From the Guidelines
Sarcoidosis patients should be treated based on their risk of death or permanent disability and impact on quality of life (QoL), with corticosteroids like prednisone being the first-line therapy for symptomatic disease. The management of sarcoidosis can be challenging due to its variable outcomes, with some patients having a very good outcome and never requiring treatment, while others may die from advanced lung disease 1. The decision to treat sarcoidosis depends on two major factors: risk for death or organ failure and impairment of QoL, with about 5% of patients with sarcoidosis dying from the disease 1.
Some key points to consider in the treatment of sarcoidosis include:
- The use of oral glucocorticoids as first-line therapy, with implementation of non-biologic and biologic immunomodulatory therapies used primarily as steroid-sparing therapies and/or to treat steroid-refractory disease 1
- The importance of regular monitoring, including pulmonary function tests, chest imaging, and organ-specific evaluations based on involvement
- The need for treatment to be individualized based on the presence of symptomatic disease and the patient's risk for death or permanent disability and impact on QoL
The prognosis for sarcoidosis patients is generally favorable, though some patients may develop chronic disease requiring long-term management 1. Treatment should be focused on preventing progression to irreversible disease and improving QoL, with the goal of minimizing morbidity and mortality. In patients with troublesome fatigue or small-fibre neuropathy-associated symptoms, treatment is a high priority 1. Overall, the treatment of sarcoidosis requires a comprehensive approach that takes into account the patient's individual needs and risk factors.
From the Research
Definition and Diagnosis of Sarcoidosis
- Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas in any organ, most commonly the lungs and intrathoracic lymph nodes 2.
- A diagnosis of sarcoidosis should be suspected in any young or middle-aged adult presenting with unexplained cough, shortness of breath, or constitutional symptoms, especially among blacks or Scandinavians 2.
- Diagnosis relies on three criteria: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings, such as infections or malignancy 2.
Treatment of Sarcoidosis
- Corticosteroids remain the first-choice therapeutic in sarcoidosis; however, long-term use is associated with toxicity 3.
- Corticosteroid therapy has been the mainstay of therapy for those with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease 2.
- Second-line therapy includes cytotoxic agents such as methotrexate and azathioprine, which have significant steroid-sparing potency and a similar positive effect on lung function 3, 4.
- Immunosuppressives such as methotrexate, azathioprine, leflunomide, and mycophenolate have all been reported as effective in sarcoidosis 5.
- Biologics and other agents are third-line therapy, with monoclonal antibodies directed against tumor necrosis factor shown to be particularly effective for advanced disease 5.
Monitoring and Management of Sarcoidosis
- Effective regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications 6.
- A multidisciplinary approach is recommended for best outcomes, with the pulmonologist often being the referral physician for diagnosis and management 6.
- A comprehensive diagnostic work-up and monitoring protocol is proposed to confirm diagnosis, assess organ involvement/impairment, and establish whether the patient is likely to benefit from treatment 6.