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Egg-shell calcification in Pick's disease (chronic constrictive pericarditis).

ABSTRACT: Egg-shell calcification is rare in cases with constrictive pericarditis. It leads to significant right heart failure and the only treatment is surgical excision of the pericardium. We present a case of a 22-year-old-male who was diagnosed to have severe pericardial calcification on the chest X-ray, which eventually led to a diagnosis of constrictive pericarditis and required an early surgical correction. This case shows that a common diagnostic test such as a chest X-ray does help to diagnose a severe systemic condition.

SUBMITTER: Deshpande SA

PROVIDER: S-EPMC7848771 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature

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Egg-shell calcification in Pick's disease (chronic constrictive pericarditis).

Deshpande Saurabh Ajit SA Udyavar Ameya A

Radiology case reports 20210129 4

Egg-shell calcification is rare in cases with constrictive pericarditis. It leads to significant right heart failure and the only treatment is surgical excision of the pericardium. We present a case of a 22-year-old-male who was diagnosed to have severe pericardial calcification on the chest X-ray, which eventually led to a diagnosis of constrictive pericarditis and required an early surgical correction. This case shows that a common diagnostic test such as a chest X-ray does help to diagnose a ...[more]

PMID: 33552337

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Project description:BackgroundConstrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren's syndrome, usually occurring in association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren's syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain. To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported.Case presentationWe present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren's syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted. Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed. Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy.ConclusionAlbeit uncommon, connective tissue disorders, such as primary Sjögren's syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction. In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.

Dataset Information

Egg-shell calcification in Pick's disease (chronic constrictive pericarditis).

Publications

Egg-shell calcification in Pick's disease (chronic constrictive pericarditis).

Similar Datasets

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