The Importance of Early Diagnosis of Systemic Scleroderma

Systemic scleroderma (SSc) is a progressive and severely debilitating autoimmune disease characterized by inflammation, vasculopathy, and extensive fibrosis. SSc is highly heterogeneous in its clinical presentation and severity of skin and internal organ lesions, and it has also the highest mortality rate among connective tissue diseases. Recently, Raynaud’s phenomenon (RP), puffy fingers turning into sclerodactyly, and antinuclear antibody (ANA) positivity are considered the three red flags for the suspicion of a very early SSc. Further signs such as positivity of other specific autoantibodies and/or video capillaroscopy pattern may allow very early SSc diagnosis. No current therapy is able to reverse or stop the natural progression of SSc, which reflects its complex pathogenesis. In our article, we analyzed a clinical case of SSc and modern methods of pathogenetic therapy, substantiating the importance of new diagnostic approaches for the early detection of the disease, and increasing the effectiveness of treatment. An ultrasound examination of the internal organs revealed a change in the structure of the thyroid gland. SSc was diagnosed and the proper medication prescribed. Further diagnosis revealed diffuse form of SSc, and activity of the II degree. Later on, a seroma of the right groin area was diagnosed. After some times, pulmonary arterial hypertension associated with SSc was verified. Revealed signs of fibrosis in the myocardium of both ventricles indicated heart damage. Therefore, Bosentan was prescribed. It was revealed that drugs cannot be canceled and replaced, since even a short-term cancellation of specific therapy for pulmonary arterial hypertension leads to a rapid progression of the disease with the possible development of a fatal outcome.

کلیدواژه ها:

antinuclear factor ، Immune Cells ، immunofluorescence test ، microvascular damage