Treatment Of Sarcoidosis Research Articles

Drug-induced comorbidities in patients with sarcoidosis.

Purpose of reviewSarcoidosis is a chronic multisystemic inflammatory disease of unknown aetiology with a wide range of highly variable clinical manifestations and unpredictable disease course. Sarcoidosis patients may present with specific organ-related symptoms involving functional impairments, and less specific symptoms. The decision whether and when to treat a sarcoidosis patient with pharmacotherapy depends on two major factors: risk of organ failure and/or death and impairment of quality of life. This decision is complex and not standardized.Recent findingsGlucocorticoids (GCs) are recommended as initial treatment, when needed. Subsequent GC-sparing alternatives frequently follow. Comorbidities or adverse drug reactions (ADRs) from drugs used in sarcoidosis treatment are sometimes very hard to differentiate from symptoms associated with the disease itself, which may cause diagnostic dilemmas. An ideal approach to minimalize ADRs would involve genetic screening prior to prescribing certain ‘high-risk drugs’ and therapeutic drug monitoring during treatment. Pharmacogenomic testing aims to guide appropriate selection of medicines, with the potential of reducing unnecessary polypharmacy while improving clinical outcomes.SummaryA multidisciplinary approach to the management of sarcoidosis may avoid unnecessary ADRs. It is important to consider the possibility of drug-induced damage in sarcoidosis, especially if the clinical situation deteriorates after the introduction of a particular drug.

The treatment of sarcoidosis: translating the European respiratory guidelines into clinical practice.

Recently, the European Respiratory Society (ERS) developed new international guidelines for the treatment of sarcoidosis. This manuscript attempts to distill the ERS Sarcoidosis Treatment Guidelines to a manageable format that can be easily used by practitioners. The ERS Sarcoidosis Treatment Guidelines addressed the treatment of pulmonary, skin, cardiac, neurologic, and sarcoidosis-associated fatigue. Therapeutic drug dosing and treatment algorithms for these conditions were also addressed. Glucocorticoids were the initial recommended treatment for these conditions except for sarcoidosis-associated fatigue where a pulmonary exercise program or a neurostimulant was initially suggested. Because of the risk of glucocorticoid side-effects, the Guidelines recommended early consideration of glucocorticoid-sparing therapy including certain antimetabolites and two specific tumor necrosis alpha antagonists: infliximab and adalimumab. The ERS Sarcoidosis Treatment Guidelines used a rigorous GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology to update treatment recommendations for this condition. This manuscript summarizes the Guideline findings in practical terms for clinicians. Suggested algorithms and treatment dosing recommendations are provided.

Treatment of Pulmonary Sarcoidosis Using Allogenic Bone Marrow-Derived Mesenchymal Stem Cell Therapy is Safe: A Case Report

Mesenchymal stem cells have a proven potent immunomodulatory effect both in vitro and in vivo. We report a case of a 67-year-old male patient, first diagnosed with sarcoidosis in 2005, that responded well clinically and biologically to ImmunoARTTM (developed by Educell Ltd.) allogenic, HLA-incompatible, and non-related bone marrow-derived MSCs in a dose of 106/kg. The patient presented to St. Catherine Specialty Hospital in 2021 with an exacerbation of respiratory symptoms. After a clinical and radiological examination with laboratory workup, radiological findings were consistent with pulmonary sarcoidosis, while laboratory work revealed increased leucocytes at 14.2 g/L, CRP at 51.2 mg/L, and lymphocytes at 4.25 g/L. The patient was then administered intravenous application of MSCs on three occasions in the out-patient clinic. MSC doses were prepared from a young, healthy donor who agreed to donate bone marrow for allogeneic treatment and who was negative for viral markers (HBs Ag, HBc Ab, HCV Ab, HIV 1-2 Ab, TPHA, HBV NAT, HCV NAT, HIV NAT) according to EU legislation. Cells were prepared in a controlled and verified laboratory for “Hospital exemption” cell preparation in the cleanroom facility in safety cabinet class A and expressed CD105, CD 73, and CD 90 but lacked the expression of CD45 and CD34. Before the treatment, standard print and detailed verbal information were provided to patients undergoing treatment. Immediately after the informed consent form (ICF) was signed by the patient. Throughout MSC therapy, the patient showed an improvement clinically and biologically with a decrease in inflammatory parameters. Laboratory values were assessed on days 2, 5, and 7. On day 7, leucocytes were 11.2 g/L, CRP 5.1 mg/L, and lymphocytes 4.0 g/L. In the follow-up period, the patient felt subjectively better, without any side effects of the MSC therapy. Unfortunately, the patient dropped out from follow-up, therefore, the prolonged effects of this therapy were not able to be assessed. Therefore, systemic MSC therapy presents an opportunity to treat sarcoidosis that needs to be further researched.

