AB0736 Systemic sclerosis sine scleroderma: characteristics of a south indian cohort from a tertiary care centre. (12th June 2018)

• Record Type: Journal Article
• Authors: Chilukuri, B.; Chinnadurai, S.; Mahendran, B.; Ramamoorthy, R.; Selvakumar, B.; Sankaralingam, R.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1506
• Page End: 1507
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.4481
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Systemic Sclerosis is a complex disorder characterised by autoimmunity, vasculopathy and fibrosis. The hallmark of Systemic Sclerosis is skin thickening. Systemic Sclerosis sine scleroderma is a variant of Systemic sclerosis which shares visceral, serological and vascular manifestations but lacks skin thickening. Systemic Sclerosis sine scleroderma still remains a rarely reported subtype of Systemic Sclerosis unlike commonly reported limited cutaneous and diffuse cutaneous types. Objectives: Aim of this study was to analyse the characteristics of patients with Systemic Sclerosis sine scleroderma from a cohort of Systemic Sclerosis patients. Methods: This study was done at Institute of Rheumatology, Madras Medical College, Chennai, India. It was a retrospective observational study(January 2006 to November 2016). Patients satisfying ACR/ EULAR 2013 Classification criteria for Systemic Sclerosis were included in this study. Patients with Overlap syndrome, MCTD, UCTD, children and pregnant women were excluded. Poormoghim criteria was used for diagnosis of Systemic Sclerosis sine scleroderma:Absent skin thickening +Raynaud's Phenomenon or peripheral vascular involvement +Positive ANAs+One visceral organ involvement typical of SSc +Absence of another defined connective tissue disease. Demographic, clinical and laboratory profile of all patients were analysed. Results: Among the 249 patients included in this study, patients with limited cutaneous systemic sclerosis were 59.4% (n=148) and diffuse cutaneous systemic sclerosis were 31.3% (n=78). 9.2% (n=23) of patients satisfied poormoghim criteria for Systemic Sclerosis sine scleroderma. The following characteristics of Systemic Sclerosis sine scleroderma were observed in our cohort. Dyspnoea (82.6%) was the most common presenting complaint and Raynauds (100%) the most common sign observed. Most common internal organ involvement was oesophageal dysfunction (78%) followed by lung involvement in the form of interstitial lung disease (52.1%) and pulmonary hypertension (47.8%). Renal involvement was not observed in any of the patients. Most common autoantibody detected among patients with Systemic Sclerosis sine scleroderma was anti-centromere antibody (78.2%) followed by anti-scl70 antibody (21.8%). Nine patients (39.1%) received pulse cyclophosphamide therapy and 3 patients received rituximab therapy in view of interstitial lung disease. Conclusions: The most common pattern of Systemic Sclerosis sine scleroderma observed in our cohort was Oesophageal dysfunction +Raynauds Phenomenon+Anti centromere antibody positivity +Interstitial lung disease with or without pulmonary hypertension which are usually the characteristics of limited cutaneous type of Systemic Sclerosis. Hence Systemic Sclerosis sine scleroderma could be a subset of limited cutaneous type without the phenotypic skin involvement. References: [1] Rodnan GP, Fennel RH. Progressive systemic sclerosis sine scleroderma. JAMA1962;180:66570 [2] Poormoghim H, Lucas M, Fertig N, et al. Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum2000;43:44451 Disclosure of Interest: None declared