Arthritis und Rheuma 2022; 42(05): 337-344
DOI: 10.1055/a-1864-8212
Schwerpunkt

Pulmonale Sarkoidose

Pulmonary sarcoidosis
Raoul Bergner
1   Medizinische Klinik A – Nephrologie, Rheumatologie, Hämato-Onkologie, Klinikum der Stadt Ludwigshafen gGmbH, Ludwigshafen
,
Yannick Forst
2   Medizinische Klinik B – Kardiologie, Pneumologie, Angiologie, Klinikum der Stadt Ludwigshafen gGmbH, Ludwigshafen
› Author Affiliations

ZUSAMMENFASSUNG

Die Sarkoidose ist in Nordeuropa die häufigste granulomatöse Erkrankung. Sie kann prinzipiell nahezu alle Organe betreffen. Die Lunge ist jedoch mit 90–95 % das am häufigsten betroffene Organ. Die pulmonale Sarkoidose kann von einem asymptomatischen Befall bis hin zu einer interstitiellen Fibrose mit schwer eingeschränkter Lungenfunktion auftreten. Mit dem konventionellen Röntgen wird die pulmonale Sarkoidose in 4 Typen unterteilt. Neben der Lungenfunktion ist der radiologische Befallstyp mit entscheidend für die Therapieindikation. Wichtige Bausteine in der Diagnostik sind neben der radiologischen Bildgebung die Bestimmung der Lungenfunktion sowie die Bronchoskopie zur histologischen Diagnosesicherung. Daneben muss bei möglichem Multiorganbefall eine umfangreiche Organdiagnostik zur Erfassung weiterer Organmanifestationen erfolgen. Die Therapie besteht an erster Stelle aus der Gabe von Glukokortikoiden sowie bei unzureichender Wirkung oder Steroid-induzierten Nebenwirkungen aus der zusätzlichen Gabe von Immunsuppressiva. Hier steht an vorderster Stelle eine Therapie mit Methotrexat (MTX).

ABSTRACT

Sarcoidosis is the most common granulomatous disease in Northern Europe. In principle, it can affect almost all organs. However, the lung is the most frequently affected organ at 90–95 %. Pulmonary sarcoidosis can range from asymptomatic involvement to interstitial fibrosis with severely impaired lung function. With conventional X-rays, pulmonary sarcoidosis is divided into 4 types. In addition to the lung function, the type of radiological involvement is also decisive for the indication for therapy. In addition to radiological imaging, important components in diagnostics are the determination of lung function and bronchoscopy for histological confirmation of the diagnosis. In addition, in the event of possible multi-organ involvement, extensive organ diagnostics must be carried out to record further organ manifestations. The therapy consists primarily of the administration of glucocorticoids and, in the case of insufficient effect or steroid-induced side effects, of the additional administration of immunosuppressants. Therapy with MTX is the first choice.



Publication History

Article published online:
05 November 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • Literatur