Treatment of Pulmonary Sarcoidosis Using Allogenic Bone Marrow-Derived Mesenchymal Stem Cell Therapy is Safe: A Case Report

Treatment of Pulmonary Sarcoidosis Using Allogenic Bone Marrow-Derived Mesenchymal Stem Cell Therapy is Safe: A Case Report

Angiotensin-converting Enzyme Inhibitors in the Treatment of Sarcoidosis and Association with ACE Gene Polymorphism: Case Series

Angiotensin-converting enzyme (ACE) is used as a marker for sarcoid disease activity. We present an observational study of four African-American patients all of whom demonstrated improvement in their sarcoidosis after treatment with ACE inhibitors for hypertension.

Tuberculosis Lymphadenitis in a Well Managed Case of Sarcoidosis

Differentiation between tuberculosis (TB) and sarcoidoisis is sometimes extremely difficult. Sequential occurrence of sarcoidosis and TB in the same patient is uncommon. We present the case of a young man, with a proven diagnosis of sarcoidosis who later developed TB after completion of treatment for sarcoidosis. A 32-year-old male patient presented with low-grade fever since two months. Physical examination revealed cervical lymphadenopathy. Initial fine needle aspiration cytology (FNAC) of the cervical lymph node was suggestive of granulomatous inflammation; the chest radiograph was normal. Repeat FNAC from the same lymph node was suggestive of reactive lymphoid hyperplasia. The patient was treated with antibiotics and followed-up. He again presented with persistence of fever and lymphadenopathy and blurring of vision. Ophthalmological examination revealed uveitis, possibly due to a granulomatous cause. His repeat Mantoux test again was non-reactive; serum angiotensin converting enzyme (ACE) levels were raised. This time an excision biopsy of the lymph node was done which revealed discrete, non-caseating, reticulin rich granulomatous inflammation suggestive of sarcoidosis. The patient was treated with oral prednisolone and imporved symptomatically. Subsequently, nearly nine months after completion of corticosteroid treatment, he presented with low-grade, intermittent fever and a lymph node enlargement in the right parotid region. FNAC from this lymph node showed caseating granulomatous inflammation and the stain for acid-fast bacilli was positive. He was treated with Category I DOTS under the Revised National Tuberculosis Control Programme and improved significantly. The present case highlights the need for further research into the aetiology of TB and sarcoidosis.

Clinical features and visual outcomes of ocular sarcoidosis at a tertiary referral center in Tokyo.

To analyze clinical features, treatment, complications, and visual outcomes of ocular sarcoidosis at a tertiary center in Tokyo. Clinical records of 53 patients with ocular sarcoidosis ("definite" or "presumed") presenting between 2013 and 2018 to the Kyorin Eye Center were retrospectively reviewed. Diagnosis was based on the revised criteria of the International Workshop on Ocular Sarcoidosis. Definite (biopsy-proven) disease was present in 87% of patients and presumed disease in 13%. The mean age at presentation was 58years (13-81years) and 68% were women. The mean follow-up was 34months (6-70months). Forty-five patients (85%) had panuveitis, and the most common ocular clinical sign suggestive of ocular sarcoidosis was bilaterality (92%). Ocular complications were observed in 93 eyes (85%),most commonly cataract (73%), epiretinal membrane (24%), macular edema (24%) and glaucoma (19%). Thirty-one eyes (30%) underwent cataract surgery and 12 eyes (12%) underwent pars plana vitrectomy. Ten patients (19%) received systemic corticosteroid therapy and 33 eyes (32%) received periocular corticosteroid injections. The best-corrected visual acuity was 1.0 or better in 51% of eyes at presentation, 57% at 6months, 50% at 12months, and 58% at 36months. The majority of ocular sarcoidosis patients were women, had bilateral disease and panuveitis involvement. Most eyes maintained good visual acuity, although surgical interventions for cataract and epiretinal membrane were common.

