Rapidly progressive course of systemic sclerosis sine scleroderma: a case report and short literature review

Systemic sclerosis (SSc) is an autoimmune disease that affects the connective tissue. It is progressive and characterized by a diverse course, resulting in a heterogeneous clinical picture. One form of SSc is systemic sclerosis sine scleroderma which affects only 10% of patients. Common manifestations such as skin involvement, Raynaud's phenomenon or telangiectasia are not always the first signs of the disease, which is particularly important for the proper establishment of a diagnosis and initiation of treatment. The success of therapy depends on the stage of the disease, but even with appropriate ongoing treatment, it may not yield the desired effects. A rare case of systemic sclerosis with delayed cutaneous manifestation was reported. The disease appeared as acute renal insufficiency. Skin involvement was clinically overt and detectable after development of kidney failure. Earlier the patient had enhanced titer of anti-topoisomerase I (Scl-70) antibodies and unspecific alterations visible at capillaroscopy only. The patient required treatment with dialysis, intravenous cyclophosphamide was initiated, followed by oral administration, with good tolerance and achieving reduction of symptoms. Nevertheless, the disease was very severe, rapidly progressive, and fatal.