  • 1 Bergner R, Groot K de, Müller GA, Korsten P. Frequency of organ manifestations in chronic sarcoidosis. Annals of the Rheumatic Diseases 2017; 76: 721 LP-721 Im Internet http://ard.bmj.com/content/76/Suppl_2/721.2.abstract
  • 2 Hillerdal G, Nöu E, Ostermann K, Schmekel B. Sarcoidosis: epidemiology and prognosis. A 15-year European study. American Review of Respiratory Diseases 1984; 130: 29-32 Im Internet https://www.atsjournals.org/doi/pdf/10.1164/arrd.1984.130.1.29
  • 3 Milman N, Selroos O. Pulmonary sarcoidosis in the Nordic countries 1950–1982 Epidemiology and clinical picture. Sarcoidosis 1990; 07: 50-57
  • 4 Arkema EV, Grunewald J, Kullberg S. et al Sarcoidosis incidence and prevalence : a nationwide register-based assessment in Sweden. European Respiratory Journal 2016; 48: 1690-1699 Im Internet https://erj.ersjournals.com/content/48/6/1690
  • 5 Dumas O, Abramovitz L, Wiley AS. et al Epidemiology of sarcoidosis in a prospective cohort study of U. S. Women. Annals of the American Thoracic Society 2016; 13: 67-71
  • 6 Ungprasert P, Carmona EM, Utz JP. et al Epidemiology of Sarcoidosis 1946–2013 A Population Based Study. Mayo Clinical Proceedings 2016; 91: 183-188
  • 7 Pietinalho A, Hiraga Y, Hosoda Y. et al The frequency of sarcoidosis in Finland and Hokkaido, Japan. A comparative epidemiological study. Sarcoidosis 1995; 12: 61-67
  • 8 Yoon H, Song J, Kim Y. Sarcoidosis in South Korea: Analysis of prevalence and incidence by a nationwide population-based study. American Journal of Respiratory and Critical Care Medicine 2018; 197: 1-8 Im Internet https://www.atsjournals.org/doi/abs/10.1164/ajrccm-conference.2018.197.1_MeetingAbstracts.A4926
  • 9 Jeon MH, Kang T, Yoo SH. et al The incidence, comorbidity and mortality of sarcoidosis in Korea, 2008–2015 A nationwide population-based study. Sarcoidosis Vasculitis and Diffuse Lung Diseases 2020; 37: 24-36
  • 10 Arkema EV, Cozier YC. Sarcoidosis epidemiology: recent estimates of incidence, prevalence and risk factors. Current Opinion in Pulmonary Medicine 2020; 26: 527-534 Im Internet https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7755458/pdf/nihms-1651324.pdf
  • 11 Ehrenstein B, Brochhausen C. Differenzialdiagnose granulomatöser Erkrankungen. Zeitschrift fur Rheumatologie 2017; 76: 415-424
  • 12 Drent M, Crouser ED, Grunewald J. Challenges of Sarcoidosis and Its Management. New England Journal of Medicine 2021; 385: 1018-1032
  • 13 Gilbert S, Steinbrech DS, Landas SK, Hunninghake GW. Amounts of angiotensin-converting enzyme mRNA reflect the burden of granulomas in granulomatous lung disease. American Review of Respiratory Disease 1993; 148: 483-486
  • 14 Tomita H, Ina Y, Sugiura Y. et al Polymorphism in the angiotensin-converting enzyme (ACE) gene and sarcoidosis. American Journal of Respiratory and Critical Care Medicine 1997; 156: 255-259
  • 15 Vorselaars ADM, Van Moorsel CHM, Zanen P. et al ACE and sIL-2R correlate with lung function improvement in sarcoidosis during methotrexate therapy. Respiratory Medicine 2015; 109: 279-285 Im Internet DOI: 10.1016/j.rmed.2014.11.009.
  • 16 Miyoshi S, Hamada H, Kadowaki T. et al Comparative evaluation of serum markers in pulmonary sarcoidosis. Chest 2010; 137: 1391-1397 Im Internet DOI: 10.1378/chest.09-1975.
  • 17 Damoiseaux J. The IL-2 – IL-2 receptor pathway in health and disease: The role of the soluble IL-2 receptor. Clinical Immunology 2020; 218: 108515 Im Internet DOI: 10.1016/j.clim.2020.108515.
  • 18 Eurelings LEM, Miedema JR, Dalm VASH. et al Sensitivity and specificity of serum soluble interleukin-2 receptor for diagnosing sarcoidosis in a population of patients suspected of sarcoidosis. PLoS ONE 2019; 14: 1-15 Im Internet DOI: 10.1371/journal.pone.0223897.
