New advances in the management of pulmonary sarcoidosis

Abstract

Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.

Pulmonary Sarcoidosis: A Clinical Update

“…The evidence-base behind the use of corticosteroids in sarcoidosis is limited and the timing of initiation is controversial. 3 For pulmonary sarcoidosis in the absence of life-threatening disease, some clinicians will opt for monotherapy with oral corticosteroids, usually starting with a dose of 20-40 mg of prednisolone for a month, followed by slow tapering to a maintenance dose, usually aiming to reach 5-10 mg once daily (Figure 4). If there is a rapid response then it may be possible to begin weaning towards zero as early as six months.…”

“…It is beyond the remit of this article to discuss these drug therapies in detail, but they are described elsewhere. 3 All these drugs have potential for toxicity and therefore should be initiated only after careful thought by clinicians with expertise in their use, with 'shared care' monitoring with primary care partners.…”

“…Diagnosis and treatment of sarcoidosis remain challenging. 2,3 While its aetiology and immunopathogenesis are still poorly understood, the last decade has brought considerable advances in managing sarcoidosis: endobronchial ultrasound (EBUS)-guided lymph node sampling is now routine, and 18 F-fl urodeoxyglucose ( 18 F-FDG) positron emission tomography (PET) scanning is sometimes used in selected cases. Furthermore, although corticosteroids remain a cornerstone treatment, newer 'steroid-sparing' therapies, such as biologics, can be used to treat sarcoidosis.…”

Sarcoidosis as an Autoimmune Disease

“…Sarcoidosis is a multisystem granulomatous disease of unknown origin with predominant lung involvement, variable clinical course, and no universally accepted treatment algorithm (1,2). The disease occurs worldwide, varying in prevalence and clinical course between regions, and populations.…”

“…The most common clinical symptoms of pulmonary sarcoidosis are a dry cough, shortness of breath, and chest pain, which are described in more than 50% of patients. Up to 30% of cases include fever, unexplained weight loss, and chronic fatigue (1,2). Extrapulmonary manifestations of the disease are described in 15-25% of cases.…”