Systemic sclerosis is one of the most severe and fatal autoimmune disease, characterized by fibrosis, vasculopathy and impaired immune response. Most common clinical signs of systemic sclerosis include Raynaud syndrome and progressive fibrosis of the skin, lungs, kidneys, heart and gastrointestinal tract.
We report a case of a 44-year-old female who presented with fatigue, joint pain, weight loss, shortness of breath, dysphagia and faecal incontinence. Clinical signs included hyperpigmentation and widespread thickening of the skin, Raynaud’s phenomenon and digital stiffness with calcinosis and intermittent ulceration. The duration between the first symptoms and diagnosis of systemic sclerosis was 6 months. Due to rapid progression of the disease and young age, autologous hematopoietic stem cell transplantation was considered, however, given high risks, the initial therapy of methotrexate was chosen. After 6 months of treatment, the patient developed significant breathlessness due to rapid pulmonary fibrosis as evidenced by high-resolution computed tomography findings and deteriorating pulmonary function tests at which point cyclophosphamide therapy was commenced. After the second cyclophosphamide infusion, patient developed acute respiratory tract infection symptoms. Therefore, immuno suppressive ther apy was suspended.This case report highlights the necessity of aggressive immunosuppressive treatment in rapidly progressive internal organs fibrosis. Appropriate management requires frequent consultations with rheumatology specialists and carefful evaluation of visceral organ involvement.
