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Reviewing ERS Treatment Guidelines in Sarcoidosis

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Task force members of the European Respiratory Society have evaluated the available evidence on treatments for various symptoms of sarcoidosis, but said their recommendations are not the final word.

With increasing interest in addressing disparities in healthcare, a rare condition known to affect populations disproportionately is sarcoidosis. Sarcoidosis is a rare condition in which small inflammatory cells called granulomas appear in the body, especially in the lungs and lymph nodes.

according to mayo clinicAlthough researchers do not fully understand the cause of sarcoidosis, it is believed to be caused by the body’s immune response to an unknown substance. Possible causes are infectious agents, chemicals, or allergens. For some people, a genetic predisposition may cause the body to respond to its own proteins.

What is known is that sarcoidosis affects some populations more than others. American Lung AssociationIn the United States, blacks are three times more likely than whites to develop the disease, and women are more likely than men to develop it. In addition to the lungs and lymph nodes, the disease can also affect the eyes, skin, heart, and other organs. Morbidity can be significant and the disease can be fatal.

Initial treatment for sarcoidosis usually includes glucocorticoids (GCs), but long-term use can cause other problems due to toxicity. That is why in 2021 the task force of the European Respiratory Society will propose his GRADE (Grading of

recommendation, evaluation, development, and evaluation) methodology to guide decision-making.1

Task force members developed eight prompts that will be used to direct evidence-based recommendations. This is called the PICO (Patient, Intervention, Comparison, Outcome) question. Members were asked to declare conflict and refrain from engaging in potentially conflicting discussions.

This process yielded 12 recommendations for the 7 PICO questions. The task force then presented algorithms for the various scenarios outlined in the question, covering treatment (or device) options in pulmonary, cutaneous, and cardiac sarcoidosis, symptomatic neurosarcoidosis, and sarcoidosis-related fatigue. did.

Based on current practice, an algorithm for symptoms associated with fibril neuropathy was presented. However, in response to PICO’s inquiry, the Commission said the current evidence was insufficient to make a formal recommendation.

A devotee pointed out that treating patients with sarcoidosis is very difficult. “Instead of focusing on a single symptom, clinicians are encouraged to observe a variety of symptoms initially and over time.” We must remember,” the committee wrote.

Results are highly variable. Mortality is less than 10% he has, but at least 25% he needs more than 2 years of treatment. The Task Force notes that stopping treatment prematurely can lead to recurrence, making these decisions extremely difficult.

Task force members also developed a table summarizing the different treatment options that account for toxicity and comorbidity risks.

pulmonary sarcoidosis

The algorithm was derived from a systematic review of 1,747 potentially relevant articles. Of these, 36 were reviewed and 19 were selected. A total of 134 patients were included in the study. Of all the algorithms presented, this one offered the most options.

Providers are first asked to determine if the patient is at low risk, intermediate risk, or high risk. We consider minimizing the risk of disability, loss of life due to pulmonary involvement or loss of quality of life, or comorbidity from GC or other treatments. For pulmonary sarcoidosis, interstitial lung disease or pulmonary hypertension are the major risks and the leading causes of disease-related mortality. The scenario flow is as follows.

Low risk. Patients should be kept under observation.Prescribe GC if treatment is needed; This is highly recommended.

Moderate risk, but reduced quality of life. Most of the algorithms are based on current practice. After a favorable clinical response, tapering of GC treatment is recommended.

  • Start with GC and eventually taper off GC if clinical response is favorable. For GC side effects, disease continuation, or recurrence, options are methotrexate, azathioprine, leflunomide, mycophenolate mofetil, or hydroxychloroquine.
  • If there is a favorable clinical response, taper off with GC eventually. If there is continued or recurrent disease, options are infliximab or adalimumab. If there is a favorable clinical response, taper off with GC eventually.
  • If there is continued or recurrent disease, options are rituximab, JAK inhibitors, or case-by-case repository corticotropin injection (RCI). Any good response will eventually taper off in GC.

high risk. Follows similar algorithm to intermediate risk, except methotrexate in second-line therapy is conditionally recommended (based on very low-quality evidence). Infliximab in third-line therapy has a conditional recommendation (based on low-quality evidence).

Task force members will seek to “evaluate the efficacy, safety and cost-effectiveness” of rituximab, RCIs, anti-tumor necrosis factor biosimilars, other immunosuppressants, and antifibrotic agents such as nintedanib and pirfenidone. I asked for further research. Members wrote that new endpoints, including changes in quality of life, need to be validated.

“We do not believe that these guidelines are the final word on the management of sarcoidosis,” the authors wrote, and enforce “informed recommendations” based on current evidence and clinical experience. However, the task force members were outspoken when the evidence was lacking.

“We anticipate that updates to this guideline will be required within the next five years as more information becomes available,” they wrote.


1. Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines for the treatment of sarcoidosis. Eulerspear J 2021; 58: 2004079. DOI: 10.1183/13993003.04079-2020.

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