Zusammenfassung
Rheumatische Erkrankungen treten oft in Begleitung von sekundären Lungenerkrankungen auf, die in der Regel einen großen Einfluss auf die Lebensqualität und Mortalität der Patienten haben. Die richtige Zuordnung solcher Lungenerkrankungen ist für Therapie und Prognose von enormer Bedeutung. Die diagnostische und therapeutische Herausforderung besteht darin, in der Vielzahl möglicher Ursachen von respiratorischen Beschwerden eine präzise Diagnose und angemessene Therapie zu finden. Dabei sollte ein vernünftiges Maß an Invasivität bei der diagnostischen Zuordnung gewahrt werden, auch wenn gleichzeitig die Abgrenzung zu Infektionen oder toxischen Lungenerkrankungen von enormer Wichtigkeit ist. Kompliziert wird diese Entscheidung weiterhin durch die oft fehlende Evidenz für Therapien. Da insbesondere die Lungenfibrose in ihrer Komplexität und Mortalität mit Tumorerkrankungen vergleichbar ist, sollten Diagnostik und Therapie in multidisziplinären Fallkonferenzen abgestimmt werden.
Schlüsselwörter
Interstitielle Lungenerkrankungen Bildgebung Histologie Immunsuppression TherapiePulmonary fibrosis in rheumatic diseases
Abstract
Rheumatic diseases are frequently complicated by secondary pulmonary diseases, which often impair the quality of life and increase the mortality of patients. A correct classification of such pulmonary complications is important to ensure appropriate treatment and optimal prognosis. The diagnostic and therapeutic challenge is to find the precise diagnosis and appropriate therapy among the multitude of potential causes for respiratory symptoms. It is important to maintain a cautious approach to invasive diagnostics, even though the differential diagnostics of infections or toxic lung disease might be crucial. The situation is further complicated by the frequent lack of evidence for therapies. Especially in the case of pulmonary fibrosis which is comparable to cancer in its complexity and high mortality, the diagnostics and therapy should be discussed in appropriate interdisciplinary boards.
Keywords
Interstitial lung disease Therapy Imaging Histology ImmunosuppressionNotes
Einhaltung ethischer Richtlinien
Interessenkonflikt
D. Grund und E. Siegert geben an, dass kein Interessenkonflikt besteht.
Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.
Literatur
- 1.American Thoracic Society, European Respiratory Society (2002) American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 165(2):277–304. doi: 10.1164/ajrccm.165.2.ats01 CrossRefGoogle Scholar
- 2.Vivero M, Padera RF (2015) Histopathology of lung disease in the connective tissue diseases. Rheum Dis Clin North Am 41(2):197–211CrossRefPubMedGoogle Scholar
- 3.Raghu G, Anstrom KJ, King TE Jr., Lasky JA, Martinez FJ (2012) Prednisone, azathioprine, and N‑acetylcysteine for pulmonary fibrosis. N Engl J Med 366(21):1968–1977CrossRefPubMedGoogle Scholar
- 4.Nogueira CR, Nápolis LM, Bagatin E, Terra-Filho M, Müller NL, Silva CIS, Rodrigues RT, Neder JA, Nery LE (2011) Lung diffusing capacity relates better to short-term progression on HRCT abnormalities than spirometry in mild asbestosis. Am J Ind Med 54(3):185–193 (Epub 2010 Dec 22) doi: 10.1002/ajim.20922 CrossRefPubMedGoogle Scholar
- 5.Suliman YA, Dobrota R, Huscher D, Nguyen-Kim TD, Maurer B, Jordan S, Speich R, Frauenfelder T, Distler O (2015) Brief report: Pulmonary function tests: High rate of false-negative results in the early detection and screening of Scleroderma-related interstitial lung disease. Arthritis Rheumatol (hoboken Nj) 67(12):3256–3261CrossRefGoogle Scholar
- 6.Gargani L, Doveri M, D’Errico L, Frassi F, Bazzichi ML, Delle Sedie A, Scali MC, Monti S, Mondillo S, Bombardieri S et al (2009) Ultrasound lung comets in systemic sclerosis: A chest sonography hallmark of pulmonary interstitial fibrosis. Rheumatology (Oxford) 48(11):1382–1387CrossRefGoogle Scholar
- 7.Vananuvat P, Suwannalai P, Sungkanuparph S, Limsuwan T, Ngamjanyaporn P, Janwityanujit S (2011) Primary prophylaxis for pneumocystis jirovecii pneumonia in patients with connective tissue diseases. Semin Arthritis Rheum 41(3):497–502CrossRefPubMedGoogle Scholar
- 8.Wells AU, Denton CP (2014) Interstitial lung disease in connective tissue disease – mechanisms and management. Nat Rev Rheumatol 10(12):728–739CrossRefPubMedGoogle Scholar
- 9.Roth MD, Tseng CH, Clements PJ, Furst DE, Tashkin DP, Goldin JG, Khanna D, Kleerup EC, Li N, Elashoff D et al (2011) Predicting treatment outcomes and responder subsets in scleroderma-related interstitial lung disease. Arthritis Rheum 63(9):2797–2808CrossRefPubMedPubMedCentralGoogle Scholar
- 10.Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V, Pantelidis P, Haslam PL, Vassilakis DA, Black CM, du Bois RM (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165(12):1581–1586CrossRefPubMedGoogle Scholar
