Management of extrapulmonary sarcoidosis: challenges and solutions

Abstract

Background: Sarcoidosis 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.

Objective: To review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases.

Materials and methods: We 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 conclusion: Isolated 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.

Keywords: chronic granulomatous diseases; diagnostic tests; immunosuppressive agents; sarcoidosis.

Figure 1

Estimated frequencies of extrapulmonary organ involvement. Notes: Modified from OpenStax College, Anatomy and Physiology. OpenStax CNX. http://cnx.org/contents/14fb4ad7-39a1-4eee-ab6e-3ef2482e3e22@8.24.

Figure 2

Proposed management algorithm for extrapulmonary manifestations of sarcoidosis. Note: “?” indicates “conflicting data”. Abbreviations: ADA, adalimumab; AZA, azathioprine; CNS, central nervous system; CsA, cyclosporine A; CYC, cyclophosphamide; ESRD, end-stage renal disease; GCs, glucocorticoids; HCQ, hydroxychloroquine; HTx, heart transplantation; ICD, implantable cardioverter defibrillator; INF, infliximab; LTx, liver transplantation; MMF, mycophenolate mofetil; MTX, methotrexate; RTx, renal transplantation; THA, thalidomide; UDC, ursodeoxycholic acid; NSAID, non-steroidal anti-inflammatory drugs.