Advances in therapy of systemic sclerosis

Misra, R.; Khanna, D.

Journal of the Association of Physicians of India 54 Suppl: 48-51

2006


ISSN/ISBN: 0004-5772
PMID: 16909717
Document Number: 8220
Systemic sclerosis (SSc) is a chronic autoimmune disorder, characterized by microvascular abnormalities, inflammatory cell infiltrate and an excessive collagen deposition in the dermis and internal organs, leading to excessive fibrosis. Not many large series have been reported from India of this disease. The spectrum of the disease is no different than seen elsewhere in the world. However, there are reports suggesting a much higher prevalence of the diffuse form of the disease as compared to the West) Another study from south India in 98 patients has showed a lower prevalence of the diffuse form of the disease. There is a dysregulated collagen production in SSc in both forms of the disease; the limited and the diffuse cutaneous variants. In the former, the skin involvement is limited to the acral distal parts, while in the latter form, there is widespread involvement of the skin, gastrointestinal tract, lungs, heart and the kidneys. In both, there occurs Raynaud's phenomenon (three phase colour change of finger and toes on exposure to cold) which precedes skin involvement by several months to years. This and the capillary loop abnormalities that occur before the skin involvement suggest that initial vascular events clearly play an important role in establishing the disease. The disease is believed to be due to a complex interaction between the activation of the immune system and microvascular injury which results in dysregulated collagen production. Environmental agents like drugs and toxins (vinyl chloride, silica, and contaminated oil), occupational agents (like vibrational tools etc) have long been implicated as aetiological agents in susceptible individuals. In this review, we will summarize the progress done in the last 5 years on the pathogenesis and treatment of this disease.

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Advances in therapy of systemic sclerosis