Algorithms of systemic scleroderma treatment with the predominant heart lesion, pulmonary arterial hypertension and interstitial lung disease according to modern guidelines
Systemic scleroderma (SSD, systemic sclerosis) 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 severity of tissue fibrosis. All organs having connective tissue and blood vessels can be involved in the pathological process. At present, the use of treatment algorithms is a modern strategy for patient management, especially after unsuccessful use of the first-line drugs. Cardiac lesion at SSD is one of the most frequent and most unfavorable localizations of the pathological process, taking the first place among the causes of sudden death in patients with SSD. The most dangerous and severe conditions are myocarditis and pericarditis; treatment of these conditions requires the administration of steroids and cytostatics, patient’s management with such frequent concomitant conditions as arrhythmias and heart failure is consistent with general cardiologic recommendations. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids are used to treat pulmonary arterial hypertension associated with SSD (PAH-SSdD), and in this case, immunosuppressive therapy is not effective. In the treatment of interstitial lung disease (ILD) associated with PAH according to modern recommendations, the first line drug is mycophenolate mofetil, the second is cyclophosphamide, azathioprine is used for supportive therapy. Lung transplantation is a salvage therapy, both for PAH-SSD and for ILD-SSD. Therefore, the use of modern treatment algorithms helps to provide step-by-step and effective therapy.