Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study

Rahaghi, Franck; Sweiss, Nadera J.; Saketkoo, Lesley Ann; Scholand, Mary Beth; Barney, Joseph B.; Gerke, Alicia K.; Lower, Elyse E.; Mirsaeidi, Mehdi; O'Hare, Lanier; Rumbak, Mark J.; Samavati, Lobelia; Baughman, Robert P.

doi:10.1183/16000617.0147-2019

Cited by 13 publications

(13 citation statements)

References 30 publications

(40 reference statements)

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“…The dose and duration of glucocorticoid use were major determinants of when to switch to non-biologics. While methotrexate and azathioprine were the most commonly cited non-biologics, specific details regarding individual non-biologics are discussed elsewhere in this issue of the European Respiratory Review [26].…”

Section: Phenotyping For Treatment Decisionsmentioning

confidence: 99%

Clinical phenotyping: role in treatment decisions in sarcoidosis

2020

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A variety of phenotypic categorisations have been developed for sarcoidosis. Phenotyping has been used for genetics studies and to guide treatment selection. The authors participated in a Delphi expert consensus panel to develop a proposed phenotype categorisation and treatment recommendations for pulmonary sarcoidosis patients. Panellists reached consensus that asymptomatic patients with normal pulmonary function and adenopathy alone or normal chest imaging do not require therapy, while symptomatic patients with impaired pulmonary function or infiltrates should be treated. The panel did not reach consensus on asymptomatic patients with abnormal chest imaging or reduced pulmonary function, or symptomatic patients with normal chest imaging and pulmonary function. The proposed phenotype categories and associated treatment recommendations are asymptomatic (no therapy), acute (disease duration <1–2 years, apparently self-limited, corticosteroids), chronic (antimetabolites and other second-line therapies) and advanced (biologics). Some clinical settings, such as dyspnoea/hypoxaemia at rest, severely impaired or rapidly decreasing pulmonary function tests, and severe cardiac, neurologic, ocular or renal involvement warrant immediate therapy.

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Section: Phenotyping For Treatment Decisionsmentioning

confidence: 99%

Clinical phenotyping: role in treatment decisions in sarcoidosis

2020

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“…In a single-blind prospective study, ACTHAR gel, a repository corticotropin injection therapy consisting of a mixture of pituitary extracts enriched with ACTH, at a subcutaneous dose of 80 or 40 IU twice a week offered benefits and GC-sparing effects in 16 patients with chronic pulmonary sarcoidosis who completed 24 weeks of treatment [ 43 ]. A Delphi panel recommended that lack of efficacy for ACTHAR gel should be evaluated after 3–6 months of therapy [ 44 ].…”

Section: Role Of Antimetabolites In the Management Of Sarcoidosismentioning

confidence: 99%

A Comprehensive Review of Sarcoidosis Treatment for Pulmonologists

et al. 2021

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Due to frequent lung involvement, the pulmonologist is often the reference physician for management of sarcoidosis, a systemic granulomatous disease with a heterogeneous course. Treatment of sarcoidosis raises some issues. The first challenge is to select patients who are likely to benefit from treatment, as sarcoidosis may be self-limiting and remit spontaneously, in which case treatment can be postponed and possibly avoided without any significant impact on quality of life, organ damage or prognosis. Systemic glucocorticosteroids (GCs) are the drug of first choice for sarcoidosis. When GCs are started, there is a [ 50% chance of long-term treatment. Prolonged use of prednisone at [ 10 mg/day or equivalent is often associated with severe side effects. In these and refractory cases, steroid-sparing options are advised. Antimetabolites, such as methotrexate, are the second-choice therapy. Biologics, such as anti-TNF and especially infliximab, are third-choice drugs. The three treatments can be used concomitantly. Regardless of whether treatment is started, the clinician needs to organize regular follow-up to monitor remissions, flares, progression, complications, toxicity and relapses in order to promptly adjust the drugs used.

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“…Unlike methotrexate, infliximab, adalimumab, rituximab, and mycophenolate, RCI is FDA-approved for symptomatic sarcoidosis and has a long-standing history in the treatment of autoimmune and inflammatory disorders [ 16 ]. Taking the unique mechanism of action, favorable safety profile, and clinical history into account, RCI is expected to soon become an effective treatment option worldwide [ 65 ].…”

Section: Introductionmentioning

confidence: 99%

“…A panel of experts used a modified Delphi process to agree on a starting dose of 40 U RCI twice weekly for patients with less severe disease and that the drug be continued at a maintenance dose (individualized for each patient) for those who responded, particularly those with chronic refractory sarcoidosis [ 65 ]. Concomitant steroids are recommended to be quickly tapered in patients receiving RCI, but concomitant use of immunosuppressive medications can be continued [ 65 ]. RCI should be down-titrated or discontinued if severe AEs arise or if management of AEs fail [ 65 ].…”

Section: Introductionmentioning

confidence: 99%

“…Concomitant steroids are recommended to be quickly tapered in patients receiving RCI, but concomitant use of immunosuppressive medications can be continued [ 65 ]. RCI should be down-titrated or discontinued if severe AEs arise or if management of AEs fail [ 65 ]. This panel of experts concluded that most patients with pulmonary sarcoidosis in their practices were started on RCI as a second-line therapy after steroids (Fig.…”

Section: Introductionmentioning

confidence: 99%

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Repository Corticotropin Injection for the Treatment of Pulmonary Sarcoidosis: A Narrative Review

Mirsaeidi

Baughman

2022

Pulm Ther

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Although corticosteroids are the standard first-line therapy for pulmonary sarcoidosis, long-term and high-dose use of these drugs are associated with increased risk of adverse events and high healthcare utilization costs. Treatment guidelines for pulmonary sarcoidosis indicate that off-label immunomodulators and biologics may be warranted for severe disease. Repository corticotropin injection (RCI, Acthar ® Gel), a complex mixture of adrenocorticotropic hormone analogs and other pituitary peptides, is one of only two therapies approved by the US Food and Drug Administration for symptomatic pulmonary sarcoidosis and is recommended by current European Respiratory Society treatment guidelines for use on a case-by-case basis. With its unique anti-inflammatory and immunomodulatory mechanism of action through activation of melanocortin receptors in various cell types, RCI has demonstrated steroid-sparing properties. RCI has a long history of use in autoimmune and inflammatory disorders, with proven safety and efficacy for pulmonary sarcoidosis. In this narrative review, we present the clinical evidence for the safety and efficacy of RCI in the treatment of pulmonary sarcoidosis, identify where RCI falls within the current treatment guidelines, and describe the unique mechanism of action of RCI for promoting anti-inflammatory and immunomodulatory effects.

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Management of repository corticotrophin injection therapy for pulmonary sarcoidosis: a Delphi study

Cited by 13 publications

References 30 publications

Clinical phenotyping: role in treatment decisions in sarcoidosis

Clinical phenotyping: role in treatment decisions in sarcoidosis

A Comprehensive Review of Sarcoidosis Treatment for Pulmonologists

Repository Corticotropin Injection for the Treatment of Pulmonary Sarcoidosis: A Narrative Review

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