Systemic sclerosis sine scleroderma with atypical clinical course: a rare case report

Systemic sclerosis (SSc) is divided into three subtypes: limited cutaneous SSc (lcSSc), diffuse cutaneous SSc (dcSSc), and systemic sclerosis sine scleroderma (ssSSc). ssSSc is a rare subtype of SSc that presents with internal organ manifestations but no cutaneous findings.

We report the case of a 58-year-old patient with a history of pulmonary hypertension who presented with symptoms of fatigue, inflammatory polyarthritis, and joint swelling. Following a comprehensive clinical examination and laboratory tests, the patient was diagnosed with ssSSc.

Due to its atypical clinical course, we present this case report, which commenced with idiopathic pulmonary hypertension. Subsequently, after 7 months, the patient presented complaints of polyarthritis with positive antinuclear antibodies. Raynaud's phenomenon was identified 2 months later during the rheumatology clinic examination. Typically, the clinical course encompasses all three features simultaneously, without any gap between them.

Diagnosis of ssSSc remains challenging, and it is essential to consider this disease form in all cases involving unexplained fibrotic involvement of the internal organs.