CD103 Expression on Regulatory and Follicular T Cells in Lymph Nodes, Bronchoalveolar Lavage Fluid and Peripheral Blood of Sarcoidosis Patients.

(1) Background: Sarcoidosis is a chronic multisystem disorder of unknown aetiology, driven by a T-cell mechanism allowing T-cell attachment and transmigration through the endothelium, and endorsed by the expression of an integrin alpha-E beta-7 (CD103). This study aimed to analyse the different distribution and compartmentalisation of CD103 expression on T cell subsets in BAL, peripheral blood mononuclear cells (PBMC) and lymph nodes (LLN) from sarcoidosis patients. (2) Patients: We consecutively and prospectively enrolled 14 sarcoidosis patients. We collected PBMC, LLN and BAL at the same time from all patients. Through flow cytometric analysis, we analysed the expression of CD103 on regulatory and follicular T cell subsets. (3) Results: All patients were in radiological Scadding stage II. The multivariate analysis found that the variables which most influenced the peripheral blood compartment were high CD8+ and low ThReg, CD8+CD103+ and Tfh cell percentages. A principal component analysis plot performed to distinguish LLN, BAL and PBMC showed that they separated on the basis of CD4+, CD4+CD103+, CD8+, CD8+CD103+, TcEffector, TcNaive, ThNaive, ThEffector, Threg, ThregCD103+, Tfh, TcfCXC5+ and CD4+CD103+/CD4+ with 65.96% of the total variance. (4) Conclusions: Our study is the first to report a link between the imbalance in circulating, alveolar and lymph node CD8+ and CD8+CD103+ T cells, ThReg, Tfh and ThNaive and the CD103+CD4+/CD4+ T cell ratio in the development of sarcoidosis. These findings shine a spotlight on the pathogenesis of sarcoidosis and may offer new predictors for diagnosis. Our study provides additional understanding for a personalised, and hopefully more effective treatment of sarcoidosis.

Chronicles of A.I. Pospelov Moscow Society of Dermatovenerologists and Cosmetologists (MSDC was founded on October 4, 1891). Bulletin of the MSDC № 1142

On March 16, 2021, the 1142nd meeting of the Moscow Society of Dermatovenereologists and Cosmetologists named after A.I. Pospelov took place.
 Two people have been accepted as members of the MODV: the list of new members was approved unanimously.
 The agenda includes a discussion of two clinical cases. The interest in the first demonstration ― mushroom mycosis lies in the relative rarity of T-cell lymphomas in the skin clinic, as well as the effectiveness of PUVA therapy in the plaque stage of the disease. Another observation is a case of acquired epidermolysis bullosa, a rare sporadic autoimmune disease with increased skin injury, the formation of subepidermal blisters, the presence of antibodies against type 7 collagen, the prognosis for which is usually favorable and even with a possible spontaneous recovery.
 A number of scientific reports were made at the meeting. The first of them is on the problems of vulgar acne. The patient-oriented approach for vulgar acne implies individual, personalized treatment of the patient. Among the strategic directions in the development of therapeutic approaches to patients with vulgar acne, special attention, in addition to external remedies, systemic therapy, physiotherapy and cosmetic care, should be paid to the adherence of patients to treatment. Among the various technologies for determining the severity of vulgar acne, an interactive electronic DIA calculator developed at the Department of Skin Diseases of Sechenov University, combined with medicinal recommendations in accordance with a certain severity of the disease of a particular patient, deserves attention.
 The report on modern and classical methods of diagnosis of human and animal mycoses represents the most pressing issues of medical mycology in general and dermatomycology in particular due to their extreme prevalence. To date, all laboratory methods for the diagnosis of mycoses can be divided into classical and modern high-tech, while in most laboratories on the ground, almost only confocal microscopy has been introduced.
 The last report was devoted to dermatoses that developed at the site of tattoos and tattooing. Since the composition of the coloring pigment includes iron oxide, titanium dioxide, isopropyl alcohol, natural organic and inorganic pigments, dermatoses such as benign lymphoplasia (lymphocytoma), sarcoidosis, psoriasis, discoid lupus, vitiligo, vasculitis, lichen planus, annular granuloma, lipoid necrobiosis, melanoma may develop in places of skin damage, basal cell carcinoma, gangrenous pyoderma, mycosis, syphilis, tuberculosis. It is noted that the treatment of lymphocytoma and sarcoidosis is a difficult task.

