Current options for the treatment of systemic scleroderma

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Systemic scleroderma (SSc) is a rare autoimmune disease of unknown cause.
• It affects small arteries, microvessels, and connective tissue, leading to significant internal organ involvement.
• Internal organ damage is the primary driver of morbidity and mortality in SSc patients.

Purpose of the Study:

• To describe the epidemiology, pathology, diagnosis, and clinical manifestations of SSc.
• To discuss current and potential therapeutic options for managing SSc.
• To highlight challenges in SSc treatment, including toxicity and drug interactions.

Main Methods:

• Review of existing literature on SSc epidemiology, pathology, and clinical features.
• Analysis of current therapeutic strategies, including supportive, palliative, remittive, and immunosuppressive approaches.
• Evaluation of the effectiveness and limitations of various treatments for dermatologic, vascular, gastrointestinal, and renal manifestations.

Main Results:

• Supportive therapies include temperature maintenance and emollients; palliative treatments show mixed results with potential toxicity.
• Dermatologic issues managed with NSAIDs, corticosteroids, DMSO, and disodium edetate; vascular issues with alpha-blockers, ACE inhibitors, and calcium-channel blockers.
• GI symptoms addressed with antacids, H2 blockers, sucralfate, and cholinergic agents; SSc renal crisis controlled by captopril and enalapril.

Conclusions:

• No current therapy consistently alters the long-term course of SSc.
• Penicillamine shows promise as a remittive therapy; immunosuppressants like fluorouracil, cyclosporine, and methotrexate require further investigation.
• Aggressive patient monitoring is crucial due to significant toxicity and drug interaction concerns in SSc management.