A case report on Sjögren Syndrome: it‘s more than just autoimmune epithelitis

Background. Primary Sjögren’s syndrome (pSS) is an autoimmune epithelitis associated with various pulmonary manifestations, including PAH (pulmonary arterial hypertension), a severe complication often found in collagen tissue disorders. Case presentation. A 26-year-old female having chest pain and exertional dyspnea diagnosed with severe PAH associated with pSS is presented in this case study. Clinical, laboratory, and imaging findings supported the diagnosis, and treatment involved immunosuppressive therapy and standard PAH medications. Discussion and conclusion. The report highlights the complexities of diagnosing and treating PAH associated with pSS, emphasizing the importance of early intervention for improved long-term outcomes. The prognosis for PAH in connective tissue diseases, encompassing pSS, remains challenging, underscoring the need for accurate diagnosis and timely management with immunosuppressants and PAH-specific therapies.