Seminars in respiratory and critical care medicine
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Hepatic sarcoidosis is a relatively common manifestation of extrapulmonary sarcoidosis. It occurs in 20 to 30% of cases and is rarely severe. However, a cluster of patients may develop severe complications such as cirrhosis and portal hypertension. In this review, we describe the current knowledge of clinical, biological, pathological, and radiological features of liver involvement in sarcoidosis and discuss essential clues for management and treatment.
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Semin Respir Crit Care Med · Oct 2020
ReviewOverlap Syndromes in Sarcoidosis: Clinical Features and Outcomes.
Sarcoidosis is a multisystem inflammatory disease characterized by noncaseating granulomatous inflammation. While pulmonary sarcoidosis is most common, extrapulmonary involvement occurs in 50 to 74% of patients and can be the presenting abnormality in some patients. The diagnosis of sarcoidosis is based on a compatible clinical presentation in combination with granulomas on histology and exclusion of other causes. ⋯ Sarcoidosis overlap syndromes are typically described when sarcoidosis is diagnosed in the presence of another disease (concurrently or sequentially) with shared clinical and histologic features, or when sarcoidosis presents with clinical features typically observed in, but not diagnostic of, other diseases. Awareness of overlap syndromes is important for clinicians to avoid diagnostic errors and evaluate for concomitant diagnoses that may impact the management and outcome of sarcoidosis. This article is intended to provide an overview of these presentations and the most commonly associated diseases, with attention to their prevalence, clinical features, and reciprocal impacts on disease outcomes.
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Semin Respir Crit Care Med · Oct 2020
ReviewBalancing Altered Calcium Metabolism with Bone Health in Sarcoidosis.
Abnormal calcium metabolism in sarcoidosis patients can lead to hypercalcemia, hypercalciuria, and kidney stones. Hypercalcemia in sarcoidosis is usually due to increased activity of 1α-hydroxylase in macrophages of pulmonary granulomata, resulting in low levels of 25-hydroxyvitamin D and high levels of calcitriol. ⋯ Diagnosis, treatment of osteoporosis, and maintenance of bone health are complex issues for sarcoidosis patients. An approach to diagnosis and treatment of bone fragility is presented.
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Approximately 5% of patients with sarcoidosis will have clinically manifest cardiac involvement presenting with one or more of ventricular arrhythmias, conduction abnormalities, and heart failure. It is estimated that another 20 to 25% of pulmonary/systemic sarcoidosis patients have asymptomatic cardiac involvement (clinically silent disease). Cardiac presentations can be the first (and/or an unrecognized) manifestation of sarcoidosis in a variety of circumstances. ⋯ The extent of left ventricular dysfunction seems to be the most important predictor of prognosis among patients with clinically manifest CS. In the current era of earlier diagnosis, modern heart failure treatment, and use of ICD therapy, the prognosis from CS is much improved. In a recent Finnish nationwide study, 10-year cardiac survival was 92.5% in 102 patients.