Cutaneous Sarcoidosis: A Review and Approach to Treatment

Sarcoidosis is a multisystem, granulomatous, inflammatory condition. There are numerous clinical manifestations of cutaneous sarcoidosis, the most common being papular sarcoidosis, which presents as red-brown or violaceous papules on the face, trunk, or extremities. Cutaneous lesions of sarcoidosis can present similarly to other conditions such as psoriasis, lichen planus, nummular eczema, granuloma annulare, and cutaneous T-cell lymphoma. It is important that an accurate diagnosis is made when patients present with cutaneous lesions as sarcoidosis can affect multiple organ systems. A patient diagnosed with sarcoidosis will typically require comprehensive care with dermatology, rheumatology, pulmonology, and other specialties as needed. Clinicians often face difficulty determining the best and most effective treatment for cutaneous sarcoidosis while keeping side effects in mind. In this article, the author presents a clinical vignette of a patient with cutaneous sarcoidosis then reviews the disease process, diagnostic work up, and treatment/management.

Steroid-Resistant Central Nervous System Sarcoidosis: The Selections of Multi-Agent Combination as the Initial Treatment

Sarcoidosis is a granulomatous multiorgan disease of unknown etiology that commonly affects the respiratory system, eyes, and skin, and less commonly affects the nervous system. Because of its rarity, a standard treatment for central nervous system (CNS) sarcoidosis has not yet been established. Corticosteroids remain the cornerstone of CNS sarcoidosis treatment. However, CNS sarcoidosis, other than isolated facial nerve paralysis, is often refractory to treatment and requires long-term corticosteroid treatment. In particular, patients with hydrocephalus have a high mortality rate and a lack of response to this treatment. Therefore, immunosuppressants, including TNF-α inhibitors and corticosteroids, should be considered as the initial treatment. For older patients, it is important to pay attention to infection as an adverse event and to the toxicity of the therapeutic agents. Because steroid-related adverse events are more common in the older patient group, the lowest effective dose should be used, and the treatment duration should be kept as short as possible after careful evaluation of disease activity. Corticosteroid-sparing agents are effective at reducing the cumulative toxicity of corticosteroids. Recently, various new potential agents have emerged and their efficacy has been assessed. It is expected that more treatment options will be available for CNS sarcoidosis in future.

Understanding Predictors of Response to Repository Corticotropin Injection Treatment Among Patients With Advanced Symptomatic Sarcoidosis.

Background: Sarcoidosis, an inflammatory systemic granulomatous disease, affects multiple organs and has a diverse clinical course. Repository corticotropin injection (RCI) is an effective treatment for advanced symptomatic sarcoidosis. Since sarcoidosis affects patients differently, treatment response may vary by patient demographic, clinical, and treatment-related characteristics and physician specialty. However, there is a paucity of literature regarding predictors of sarcoidosis treatment response.Objectives: This study investigated predictors of response to RCI treatment.Methods: Post-hoc analysis was conducted using data from a previously published retrospective cross-sectional chart review study among symptomatic sarcoidosis patients ≥18 years of age previously treated with RCI. Outcome improvement 3 months post-RCI treatment was based on the clinician’s subjective evaluation and analyzed using adjusted logistic regression. The most influential predictors for each outcome were based on statistical significance (P<.05) and the strength of the relationship assessed by the standardized β coefficients.Results: The top predictors of outcome improvements were as follows. Global health assessment: (1) improvement in current health status influenced by complete RCI compliance, moderate overall symptom severity, and presence of extrapulmonary sites; and (2) improvement in overall symptoms influenced by age, shorter duration since sarcoidosis diagnosis, and complete RCI compliance. Clinical outcomes: (1) lung function improvement influenced by mild weight loss, mild wheezing/coughing, and non–African American race; (2) reduction in pulmonary fibrosis influenced by moderate overall symptom severity, mild wheezing/coughing, and mild weight loss; and (3) reduction in inflammation influenced by physician specialty, completing a course of RCI treatment, and moderate-to-severe night sweats. Patient-related outcomes: (1) reduction in fatigue influenced by physician specialty and moderate-to-severe fatigue; and (2) improvement in quality-of-life influenced by shorter duration since sarcoidosis diagnosis, moderate-to-severe wheezing/coughing, and complete RCI compliance. Corticosteroid discontinuation/reduction was influenced by physician specialty, moderate-to-severe shortness of breath, and comedication use before RCI.Conclusions: RCI may be a better treatment option for patients with more severe disease, primarily those presenting with symptoms. Complete compliance with RCI treatment may improve patients’ health and quality of life. Understanding factors that influence RCI effectiveness across different treatment outcomes in real-world clinical practice is important for designing optimal sarcoidosis treatment strategies.

