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Aktuelle Rheumatologie
DOI: 10.1055/a-2414-1063
Original Article

The Management of Patients with Extrapulmonary Sarcoidosis in Rheumatology Clinic

Das Management von Patienten mit extrapulmonaler Sarkoidose in der Rheumatologie-Klinik
Semih Gülle
1   Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
,
Mert Can Ataca
2   Internal Medicine, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
,
İsmail Sarı
1   Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
,
Merih Birlik
1   Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
,
Gercek Can
1   Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
,
Fatoş Önen
1   Rheumatology, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
› Author Affiliations Fundings The authors received no financial support for the research and/or authorship of this article.
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Abstract

Background This study aimed to evaluate patients with extrapulmonary and refractory sarcoidosis who were followed up on in our rheumatology clinic for a long follow-up period and to summarise the applications in rheumatology practice.

Materials and Methods The study included patients diagnosed with sarcoidosis at our institution between 2010 and 2022. Patientsʼ age, sarcoidosis diagnosis dates, inflammatory rheumatic disease (iRMD) diagnoses, autoimmune markers, and imaging findings were all evaluated. Estimates were made for cases of glucocorticoid (GC) use and cumulative and mean daily GC exposures.

Results 90 patients with sarcoidosis, 79 (87.8) female and 11 (12.2) male, whose mean age was 56.4±12.4 years, were included. Since 48 (53.3%) of the patients required at least one additional treatment in addition to CS, they were classified as having refractory sarcoidosis and followed up accordingly. While 43 (89%) of the patients received second-line sarcoidosis treatment, it was found that 5 patients were switched to third-line sarcoidosis treatment (p<0.001). Comorbidity distributions of refractory and nonrefractory patients were similar. It was determined that 9 (10%) patients had a history of osteoporotic fracture.

Conclusions In conclusion, it appears critical to develop a detailed patient management plan and a multidisciplinary approach in the early stages of extrapulmonary sarcoidosis management. There is currently no detailed algorithm covering refractory disease definition and treatment practice differences.

Keywords

sarcoidosis - glucocorticoid - iRMD - osteoporosis - TNFi

Schlüsselwörter

iRMD - TNFi - Sarkoidose - Glukokortikoid - Osteoporose


Publication History

Article published online:
24 October 2024

© 2024. Thieme. All rights reserved.

