Contemporary optimized practice in the management of pulmonary sarcoidosis

Abstract

Pulmonary sarcoidosis is the most common form of sarcoidosis, accounting for the initial presentation in over 70% patients and with eventual presence in 90% of patients with sarcoidosis. However, the course of the disease is often unpredictable; its manifestations can be highly variable and its treatment may not be effective in all patients. As such, the optimized treatment of pulmonary sarcoidosis often requires a thoughtful personalized approach with the need to get the patient involved in decisions of management. In many patients with pulmonary sarcoidosis, the disease is self-limited and nonprogressive, thus treatment is not necessary. In other patients, the presence of significant symptoms or functional limitation often associated with worsening radiological changes and pulmonary function tests warrants treatment. Corticosteroids are the first-line treatment for pulmonary sarcoidosis; antimetabolites are second-line agents, with methotrexate being most commonly employed. Antitumor necrosis alpha antibodies, especially infliximab, are emerging as potential third-line agents. A high index of suspicion should be held for pulmonary hypertension and other comorbidities that may complicate the course of patients with advanced sarcoidosis. Lung transplantation may be the only option for patients who have refractory disease despite maximal medical therapy.

Recommendations for the management of childhood juvenile idiopathic arthritis‐type chronic anterior uveitis

“…Given that prescription of non‐corticosteroid immunomodulatory drugs was by the co‐managing medical specialist, recommendations were not built around the dosing of conventional or biologic immunosuppressive drugs. As a general principle in managing inflammatory disease, cessation of immunomodulatory therapy is considered after a period of quiescence 23‐25 . The Working Group recommended that after inflammation was inactive for 24 months, cessation of the systemic immunosuppressive drug could be considered.…”

Cardiac sarcoidosis: diagnosis and management

“…Corticosteroids have been used for decades as first line treatment of cardiac sarcoidosis (Aryal and Nathan, 2019;Grutters and van den Bosch, 2006). Steroid-sparing immunomodulators have been used or tested as second line treatment for refractory disease.…”

Comprehensive Care for Patients with Sarcoidosis

“…Presently, oral corticosteroids (e.g., prednisolone) are recommended as the first-choice therapy for sarcoidosis [92]. This recommendation is mainly based on expert opinion and a few relatively small observational studies and low-quality randomized trials from over 20 years ago [91,93]. Older studies have demonstrated that corticosteroids lead to an improvement in lung function, especially in patients with initial severe impairment of lung function [94,95].…”

“…Disease relapse occurs in over 30% of patients after discontinuation of corticosteroids. Furthermore, due to the lack of larger randomized trials the optimal dosage and duration of treatment remains unclear [93].…”

“…A multicenter trial evaluating the efficacy and side-effects of prednisone and methotrexate as first-line treatment for pulmonary sarcoidosis has recently started in the Netherlands. A detailed description of medication for sarcoidosis is outside the scope of this review; for an extensive overview, we refer you to other published reviews specifically focusing on this topic [1,2,12,16,90,93].…”