Committee Japan. J Dermatol 23(JAPAN PT-Comparative Study PT-Journal Article PT-Research Support, Non-U.S. Gov’t LG-English DC-19970121 OVID MEDLINE UP 20151216):677–682CrossRefGoogle Scholar
- 10.Meier FMP, Frommer KW, Dinser R, Walker UA, Czirjak L, Denton CP et al (2012) Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 71(8):1355–1360. https://doi.org/10.1136/annrheumdis-2011-200742 CrossRefPubMedGoogle Scholar
- 16.Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Pölzleitner D, Burghuber OC, Tscholakoff D (1990) Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 176(3):755–759. https://doi.org/10.1148/radiology.176.3.2389033 CrossRefPubMedGoogle Scholar
- 18.Foocharoen C, Suwannachat P, Netwijitpan S, Mahakkanukrauh A, Suwannaroj S, Nanagara R (2016) Clinical differences between Thai systemic sclerosis patients with positive versus negative anti-topoisomerase I. Int J Rheum Dis 19(3):312–320. https://doi.org/10.1111/1756-185X.12492 CrossRefPubMedGoogle Scholar
- 19.Domsic RT (2014) Scleroderma: The role of serum autoantibodies in defining specific clinical phenotypes and organ system involvement. Curr Opin Rheumatol 26(6):646–652. https://doi.org/10.1097/BOR.0000000000000113
- 21.Wu C, Wang Q, Xu D, Li M, Hou Y, Zeng X (2014) The prevalence and clinical significance of arthritis in patients with systemic sclerosis. Zhonghua Nei Ke Za Zhi 53(6):460–463. in ChineseGoogle Scholar