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  • References

  • 1 Jain R, Yadav D, Puranik N. et al. Sarcoidosis: Causes, Diagnosis, Clinical Features, and Treatments. J Clin Med 2020; 9
    CrossrefPubMedSearch in Google Scholar
  • 2 Judson MA, Boan AD, Lackland DT. The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 2012; 29: 119-127
    PubMedSearch in Google Scholar
  • 3 Baughman RP, Field S, Costabel U. et al. Sarcoidosis in America. Analysis Based on Health Care Use. Ann Am Thorac Soc 2016; 13: 1244-1252
    CrossrefPubMedSearch in Google Scholar
  • 4 Arkema EV, Grunewald J, Kullberg S. et al. Sarcoidosis incidence and prevalence: a nationwide register-based assessment in Sweden. Eur Respir J 2016; 48: 1690-1699
    CrossrefPubMedSearch in Google Scholar
  • 5 Sakthivel P, Bruder D. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol 2017; 24: 59-65
    CrossrefPubMedSearch in Google Scholar
  • 6 Grunewald J, Grutters JC, Arkema EV. et al. Sarcoidosis. Nat Rev Dis Prim 2019; 5: 45
    CrossrefPubMedSearch in Google Scholar
  • 7 Kaiser Y, Eklund A, Grunewald J. Moving target: shifting the focus to pulmonary sarcoidosis as an autoimmune spectrum disorder. Eur Respir J 2019; 54: 1802153
    CrossrefPubMedSearch in Google Scholar
  • 8 Hammam N, Evans M, Morgan E. et al. Treatment of Sarcoidosis in Rheumatology Practices: Data From the American College of Rheumatologyʼs Rheumatology Informatics System for Effectiveness Registry. Arthritis Care Res (Hoboken) 2022; 74: 371-376
    CrossrefPubMedSearch in Google Scholar
  • 9 Rizzato G, Palmieri G, Agrati AM. et al. The organ-specific extrapulmonary presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 2004; 21: 119-126
    PubMedSearch in Google Scholar
  • 10 Hu X, Carmona EM, Yi ES. et al. Causes of death in patients with chronic sarcoidosis. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 2016; 33: 275-280
    PubMedSearch in Google Scholar
  • 11 Judson MA. The Diagnosis of Sarcoidosis. Clin Chest Med 2008; 29: 415-427
    CrossrefPubMedSearch in Google Scholar
  • 12 Judson MA, Baughman RP, Teirstein AS. et al. Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. ACCESS Research Group. A Case Control Etiologic Study of Sarcoidosis. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 1999; 16: 75-86
    PubMedSearch in Google Scholar
  • 13 Korsten P, Strohmayer K, Baughman RP. et al. Refractory Pulmonary Sarcoidosis. Clin Pulm Med 2016; 23: 67-75
    CrossrefPubMedSearch in Google Scholar
  • 14 Soto-Gomez N, Peters JI, Nambiar AM. Diagnosis and Management of Sarcoidosis. Am Fam Physician 2016; 93: 840-848
    PubMedSearch in Google Scholar
  • 15 Hunninghake GW, Costabel U, Ando M. et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 1999; 16: 149-173
    PubMedSearch in Google Scholar
  • 16 Baughman RP, Teirstein AS, Judson MA. et al. Clinical Characteristics of Patients in a Case Control Study of Sarcoidosis. Am J Respir Crit Care Med 2001; 164: 1885-1889
    CrossrefPubMedSearch in Google Scholar
  • 17 De Vries F, Bracke M, Leufkens HGM. et al. Fracture risk with intermittent high-dose oral glucocorticoid therapy. Arthritis Rheum 2007; 56: 208-214
    CrossrefPubMedSearch in Google Scholar
  • 18 Sweiss NJ, Lower EE, Mirsaeidi M. et al. Rituximab in the treatment of refractory pulmonary sarcoidosis. Eur Respir J 2014; 43: 1525-1528
    CrossrefPubMedSearch in Google Scholar
  • 19 Pereira CAC, Dornfeld MC, Baughman R. et al. Clinical phenotypes in sarcoidosis. Curr Opin Pulm Med 2014; 20: 496-502
    CrossrefPubMedSearch in Google Scholar
  • 20 Arkema EV, Cozier YC. Epidemiology of sarcoidosis: current findings and future directions. Ther Adv Chronic Dis 2018; 9: 227-240
    CrossrefPubMedSearch in Google Scholar
  • 21 Valeyre D, Prasse A, Nunes H. et al. Sarcoidosis. Lancet 2014; 383: 1155-1167
    CrossrefPubMedSearch in Google Scholar
  • 22 Sellarés J, Francesqui J, Llabres M. et al. Current treatment of sarcoidosis. Curr Opin Pulm Med 2020; 26: 591-597
    CrossrefPubMedSearch in Google Scholar
  • 23 McKinzie BP, Bullington WM, Mazur JE. et al. Efficacy of Short-Course, Low-Dose Corticosteroid Therapy for Acute Pulmonary Sarcoidosis Exacerbations. Am J Med Sci 2010; 339: 1-4
    CrossrefPubMedSearch in Google Scholar
  • 24 Schutt AC, Bullington WM, Judson MA. Pharmacotherapy for pulmonary sarcoidosis: A delphi consensus study. Respir Med 2010; 104: 717-723
    CrossrefPubMedSearch in Google Scholar
  • 25 Ussavarungsi K, Gerke AK. Approach to tapering antisarcoidosis therapy. Curr Opin Pulm Med 2019; 25: 526-532
    CrossrefPubMedSearch in Google Scholar
  • 26 Grutters JC, van den Bosch JMM. Corticosteroid treatment in sarcoidosis. Eur Respir J 2006; 28: 627-636
    CrossrefPubMedSearch in Google Scholar
  • 27 Al-Kofahi K, Korsten P, Ascoli C. et al. Management of extrapulmonary sarcoidosis: challenges and solutions. Ther Clin Risk Manag 2016; 12: 1623-1634
    CrossrefPubMedSearch in Google Scholar
  • 28 Rice JB, White A, Lopez A. et al. High-Cost Sarcoidosis Patients in the United States: Patient Characteristics and Patterns of Health Care Resource Utilization. J Manag Care Spec Pharm 2017; 23: 1261-1269
    CrossrefPubMedSearch in Google Scholar
  • 29 Fang C, Zhang Q, Wang N. et al. Effectiveness and tolerability of methotrexate in pulmonary sarcoidosis: A single center real-world study. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG 2019; 36: 217-227
    CrossrefPubMedSearch in Google Scholar
  • 30 Vorselaars ADM, Wuyts WA, Vorselaars VMM. et al. Methotrexate vs Azathioprine in Second-line Therapy of Sarcoidosis. Chest 2013; 144: 805-812
    CrossrefPubMedSearch in Google Scholar
  • 31 Bitoun S, Bouvry D, Borie R. et al. Treatment of neurosarcoidosis. Neurology 2016; 87: 2517-2521
    CrossrefPubMedSearch in Google Scholar
  • 32 Moravan M, Segal BM. Treatment of CNS sarcoidosis with infliximab and mycophenolate mofetil. Neurology 2009; 72: 337-340
    CrossrefPubMedSearch in Google Scholar
  • 33 Sakkat A, Cox G, Khalidi N. et al. Infliximab therapy in refractory sarcoidosis: a multicenter real-world analysis. Respir Res 2022; 23: 54
    CrossrefPubMedSearch in Google Scholar
  • 34 James WE, Baughman R. Treatment of sarcoidosis: grading the evidence. Expert Rev Clin Pharmacol 2018; 11: 677-687
    CrossrefPubMedSearch in Google Scholar
  • 35 Alnaimat F, Al Oweidat K, Alrwahdeh A. et al. Sarcoidosis in Jordan: A Study of the Clinical Phenotype and Disease Outcome. Arch Rheumatol 2020; 35: 226-238
    CrossrefPubMedSearch in Google Scholar
  • 36 Soto-Gomez N, Peters JI, Nambiar AM. Diagnosis and management of sarcoidosis. Am Fam Physician 2016; 93: 840-848
    PubMedSearch in Google Scholar
  • 37 Bussinguer M, Danielian A, Sharma OP. Cardiac Sarcoidosis: Diagnosis and Management. Curr Treat Options Cardiovasc Med 2012; 14: 652-664
    CrossrefPubMedSearch in Google Scholar
  • 38 Krause ML, Cooper LT, Chareonthaitawee P. et al. Successful use of rituximab in refractory cardiac sarcoidosis. Rheumatology 2016; 55: 189-191
    CrossrefPubMedSearch in Google Scholar
  • 39 Ho JSY, Chilvers ER, Thillai M. Cardiac sarcoidosis – an expert review for the chest physician. Expert Rev Respir Med 2019; 13: 507-520
    CrossrefPubMedSearch in Google Scholar