New sarcoidosis guidelines: Are we near to perfection?

New sarcoidosis guidelines: Are we near to perfection?

Phenotypic and HLA-DRB1 allele characterization of Swedish cardiac sarcoidosis patients

BackgroundEarly detection and initiation of treatment in cardiac sarcoidosis (CS) is believed to be crucial to reduce morbidity and mortality. The diagnosis of CS is challenging, especially in isolated CS (ICS). Certain human leukocyte antigen (HLA-DRB1) alleles associate with different phenotypes of sarcoidosis. Phenotypic and genotypic characterization of patients with CS may improve our ability to identify patients being at risk for developing CS. Methods87 patients with CS, identified at two Swedish university hospitals were included. Phenotypic characteristics were extracted from the medical records and the patients were HLA-DRB1 typed. ResultsMedian age at diagnosis was 55 years, 37% were women. HLA-DRB1 distribution was similar to a general sarcoidosis population. A majority of patients (51/87) had CS as the first sarcoidosis presentation. They were younger (p = 0.04), more often presenting with ventricular tachycardia (VT) or atrioventricular block (AVB) grade II or III (p < 0.001), had lower left ventricular ejection fraction (LVEF) (p = 0.002), lower serum angiotensin converting enzyme (s-ACE) (p = 0.025), and fewer extra cardiac manifestations (ECM) (p = 0.02) than those presenting with CS later. ConclusionsOf Swedish CS patients, 59% presented with cardiac involvement as first manifestation. They had more severe cardiac symptoms than patients presenting with CS later. This phenotype disclosed less ECM and lower s-ACE thus diagnosis can be missed or delayed. We did not observe significant differences in HLA-DRB1 allele frequency between patients with CS compared to sarcoidosis in general. Awareness of CS as a primary manifestation can enable early detection and adequate intervention.

Combination Therapy With Mycophenolate Mofetil And Prednisone In The Treatment Of Cardiac Sarcoidosis

