Clinical Rheumatology

, Volume 38, Issue 11, pp 3179–3187 | Cite as

Unique clinical and autoantibody profile of a large Asian Indian cohort of scleroderma—do South Asians have a more aggressive disease?

  • Ramya Janardana
  • Aswin M Nair
  • Ajit K Surin
  • John Anthony Jude Prakash
  • Mahasampath Gowri
  • Debashish DandaEmail author
Original Article


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.

Key Points

• 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.


Anti-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




  1. 1.
    Bernatsky S, Joseph L, Pineau CA, Belisle P, Hudson M, Clarke AE (2009) Scleroderma prevalence: demographic variations in a population-based sample. Arthritis Care Res 61(3):400–404. CrossRefGoogle Scholar
  2. 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
  3. 3.
    Ghosh S, Saha I, Bandyopadhyay D, Barua J (2012) Mucocutaneous and demographic features of systemic sclerosis: a profile of 46 patients from Eastern India. Indian J Dermatol 57:201–205. CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Sharma VK, Trilokraj T, Khaitan BK, Krishna SM (2006) Profile of systemic sclerosis in a tertiary care center in North India. Indian J Dermatol Venereol Leprol 72(6):416–420. CrossRefPubMedGoogle Scholar
  5. 5.
    Kumar A, Malaviya AN, Tiwari SC, Singh RR, Kumar A, Pande JN (1990) Clinical and laboratory profile of systemic sclerosis in northern India. J Assoc Physicians India 38(10):765–768PubMedGoogle Scholar
  6. 6.
    Deepa AS, Rachel RP, Ramchandran P, Devaraj U, Arnold SA, Shobha V, D’souza G (2016) Pulmonary involvement in systemic sclerosis: a clinical profile. Lung India 33:144–147. CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Krishnamurthy V, Porkodi R, Ramakrishnan S, Rajendran CP, Madhavan R, Achuthan K, Chandrasekaran AN (1991) Progressive systemic sclerosis in south India. J Assoc Physicians India 39(3):254–257PubMedGoogle Scholar
  8. 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
  9. 9.
    Tamaki T, Mori S, Takehara K (1991) Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res 283(6):366–371. CrossRefPubMedGoogle Scholar
  10. 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. CrossRefPubMedGoogle Scholar
  11. 11.
    Steen V, Domsic RT, Lucas M, Fertig N, Medsger TA (2012) A clinical and serologic comparison of African American and Caucasian patients with systemic sclerosis. Arthritis Rheum 64(9):2986–2994. CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Skare TL, Fonseca AE, Luciano AC, Azevedo PM (2011) Autoantibodies in scleroderma and their association with the clinical profile of the disease. A study of 66 patients from southern Brazil. An Bras Dermatol 86(6):1075–1081. CrossRefPubMedGoogle Scholar
  13. 13.
    Wang J, Assassi S, Guo G, Tu W, Wu W, Yang L et al (2013) Clinical and serological features of systemic sclerosis in a Chinese cohort. Clin Rheumatol 32(5):617–621. CrossRefPubMedGoogle Scholar
  14. 14.
    Medsger TAJ, Bombardieri S, Czirjak L, Scorza R, Della Rossa A, Bencivelli W (2003) Assessment of disease severity and prognosis. Clin Exp Rheumatol 21(3 Suppl 29):S42–S46PubMedGoogle Scholar
  15. 15.
    Hashimoto A, Endo H, Kondo H, Hirohata S (2012) Clinical features of 405 Japanese patients with systemic sclerosis. Mod Rheumatol 22(2):272–279. CrossRefPubMedGoogle Scholar
  16. 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. CrossRefPubMedGoogle Scholar
  17. 17.
    Steen VD, Conte C, Owens GR, Medsger T a (1994) Severe restrictive lung disease in systemic sclerosis. Arthritis Rheum 37(9):1283–1289. CrossRefPubMedGoogle Scholar
  18. 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. CrossRefPubMedGoogle Scholar
  19. 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.
  20. 20.
    Domsic RT, Medsger TA (2016) Autoantibodies and their role in scleroderma clinical care. Current Treatment Options in Rheumatology 2(3):239–251. CrossRefGoogle Scholar
  21. 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

Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of Clinical Immunology and RheumatologyChristian Medical CollegeVelloreIndia
  2. 2.Department of MicrobiologyChristian Medical CollegeVelloreIndia
  3. 3.Department of BiostatisticsChristian Medical CollegeVelloreIndia

Personalised recommendations