Management of extrapulmonary sarcoidosis: challenges and solutions

Abstract

BackgroundSarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients.ObjectiveTo review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases.Materials and methodsWe performed a literature search using Medline and Google Scholar for individual or combined keywords of “sarcoidosis, extrapulmonary, treatment, kidney, neurosarcoidosis, cardiovascular, gastrointestinal, transplantation, musculoskeletal, rheumatology, arthritis, and skin”. Peer-reviewed articles, including review articles, clinical trials, observational trials, and case reports that were published in English were included. References from retrieved articles were also manually searched for relevant articles.Results and conclusionIsolated involvement of a single organ or organ system is rare in sarcoidosis, and thus all patients must be thoroughly evaluated for additional disease manifestations. Cardiac sarcoidosis and neurosarcoidosis may be life-threatening. Clinicians need to assess patients comprehensively using clinical, laboratory, imaging, and histopathological data to recommend competently the best and least toxic treatment option for the individual patient.

Application of the modified Zajicek criteria to diagnose probable spinal cord neurosarcoidosis

“…Furthermore, cases of isolated pulmonary sarcoidosis frequently resolve spontaneously within 2 years, and a careful watch and wait strategy is advocated . Indications for immediate treatment of sarcoidosis are generally recognized as functional respiratory impairment or extrapulmonary manifestations including neurological, cardiac, or renal involvement, ocular disease refractory to topical therapy, or symptomatic refractory hypercalcaemia…”

“…Immediate treatment for patients with confirmed systemic sarcoidosis with evidence of CNS involvement has been advocated, as unlike pulmonary sarcoidosis spontaneous resolution of neurosarcoidosis is rare, and there is significant risk of morbidity and mortality . Treatment with corticosteroids, typically 1 mg/kg/d is first line for neurosarcoidosis.…”

“…Treatment with corticosteroids, typically 1 mg/kg/d is first line for neurosarcoidosis. For patients presenting with severe disabling disease such as brain or spinal cord lesions, leptomeningeal involvement or hydrocephalus, aggressive management with pulsed IV methylprednisolone followed by prolonged corticosteroid wean over six to twelve months is recommended . Treatment with disease‐modifying antisarcoidosis drugs (DMASDs) such as methotrexate, azathioprine, or cyclophosphamide has been reported for patient with disease refractory to glucocorticoid monotherapy, or when the side effect profile of glucocorticoids is prohibitory .…”

Increased Risk of Multimorbidity in Patients With Sarcoidosis: A Population-Based Cohort Study 1976 to 2013

“…Second, the increased incidence of comorbidity and multimorbidity could be a complication of glucocorticoids, the most commonly used medications for treatment of sarcoidosis 29, 30 . Several comorbidities analyzed in this study, including diabetes mellitus, hypertension, depression and osteoporotic fracture, are well-established complications of glucocorticoids 31 .…”

Reliability of Cardiovascular Risk Calculators to Estimate Accurately the Risk of Cardiovascular Disease in Patients With Sarcoidosis

“…19, 20 Use of glucocorticoids is associated with increased risk of several CV risk factors such as diabetes mellitus and dyslipidemia and could increase the risk of CVD. 21, 22 Medications are not included in either the FRS or ACC/AHA risk score.…”