What are the recommended treatment options for sarcoidosis with significant pulmonary involvement?

Treatment options for sarcoidosis with significant pulmonary involvement primarily involve specialist-led management in secondary care. Oral corticosteroids are generally the first-line treatment for active pulmonary sarcoidosis, especially when the disease is dangerous or causes unacceptable loss of quality of life. Topical corticosteroids may suffice for limited skin or eye involvement but are not adequate for significant pulmonary disease ¹.

For patients who require steroid-sparing strategies or have refractory disease, second-line immunosuppressive agents such as methotrexate, hydroxychloroquine, azathioprine, mycophenolate, and leflunomide are recommended. If these are ineffective or not tolerated, third-line options include anti-TNF agents like infliximab or adalimumab ¹.

Ongoing monitoring with chest X-rays, lung function tests, and other investigations is essential to assess disease progression or remission, with longer-term follow-up for stage II to IV pulmonary sarcoidosis or significant extrapulmonary involvement ¹.

Supportive care measures are important adjuncts, including smoking cessation, healthy diet, physical activity, and pulmonary rehabilitation to improve symptoms and quality of life ¹.

In cases complicated by pulmonary hypertension, which can occur in sarcoidosis, specialist cardiopulmonary assessment and management are necessary, although specific pharmacological treatments for sarcoidosis-associated pulmonary hypertension remain under investigation (Liu et al., 2024).

Given the potential adverse effects of corticosteroids and immunosuppressants, treatment is reserved for those with significant disease impact, balancing risks and benefits carefully ¹.