Clinical Essentials | Treatment and Follow-up of Pulmonary Sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by noncaseating necrotizing epithelioid granulomas. It can affect all organs of the body, but the lungs and intrathoracic lymph nodes are the most common. Sarcoidosis usually causes no obvious symptoms in the early stages, but when it affects the lungs or heart and progresses, it can cause severe and life-threatening symptoms. Involvement of other organs such as the eyes, nerves, and skin usually does not affect the patient’s lifespan, but it also significantly reduces the patient’s quality of life. Appropriate interventions are therefore also required. TreatmentBecause pulmonary sarcoidosis has a certain rate of spontaneous remission (stage I spontaneous remission rate is 55%~ 90%; the spontaneous remission rate of stage II is 40%-70% or the spontaneous remission rate of stage III is 10%-20%), so the treatment of sarcoidosis should be selected according to the clinical manifestations, involved parts, and severity. Treatment is usually not required when patients are in asymptomatic stage 0 or I, for asymptomatic In stage II or III pulmonary sarcoidosis, if the disease is stable and only mild pulmonary function abnormalities are present, systemic corticosteroid therapy is not recommended. If there are ① obvious respiratory symptoms, such as cough, dyspnea, chest pain, etc., or obvious systemic symptoms, such as fatigue, fever, weight loss, etc.; ② progressive deterioration of lung function; Systemic hormone therapy is used, usually prednisone 0.5 mg/kg·d or 20-40 mg/d; after 2-4 weeks of treatment, the dose is gradually reduced to 5-10 mg/d for maintenance therapy, and the total course of treatment is 6-24 months. Inhaled corticosteroids can be used in patients with intrathoracic sarcoidosis who have respiratory symptoms such as cough and shortness of breath, and multiple nodules in the bronchial mucosa. If important organs outside the lungs are involved, such as the heart, nervous system, eyes, liver, etc., which affects the patient’s quality of life, systemic hormone therapy is recommended. For patients with sarcoidosis whose disease has not been effectively controlled after systemic hormone therapy, methotrexate can be added to improve the The patient’s FVC and QoL (the usual dose is 10-15 mg/week), and azathioprine, leflunomide, mycophenolate mofetil, etc. can be selected when intolerant. In patients whose disease progresses despite methotrexate or other immunosuppressive agents, additional biologics, such as tumor necrosis factor-alpha inhibitors, may be added for further treatment: Infliximab, Adalimumab, etc. If the patient develops sarcoid-related fatigue, 6-12 weeks of pulmonary rehabilitation training and/or inspiratory muscle training can be performed first to improve fatigue. After training, there is no change in disease activity, but there is still fatigue, and neurostimulants such as methylphenidate and modafinil can be used to treat sarcoidosis-related fatigue. Follow-upThe recurrence rate of sarcoidosis patients in remission after hormone therapy is high, about 37%~ 74%, and most patients relapsed 2 to 6 months after steroid discontinuation. Therefore, it is recommended that patients with sarcoidosis should be followed up every 4 to 6 weeks in the initial stage of treatment, every 3 to 6 months after treatment improves, and every 6 months after drug discontinuation, for at least continuous follow-up. 3 years. The follow-up interval can be appropriately extended after 3 years. However, long-term follow-up is recommended for patients with Ⅳ sarcoidosis and those with involvement of important extrapulmonary organs (heart, central nervous system, etc.). References:1.Interstitial Lung Disease Group of Respiratory Branch of Chinese Medical Association, Chinese Medical Doctor Association Interstitial Lung Disease Working Committee of Respiratory Physicians Branch. Expert consensus on diagnosis and treatment of pulmonary sarcoidosis in China[J]. Chinese Journal of Tuberculosis and Respiratory Medicine, 2019,42(9):685-693. 2.Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J . 2021 Jun 17:2004079. 3.Terasaki F, Azuma A, Anzai T,et al. JCS 2016 Guideline on Diagnosis and Treatment of Cardiac Sarcoidosis. Circ J. 2019 Oct 25;83(11):2329-2388.