Introduction Long-term immunosuppression is the mainstay of treatment for cardiac sarcoidosis (CS). While steroids are the cornerstone of therapy, steroid-sparing alternatives are considered second-line or adjunctive in the management of CS. We aimed to evaluate the efficacy of mycophenolate mofetil and steroids on the clinical and radiographic outcomes of patients with CS. Methods A retrospective chart review at a single academic medical center of patients with CS that were treated with prednisone or combination therapy with prednisone and MMF was conducted between 2019-2021. Baseline demographics, clinical data, echocardiography and PET data was collected pre and post-treatment. Improvement in disease was based on improvement in left ventricular ejection fraction (LVEF), reduction of arrhythmia burden, and regional maximum Standardized Uptake Value and percentage of inflammation on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). The parameters were then compared between the MMF and non- MMF group. Results There were 19 total patients (63% male and 52% black that were found to have CS- of which 7 had a positive cardiac or extracardiac biopsy. 17 patients were treated with combination therapy consisting of prednisone and MMF. Median follow-up between scans was 154 days in MMF group and 156.5 days in the non-MMF group. In the MMF group, there was an improvement in EF with a median of 6.5 (p 0.22), regional max SUV decreased by 1.35 (p <0.05) and percent inflammation decreased by a median of 22 percentage points (p 0.11). Of the 14 patients in the MMF group that had an underlying arrhythmia, 11 (78%) had a mitigated or absent arrhythmia burden. In the non-MMF group, the LVEF decreased by a median of 9 (p 0.22), regional max SUV was increased to 1.9 (p <0.05) and percent inflammation decreased by a median of 2 percentage points. 1 of the 2 patients in the non-MMF group showed an improvement in arrhythmia burden. Conclusion CS patients treated with combination therapy consisting of prednisone and MMF appeared to have radiographic and clinical evidence of improvement. MMF may be considered an effective adjunctive therapy to prednisone alone. Long-term immunosuppression is the mainstay of treatment for cardiac sarcoidosis (CS). While steroids are the cornerstone of therapy, steroid-sparing alternatives are considered second-line or adjunctive in the management of CS. We aimed to evaluate the efficacy of mycophenolate mofetil and steroids on the clinical and radiographic outcomes of patients with CS. A retrospective chart review at a single academic medical center of patients with CS that were treated with prednisone or combination therapy with prednisone and MMF was conducted between 2019-2021. Baseline demographics, clinical data, echocardiography and PET data was collected pre and post-treatment. Improvement in disease was based on improvement in left ventricular ejection fraction (LVEF), reduction of arrhythmia burden, and regional maximum Standardized Uptake Value and percentage of inflammation on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET). The parameters were then compared between the MMF and non- MMF group. There were 19 total patients (63% male and 52% black that were found to have CS- of which 7 had a positive cardiac or extracardiac biopsy. 17 patients were treated with combination therapy consisting of prednisone and MMF. Median follow-up between scans was 154 days in MMF group and 156.5 days in the non-MMF group. In the MMF group, there was an improvement in EF with a median of 6.5 (p 0.22), regional max SUV decreased by 1.35 (p <0.05) and percent inflammation decreased by a median of 22 percentage points (p 0.11). Of the 14 patients in the MMF group that had an underlying arrhythmia, 11 (78%) had a mitigated or absent arrhythmia burden. In the non-MMF group, the LVEF decreased by a median of 9 (p 0.22), regional max SUV was increased to 1.9 (p <0.05) and percent inflammation decreased by a median of 2 percentage points. 1 of the 2 patients in the non-MMF group showed an improvement in arrhythmia burden. CS patients treated with combination therapy consisting of prednisone and MMF appeared to have radiographic and clinical evidence of improvement. MMF may be considered an effective adjunctive therapy to prednisone alone.

State-of-the-Art Treatments for Sarcoidosis.

Sarcoidosis is a heterogeneous disease with various treatment indications. Although it affects mainly the lungs, sarcoidosis can affect every organ, especially when the disease course is chronic and protracted. Regular patient follow-up is recommended for early recognition of active, ongoing organ-specific granulomatous inflammation to avoid progression to irreversible fibrosis. In this review, we elaborate on treatment indications and various anti-sarcoidosis regimens proven useful in clinical trials. We also review specialized treatment of specific disease manifestations, with a focus on cardiac sarcoidosis. We also report on treatment for special conditions such as fatigue and small fiber neuropathy. Treatment for sarcoidosis is an emerging landscape, with new data complementing the existing knowledge.

Prognostic features of sarcoidosis course in a Brazilian cohort.

ABSTRACTObjective:To identify predictive features associated with the course of sarcoidosis at initial evaluation and to develop a predictive score. Methods:This was a retrospective study involving pulmonary sarcoidosis patients, classified as having a self-limited or persistent course of disease, comparing data between the outcomes by univariate analysis. Features related to persistent disease were selected by multivariate analysis and a prognostic score was designed. Results:The sample comprised 200 patients (mean age = 49 years). The median duration of symptoms to diagnosis was 12 months, and delayed diagnosis (> 12 months) was found in 43% of the cases. The most common radiological stage was II; 37% had reduced FVC. Relevant systemic involvement was detected in 37% of the patients. Treatment for tuberculosis was prescribed in 44 patients prior to sarcoidosis diagnosis. Treatment for sarcoidosis was required in 77% of the sample, and the disease course was persistent in 115 cases. Excluding 40 patients with fibrotic disease, prognostic factors to persistent disease were parenchymal involvement, delayed diagnosis, dyspnea, relevant systemic involvement, and reduced FVC. On the basis of the analysis, a 3-letter scoring system (A, B and C) was developed according to the selected factors. The positive predictive values for persistent course for A (≤ 1 point) and C scores (≥ 4 points) were 12.5% and 81.8%, respectively. Conclusions:A score can be derived by selected features at initial evaluation, allowing the prediction of outcomes in a significant number of sarcoidosis patients.

