Pre-scleroderma: A reversible “phase” in the fibrotic disease

摘要: 1Consultant Rheumatologist, 2Consultant Immunologist and Sahara Hospital, Lucknow, Uttar Pradesh, India. Correspondence: Dr. Dhanita Khanna, email: dhanitak@yahoo.co.in doi: 10.1016/S0973-3698(11)60206-9 Scleroderma (Scl) is a chronic, idiopathic, fibrosing disorder of connective tissues manifesting characteristically in cutaneous tissues. The “limited” (LSSc) the “diffuse” (DSSc) variants are classified based upon extent skin involvement, although there more reasons to separate two entities immunologically as well predominant sites manifestation internal organs. However, pathogenetically these syndromes appear be related. There rapid progression symptoms diffuse variant within few months years, while limited disease may present only with Raynaud’s phenomenon (RP) for several years before characteristic features becomes apparent. no definite therapy available yet though anti-fibrotic agents, vasodilators, etc. help some extent. Immunosuppression does not have role preventing fibrotic or causing reversal established disease. Therefore, we wonder if reversibility can achieved at early phase process. It prudent differentiate stage this “pre-scleroderma” (pre-Scl) from apparently destined “early” scleroderma. Are diagnose immunologically, pathologically clinically? hypothesised that pre-Scl stage, albeit short, exist DSSc it prolonged LSSc. This most likely manifest onset RP, appearance first non-RP symptom specific Scl “window period” disease; therapeutic intervention here efficacious. would where ischaemic hypoxic changes microcirculation associated tissue beds still devoid chronic reparative processes namely fibrotic/sclerotic changes. Analogous rheumatoid arthritis, also considered early, active which amenable treatment by antiproliferative drugs (ensures vascular potency), antioxidants (prevent re-perfusion injury) possibly immunosuppressive agents (role autoantibodies), could inhibit subsequent target organ damage (fibrosis). Pre-scleroderma approached as: • Diagnostic certainty, Differential mimics, Precipitating events into sclerotic stage.