  • 19 Miyata J, Ogawa T, Tagami Y. et al Serum soluble interleukin-2 receptor level is a predictive marker for EBUS-TBNA-based diagnosis of sarcoidosis. Sarcoidosis Vasculitis and Diffuse Lung Diseases 2020; 37: 8-16
  • 20 Blaschke E, Eklund A, Persson U. Relationship between serum neopterin and lymphocytic alveolitis in sarcoidosis. Sarcoidosis 1988; 05: 25-30
  • 21 Homolka J, Lorenz J, Zuchold HD, Müller-Quernheim J. Evaluation of soluble CD 14 and neopterin as serum parameters of the inflammatory activity of pulmonary sarcoidosis. The Clinical Investigator 1992; 70: 909-916
  • 22 Reichel H, Koeffler HP, Tobler A, Norman AW. 1α,25-Dihydroxyvitamin D3 inhibits γ-interferon synthesis by normal human peripheral blood lymphocytes. Proceedings of the National Academy of Sciences of the United States of America 1987; 84: 3385-3389
  • 23 Dusso AS, Kamimura S, Gallieni M. et al γ-interferon-induced resistance to 1,25-(OH)2 D3 in human monocytes and macrophages: A mechanism for the hypercalcemia of various granulomatoses. Journal of Clinical Endocrinology and Metabolism 1997; 82: 2222-2232
  • 24 Monkawa T, Yoshida T, Hayashi M, Saruta T. Identification of 25-hydroxyvitamin D3 1α-hydroxylase gene expression in macrophages. Kidney International 2000; 58: 559-568
  • 25 Scadding JG. Prognosis of Intrathoracic Sarcoidosis in England. British medical journal 1961; 02: 1165-1172 Im Internet https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1970202/pdf/brmedj03022-0031.pdf
  • 26 Goyal A, Gupta D, Agarwal R. et al Value of different bronchoscopic sampling techniques in diagnosis of sarcoidosis: A prospective study of 151 patients. Journal of Bronchology and Interventional Pulmonology 2014; 21: 220-226
  • 27 Benzaquen S, Aragaki-Nakahodo AA. Bronchoscopic modalities to diagnose sarcoidosis. Current Opinion in Pulmonary Medicine 2017; 23: 433-438
  • 28 Dziedzic DA, Peryt A, Orlowski T. The role of EBUS-TBNA and standard bronchoscopic modalities in the diagnosis of sarcoidosis. Clinical Respiratory Journal 2017; 11: 58-63
  • 29 Pedro C, Melo N, EBastos HN. et al Role of bronchoscopic techniques in the diagnosis of thoracic sarcoidosis. Journal of Clinical Medicine 2019; 08: 1327
  • 30 Belperio JA, Shaikh F, Abtin FG. et al Diagnosis and Treatment of Pulmonary Sarcoidosis: A Review. JAMA – Journal of the American Medical Association 2022; 327: 856-867
  • 31 Agarwal R, Srinivasan A, Aggarwal AN, Gupta D. Efficacy and safety of convex probe EBUS-TBNA in sarcoidosis: A systematic review and meta-analysis. Respiratory Medicine 2012; 106: 883-892 Im Internet DOI: 10.1016/j.rmed.2012.02.014.
  • 32 Plit M, Pearson R, Havryk A. et al Diagnostic utility of endobronchial ultrasound-guided transbronchial needle aspiration compared with transbronchial and endobronchial biopsy for suspected sarcoidosis. Internal Medicine Journal 2012; 42: 434-438
  • 33 Costabel U, Bonella F, Ohshimo S, Guzman J. Diagnostic Modalities in Sarcoidosis: BAL, EBUS, and PET. Seminars in Respiratory and Critical Care Medicine 2010; 31: 404-408
  • 34 Danila E, Jurgauskiene L, Malickaite R. BAL fluid cells and pulmonary function in different radiographic stages of newly diagnosed sarcoidosis. Advances in Medical Sciences 2008; 53: 228-233
  • 35 Morgenthau AS, Teirstein AS. Sarcoidosis of the upper and lower airways. Expert Review of Respiratory Medicine 2011; 05: 823-833 Im Internet DOI: 10.1586/ers.11.66.
  • 36 Polychronopoulos VS, Prakash UBS. Airway involvement in sarcoidosis. Chest 2009; 136: 1371-1380 Im Internet DOI: 10.1378/chest.08-2569.
  • 37 Crapo RO, Forster RE. Carbon monoxide diffusing capacity. Clinics in Chest Medicine 1989; 10: 187-198
  • 38 Calaras D, Munteanu O, Scaletchi V. et al Ventilatory disturbances in patients with intrathoracic sarcoidosis – a study from a functional and histological perspective. Sarcoidosis Vasculitis and Diffuse Lung Diseases 2017; 34: 58-67