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

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.

Treatment of Sarcoidosis in US Rheumatology Practices: Data From the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) Registry.

Sarcoidosis is often treated with glucocorticoids, although the use of biologics is growing. Prescribing patterns for biologics for patients with sarcoidosis in US rheumatology practices have never been examined. Given that there are no steroid-sparing US Food and Drug Administration-approved therapies for sarcoidosis, we sought to characterize the real-world treatment of sarcoidosis and to assess practice-level variation in prescribing patterns. We conducted an observational study of patients with sarcoidosis using data from the Rheumatology Informatics System for Effectiveness (RISE) registry (2014-2018). The RISE registry represents an estimated 32% of the US clinical rheumatology workforce. Adult patients with ≥2 codes for sarcoidosis ≥30 days apart were included. We examined sarcoidosis-specific medication use at any time during the study period. Data were analyzed at the practice level. A total of 3,276 patients with sarcoidosis from 184 practices were included. Of those patients, 75.1% were women, with a mean age of 59.0 ± 12.5 years; 48.3% were White and 27.6% were Black. Overall, 59.3% of patients were prescribed glucocorticoids, and 24.7% received prolonged glucocorticoid therapy (≥10 mg/day for ≥90 days). In all, 12.1% received a biologic or targeted synthetic disease-modifying antirheumatic drug (tsDMARD), most commonly tumor necrosis factor inhibitors. There was wide practice-level variation among 31 practices with ≥30 patients with sarcoidosis; biologic use ranged from 15.6% to 69.2%. Infliximab represented the most common biologic prescribed. In a large sample of US rheumatology practices, 12.1% of patients with sarcoidosis received biologics or tsDMARDs. We found high variability in biologic use across practices. The significant use of long-term glucocorticoids suggests unmet therapeutic needs in this patient population.

НОВІ ПІДХОДИ ДО ВЕДЕННЯ ХВОРИХ НА САРКОЇДОЗ ЛЕГЕНЬ

Sarcoidosis is an inflammatory multi-systemic disease of unknown origin with wide spectrum of clinical manifestations. Sarcoidosis may affect practically any organ — predominantly lungs, lymphatic system, skin, eyes alone or in combination. The disease is characterized by formation of non-caseous granuloma. In 1999 American Thoracic society (ATS), European Respiratory society (ERS) and World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) adopted an international consensus statement on diagnosis and treatment of sarcoidosis «Statement on Sarcoidosis». Based on ATS, ERS and WASOG 1999 Statement the national documents such as “Evidence-based adapted clinical guideline on sarcoidosis” and “Unified clinical protocol of primary, secondary (specialized) and tertiary (highly-specialized) medical care for sarcoidosis” approved by MOH of Ukraine (decree # 634 dated 08 Sep 2014) were developed by current authors in Ukraine. In 2020 ATS experts published an updated «Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline», according to which the diagnosis of sarcoidosis was based on 3 criteria: compatible clinical symptoms, presence of non-necrotizing granulomatous inflammation in one or more tissue samples (not always required) and exclusion of alternative causes of granulomatous disorder. Finally, in 2021 ERS task force report was published “ERS clinical practice guidelines on treatment of sarcoidosis”, which outlined completely new approaches to management of sarcoidosis patients. According to this document, a decision about treatment of patient depends on 2 major factors: risk of death or organ failure and deterioration of quality of life of a patient. In addition, main principle of management of sarcoidosis patients is a balance between: a) minimization of risk of disability or death due to lung injury and decrease of quality of life, and b) risk of comorbidity and reduction of quality of life due to corticosteroid or other therapies. Current review focuses on analysis of major statements of these documents, disputable questions of initial therapy choice in patients with pulmonary sarcoidosis. Key words: pulmonary sarcoidosis, diagnosis, treatment, glucocorticosteroids, immunosuppressants, cytokine inhibitors.