Treatment of Sarcoidosis: A Multidisciplinary Approach

Abstract

Sarcoidosis is a systemic disease of unknown etiology defined by the presence of noncaseating granulomatous inflammation that can cause organ damage and diminished quality of life. Treatment is indicated to protect organ function and decrease symptomatic burden. Current treatment options focus on interruption of granuloma formation and propagation. Clinical trials guiding evidence for treatment are lacking due to the rarity of disease, heterogeneous clinical course, and lack of prognostic biomarkers, all of which contribute to difficulty in clinical trial design and implementation. In this review, a multidisciplinary treatment approach is summarized, addressing immunuosuppressive drugs, managing complications of chronic granulomatous inflammation, and assessing treatment toxicity. Discovery of new therapies will depend on research into pathogenesis of antigen presentation and granulomatous inflammation. Future treatment approaches may also include personalized decisions based on pharmacogenomics and sarcoidosis phenotype, as well as patient-centered approaches to manage immunosuppression, symptom control, and treatment of comorbid conditions.

Repository Corticotropin Injection for the Treatment of Pulmonary Sarcoidosis: A Narrative Review

“…RCI is distinct from corticosteroids, inhibitors of cell activation, proliferation, and migration (e.g., methotrexate, azathioprine, leflunomide, mycophenolate), TNF-a antagonists (e.g., adalimumab, infliximab), and anti-cluster of differentiation (CD)20 antibodies (i.e., rituximab) [56,58,59]. In other inflammatory conditions, such as systemic lupus erythematosus, idiopathic inflammatory myopathies, and rheumatoid arthritis, RCI has been found to be safe and effective for treatment of patients who were nonresponsive to corticosteroids [60][61][62].…”

“…Perhaps the best option for determining appropriate treatment would be through a personalized medicine approach. Disease and risk characteristics, side effect profiles, and patient preference should be taken into account [ 58 ]. Patients with Afro-Caribbean ancestry and women, as well as those with multiorgan involvement, high TNF-α release, and soluble interleukin-2 receptor levels, may have higher risk of mortality and progression of sarcoidosis [ 6 , 37 , 66 ].…”

Sarcoidosis: Review of Diagnosis and Treatment

“…Second, assess whether observation without systemic steroid therapy is possible [51,[52][53][54][55][56][57][58][59][60][61]. if disease is found only in the lungs a pulmonologist should assess the patient and with the assistance of Pulmonary Function Tests and a chest CT decide whether observation will substitute for corticosteroids.…”

“…Third if immunomodulatory therapy is indicated, corticosteroids should be started in a dose of 20-40 mg prednisone or its equivalent in symptomatic sites other than neurologic or cardiac [1,57,58,67]. In these cases 60 mg or more of prednisone should be given [53][54][55].…”

“…However, the majority will have disease recurrence off steroids or be unable to taper the dose of prednisone or its equivalent to less than 5 mg without a flare. Assuming that there is not an alternative explanation for a patients' symptoms, non -adherence or steroid side effects [63], if a patient cannot taper to 5 mg or less within a period of 2-3 months, then steroid sparing agents must be considered [51,52,57,58,61]. Although there are insufficient controlled trials to guide therapy, methotrexate is the most effective and commonly prescribed initial non-steroid immunomodulatory agent chosen.…”