My Approach to the Treatment of Scleroderma

Abstract

Systemic sclerosis (scleroderma) is unique among the rheumatic diseases because it presents the challenge of managing a chronic multisystem autoimmune disease with a widespread obliterative vasculopathy of small arteries that is associated with varying degrees of tissue fibrosis. The hallmark of scleroderma is clinical heterogeneity with subsets that vary in the degree of disease expression, organ involvement, and ultimate prognosis. Thus the term “scleroderma” is used to describe patients that have common manifestations that link them together, while a highly variable clinical course exists that spans from mild and subtle findings to aggressive life-threatening multisystem disease. The clinician needs to carefully characterize each patient to understand the specific manifestations and level of disease activity in order to decide appropriate treatment. This is particularly important in managing a patient with scleroderma because there is no treatment that has been proven to modify the overall disease course; while therapy that targets specific organ involvement early before irreversible damage occurs does improve both quality of life and survival. This review describes our approach as defined by evidence, expert opinion and our experience treating patients. Scleroderma is a multisystem disease with variable expression; thus any treatment plan must be holistic yet at the same time focus on the dominant organ disease. The goal of therapy is both to improve quality of life by minimizing specific organ involvement and subsequent life-threatening disease. At the same time the many factors that alter daily function need to be addressed including nutrition, pain, deconditioning, musculoskeletal disuse, co- morbid conditions and the emotional aspects of the disease such as fear, depression and the social withdrawal caused by disfigurement.

Phenotyping Exercise Limitation in Systemic Sclerosis: The Use of Cardiopulmonary Exercise Testing

“…Another reason might be that several patients categorized in the N group presented with subnormal exercise capacity due to deconditioning. Chronic inflammation, pain, musculoskeletal disuse due to cutaneous lesions or arthritis, chronic anxiety and depression, social withdrawal or isolation due to disfigurement and potential adverse effects of corticosteroid use on emotional status and muscular function [25,26,27] are only some of the potential factors leading to decreased physical activity and increased exertional dyspnea among these patients, without evident heart or lung involvement being present.…”

A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face

“…At the same time, the many factors that alter daily function need to be addressed, including nutrition, pain, deconditioning, musculoskeletal disuse, co-morbid conditions and the emotional aspects of the disease such as fear, depression and the social withdrawal caused by disfigurement. 13 In majority cases of systemic scleroderma, methotrexate, cyclosporin, tacrolimus, relaxin, low-dose penicillamine, and intravenous Ig may be beneficial in improving the skin tightness. Calcium channel blockers, the angiotensin II receptor type 1 antagonist losartan, prazosin, the prostacyclin analogue iloprost, N-acetylcysteine and the dual endothelin-receptor antagonist bosentan may be beneficial for Raynaud's phenomenon.…”

Physiotherapeutic intervention on pain and quality of life of systemic sclerosis elderly patients. Case reports

“…Physiotherapy seems to play a critical therapeutic role, since it prevents musculoskeletal injuries, improves sleep quality, depressive symptoms, fatigue, pain, cognitive aspects, body image, static condition and maintains functionality, even of hand, which is fundamental for their QL 5,18,19 . Although there is no specific physiotherapeutic protocol for SS 18 , functionality and DLA of these patients are maintained or improved if physical and occupational interventions are started early, assuring better long-term results 20 . Regular physical exercise has a possible anti-inflammatory effect on chronic diseases and may decrease immunosuppressant drugs consumption and/or doses 21 .…”