  • 39 Ranu H, Wilde M, Madden B. Pulmonary Function Tests. Ulster Medical Journal 2011; 80: 84-90
  • 40 Valeyre D, Prasse A, Nunes H. et al Sarcoidosis. The Lancet 2014; 383: 1155-1167 Im Internet https://linkinghub.elsevier.com/retrieve/pii/S0140673613606807
  • 41 Paramothayan NS, Lasserson TJ, Jones P.. Corticosteroids for pulmonary sarcoidosis (Cochrane Review). 2005: 2 Art. No.: CD001114. DOI: 10.1002/14651858.CD001114.pub2
  • 42 Baughman RP, Valeyre D, Korsten P. et al ERS clinical practice guidelines on treatment of sarcoidosis. The European respiratory journal. 2021: 58 Im Internet DOI: 10.1183/13993003.04079-2020
  • 43 The TOF, Adopted WAS, The BY, Of O, The BY. Statement on Sarcoidosis. Joint Statement of the ATS/ERS/WASOG. Am J Respir Crit Care Med 1999; 160: 736-755
  • 44 Baughman RP, Lower EE. Treatment of Sarcoidosis. Clinical Reviews in Allergy and Immunology 2015; 49: 79-92
  • 45 Broos CEB, Poell LHC, Looman CWN. et al No evidence found for an association between prednisone dose and FVC change in newly-treated pulmonary sarcoidosis. Respiratory Medicine 2018; 138: 31-37
  • 46 Baughman RP, Winget DB, Lower EE. Methotrexate is steroid sparing in acute sarcoidosis: results of a double blind, randomized trial. Sarcoidosis Vasc Diffuse Lung Dis 2000; 17: 60-66
  • 47 Vorselaars ADM, Wuyts WA, Vorselaars VMM. et al Methotrexate vs Azathioprine in Second-line Therapy of Sarcoidosis. Chest 2013; 144: 805-812
  • 48 Fang C, Zhang Q, Wang N. et al Effectiveness and tolerability of methotrexate in pulmonary sarcoidosis: A single center real-world study. Sarcoidosis Vasculitis and Diffuse Lung Diseases 2019; 36: 217-227
  • 49 Gavrysyuk V, Merenkova I, Dziublyk Y. et al Efficacy and tolerability of methotrexate and methylprednisolone in a comparative assessment of the primary and long-term outcomes in patients with pulmonary sarcoidosis. Diagnostics 2021; 11: 1-10
  • 50 Kahlmann V, Janssen Bonás M, Moor CC. et al Design of a randomized controlled trial to evaluate effectiveness of methotrexate versus prednisone as first-line treatment for pulmonary sarcoidosis: the PREDMETH study. BMC pulmonary medicine 2020; 20: 271
  • 51 Baughman RP, Drent M, Kavuru M. et al Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med 2006; 174 (07) 795-802
  • 52 Rossman MD, Newman LS, Baughman RP. et al A double-blinded, randomized, placebo-controlled trial of infliximab in subjects with active pulmonary sarcoidosis. Sarcoidosis Vasculitis and Diffuse Lung Diseases 2006; 23: 201-208
  • 53 Sweiss NJ, Noth I, Mirsaeidi M. et al Efficacy results of a 52-week trial of adalimumab in the treatment of refractory sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31: 46-54 Im Internet https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3624763/pdf/nihms412728.pdf
  • 54 Jamilloux Y, Cohen-Aubart F, Chapelon-Abric C. et al Efficacy and safety of tumor necrosis factor antagonists in refractory sarcoidosis: A multicenter study of 132 patients. Seminars in Arthritis and Rheumatism 2017; 47: 288-294 Im Internet DOI: 10.1016/j.semarthrit.2017.03.005.
  • 55 Sakkat A, Cox G, Khalidi N. et al Infliximab therapy in refractory sarcoidosis: a multicenter real-world analysis. Respiratory Research 2022; 23: 1-9 Im Internet DOI: 10.1186/s12931-022-01971-5.
  • 56 Sweiss NJ, Lower EE, Mirsaeidi M. et al Rituximab in the treatment of refractory pulmonary sarcoidosis. European Respiratory Journal 2014; 43: 1525-1528
  • 57 Friedman MA, Le B, Stevens J. et al Tofacitinib as a Steroid-Sparing Therapy in Pulmonary Sarcoidosis, an Open-Label Prospective Proof-of-Concept Study. Lung 2021; 199: 147-153 Im Internet DOI: 10.1007/s00408-021-00436-8.