Chronic Constrictive Pericarditis: A rare cardiac involvement in primary Sjögren’s Syndrome

Abstract Background Constrictive pericarditis represents a chronic condition of which systemic inflammatory diseases are a known, yet uncommon, cause. In primary Sjögren’s Syndrome, pericardial involvement is seldom reported, and it is usually in-association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution related to primary Sjögren’s Syndrome. As work-up diagnosis is challenging, clinical suspicion and multimodality imaging are essential for an early diagnosis and rapid onset of treatment. Long-term outcomes remain uncertain. To the best of our knowledge no other case linking this autoimmune disease to constrictive pericarditis have been reported. Case presentation We report the case of a 48 years-old male patient with moderated alcohol habits and two previous hospital admissions. The first hospitalization was due to pleural effusion and ascites, when the diagnosis of primary Sjögren´s Syndrome was performed and an empirical corticotherapy regiment was initiated. Two-years later, he was readmitted with complains of dyspnea and abdominal distension. Hepatic peliosis was diagnosed on liver biopsy and considered secondary to glucocorticoid therapy. A localized pericardial thickening and a thin circumferential pericardial effusion was evident on thoracic computed tomography, both assumed as secondary to autoimmune disease. Diuretic therapy was adjusted, and the patient was discharged symptom-free. He was admitted again, around 4 years later, with anasarca and right pleural effusion and at this time a constrictive pericarditis was diagnosed. A partial pericardiectomy was successfully performed. Since then, although not completely asymptomatic the patient referred clinical improvement but still with a need for baseline diuretic therapy. Conclusion Albeit uncommon, connective tissue disorders, such as primary Sjögren’s Syndrome, should be considered as a cause of constrictive pericarditis, particularly in young patients without other classical risk factors for constriction. A primary Sjögren´s Syndrome diagnosis with a likely association with constrictive pericarditis represents an interesting and challenging case attending to its rarity in modern clinical practice. A high clinical awareness and multimodal cardiac imaging are essential in earlier recognition and treatment approach.