Systemic scleroderma treatment algorithms with the predominant skin and joint lesions, Raynaud’s syndrome and digital ulcers according to modern guidelines

Abstract

Systemic scleroderma (SSD) (systemic sclerosis) is a unique rheumatic disease because it presents the challenge of managing a chronic multisystem autoimmune di­sease with a widespread obliterative vasculopathy of small arteries associated with varying degrees of tissue fibrosis. All organs ha­ving connective tissue and blood vessels can be involved in the pathological process. The progressive clinical course of SSD leads to the development of irreversible fibrotic changes, resulting in dysfunction of the affected organs. A distinctive feature of SSD is the clinical heterogeneity of subgroups of patients, which varies depending on the severity of the disease, the involvement of various organs and systems and the prognosis. The physician should carefully examine each SSD patient to understand the specific manifestations and the level of disease activity to prescribe appropriate the­rapy. At present, the use of treatment algorithms is a mo­dern strategy for patients’ management, especially after unsuccessful use of the first line drugs. In early active diffuse scleroderma with predominant skin lesion, methotrexate (MTХ) should be preferred as the first line drug, and with its ineffectiveness or intolerance, the second line drug is mofetil mycophenolate (MMF), if it’s ineffective, the third line drug is intravenous cyclophosphamide. It should be noted that with severe skin lesions, the first line drug is MMF, and MTХ — the second one, if MMF if ineffective. To date, calcium channel blo­ckers (CCBs), mainly nifedipine, are the first line drugs for the treatment of SSD-associated Raynaud’s syndrome. If these drugs are ineffective, phosphodiesterase-5 inhibitors (iFDE-5) should be added, the next step in therapy is to prescribe angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. In case of combination therapy by CCBs and iFDE-5 ineffectiveness in Raynaud’s syndrome, prostanoids should be added. The first line drug for the arthritis treatment, as well as for skin lesions, is MTX; when it is ineffective or the process has high inflammatory activity, glucocorticoids and nonsteroidal anti-inflammatory drugs should be added. Hydroxychloroquine is the third line drug and is added to the therapy if the above agents were ineffective. Biological agents (rituximab and tocilizumab) are the fourth line drugs in musculoskeletal involvement.

Keywords

• systemic sclerosis
• skin fibrosis
• Raynaud’s syndrome
• digital ulcers
• algorithm
• treatment