Systemic sclerosis (scleroderma, SSc) is a rare autoimmune-driven connective tissue disease.1 It is characterized by fibrosis of the skin and involvement of many internal organs including the lungs, the heart, the GI-tract, the kidneys, and the musculoskeletal system.1 The underlying pathophysiology is not completely understood but it is known that an initial endothelial damage leads to an early inflammatory and finally fibrotic reaction. The resulting accumulation of extracellular matrix (ECM) is responsible for tissue damage and the characteristic clinical symptoms.1 SSc is a heterogeneous disease and occurs in several subsets, which differ in the severity and course of the disease. For therapeutic consideration the limited cutaneous form, the diffuse subset, and the overlap syndrome need to be differentiated.1-3
Monitoring the disease to follow efficiency of therapies is difficult and requires clinical experience. For organ complications, several noninvasive and invasive techniques are available and are currently used. A close interdisciplinary cooperation is required for selecting the appropriate technique.
For skin fibrosis, clinical inspections and the modified Rodnan skin score (mRSS) are routinously applied. Ultrasonic evaluation of skin thickness and measuring skin hardening using a durometer are used in more specialized centers. To monitor patient-reported outcome a special composition of questions has been developed (SSPRO). Also the health assessment questionnaire disability index (HAQ-DI) or the scleroderma health assessment questionaire (SHAQ) is used in clinical studies. More recently, a combined response index for systemic sclerosis (CRISS) has been reported as a clinical outcome measure. The frequency of monitoring depends on the development of the disease and the individual patient. In stable disease, monitoring should be performed at least once a year and should include skin involvement and search for internal manifestations. In rapidly progressing diffuse SSc, patients should be seen regularly every 3 month or depending on medication and organ involvement/symptoms. See Tables 48-1 and 48-2.
Table Graphic Jump Location Table 48-1Systemic Sclerosis Treatment Table ||Download (.pdf) Table 48-1 Systemic Sclerosis Treatment Table
|SKIN FIBROSIS AND ASSOCIATED SYPMTOMS |
|MEDICATION NAME ||INDICATION ||MECHANISM OF ACTION ||DOSING** ||ADVERSE EFFECTS (SEE DETAILED SPECIALISED INFORMATION) ||SUGGESTED MONITORING ||LEVEL OF EVIDENCE (REFERENCE) |
|Topical Therapy |
|Emollients4 ||Skin fibrosis, xerosis cutis, pruritus ||Skincare, improvement of xerosis cutis and pruritus ||Apply at least once per day ||Folliculitis, skin irritation, contact dermatitis ||Clinical inspection/monitoring, individual adjustment ||III5/IV |
|Corticosteroids (medium or high potent); benefit unclear5 ||Early/active skin fibrosis, pruritus ||Anti-inflammatory, improvement of pruritus ||Depending on skin type and strength of topical corticosteroid ||Skin atrophy, telangiectasias, folliculitis ||Clinical inspection/monitoring, individual adjustment ||III5/IV |
|Topical and intralesional corticosteroids ||Calcinosis cutis ||Anti-inflammatory ||Depending on skin type and strength of topical or intralesional corticosteroid ||Skin atrophy, telangiectasias, folliculitis, Lipatrophy ||Clinical inspection/monitoring, individual adjustment ||IV |
|Physical protection ||Sensitive skin over bone prominences, calcinosis cutis ||Skin protection, prevention of wounds, reducing ...|