- 11.Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, Corte TJ, Sander CR, Ratoff J, Devaraj A et al (2008) Interstitial lung disease in systemic sclerosis: A simple staging system. Am J Respir Crit Care Med 177(11):1248–1254CrossRefPubMedGoogle Scholar
- 12.Ley B, Collard HR, King TE Jr (2011) Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 183(4):431–440CrossRefPubMedGoogle Scholar
- 13.Moore OA, Goh N, Corte T, Rouse H, Hennessy O, Thakkar V, Byron J, Sahhar J, Roddy J, Gabbay E et al (2013) Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford) 52(1):155–160CrossRefGoogle Scholar
- 14.Hansell DM, Goldin JG, King TE Jr., Lynch DA, Richeldi L, Wells AU (2015) CT staging and monitoring of fibrotic interstitial lung diseases in clinical practice and treatment trials: A position paper from the Fleischner Society. Lancet Respir Med 3(6):483–496CrossRefPubMedGoogle Scholar
- 15.Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Arriola E, Silver R, Strange C, Bolster M et al (2006) Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med 354(25):2655–2666CrossRefPubMedGoogle Scholar
- 16.Clements PJ, Tashkin D, Roth M, Khanna D, Furst DE, Tseng C, Volkmann ER, Elashoff R (2015) The Scleroderma Lung Study II (SLS II) Shows That Both Oral Cyclophosphamide (CYC) and Mycophenolate Mofitil (MMF) Are Efficacious in Treating Progressive Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc). In: 2015 ACR/ARHP Annual Meeting San FranciscoGoogle Scholar
- 17.Jordan S, Distler JH, Maurer B, Huscher D, van Laar JM, Allanore Y, Distler O (2015) Effects and safety of rituximab in systemic sclerosis: An analysis from the European Scleroderma Trial and Research (EUSTAR) group. Ann Rheum Dis 74(6):1188–1194CrossRefPubMedGoogle Scholar
- 18.Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, Baron M, Chung L, Fierlbeck G, Lakshminarayanan S et al (2016) Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): A phase 2, randomised, controlled trial. Lancet 387(10038):2630–2640 (Epub 2016 May 5) doi: 10.1016/S0140-6736(16)00232-4 CrossRefPubMedGoogle Scholar
- 19.Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, Craig R, Hirano I, Marshall K, Ruderman E et al (2011) Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): An open-label, randomised phase 2 trial. Lancet 378(9790):498–506CrossRefPubMedGoogle Scholar
- 20.Binks M, Passweg JR, Furst D, McSweeney P, Sullivan K, Besenthal C, Finke J, Peter HH, van Laar J, Breedveld FC et al (2001) Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: Procedure related mortality and impact on skin disease. Ann Rheum Dis 60(6):577–584CrossRefPubMedPubMedCentralGoogle Scholar
- 21.Nair A, Walsh SL, Desai SR (2015) Imaging of pulmonary involvement in rheumatic disease. Rheum Dis Clin North Am 41(2):167–196. doi: 10.1016/j.rdc.2014.12.001
- 22.Song JW, Do KH, Kim MY, Jang SJ, Colby TV, Kim DS (2009) Pathologic and radiologic differences between idiopathic and collagen vascular disease-related usual interstitial pneumonia. Chest 136(1):23–30CrossRefPubMedGoogle Scholar
- 23.Kim EJ, Elicker BM, Maldonado F, Webb WR, Ryu JH, Van Uden JH, Lee JS, King TE Jr, Collard HR (2010) Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J 35(6):1322–1328CrossRefPubMedGoogle Scholar
- 24.Yunt ZX, Solomon JJ (2015) Lung disease in rheumatoid arthritis. Rheum Dis Clin North Am 41(2):225–236CrossRefPubMedPubMedCentralGoogle Scholar
- 25.Capobianco J, Grimberg A, Thompson BM, Antunes VB, Jasinowodolinski D, Meirelles GS (2012) Thoracic manifestations of collagen vascular diseases. Radiographics 32(1):33–50. doi: 10.1148/rg.321105058
- 26.Tsuchiya Y, Takayanagi N, Sugiura H, Miyahara Y, Tokunaga D, Kawabata Y, Sugita Y (2011) Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 37(6):1411–1417. doi: 10.1183/09031936.00019210
- 27.Roubille C, Haraoui B (2014) Interstitial lung diseases induced or exacerbated by DMARDS and biologic agents in rheumatoid arthritis: A systematic literature review. Semin Arthritis Rheum 43(5):613–626CrossRefPubMedGoogle Scholar
- 28.Kremer JM, Alarcon GS, Weinblatt ME, Kaymakcian MV, Macaluso M, Cannon GW, Palmer WR, Sundy JS, St Clair EW, Alexander RW et al (1997) Clinical, laboratory, radiographic, and histopathologic features of methotrexate-associated lung injury in patients with rheumatoid arthritis: A multicenter study with literature review. Arthritis Rheum 40(10):1829–1837CrossRefPubMedGoogle Scholar
- 29.Valeyre D, Prasse A, Nunes H, Uzunhan Y, Brillet PY, Muller-Quernheim J (2014) Sarcoidosis. Lancet 383(9923):1155–1167CrossRefPubMedGoogle Scholar
- 30.Nardi A, Brillet PY, Letoumelin P, Girard F, Brauner M, Uzunhan Y, Naccache JM, Valeyre D, Nunes H (2011) Stage IV sarcoidosis: Comparison of survival with the general population and causes of death. Eur Respir J 38(6):1368–1373CrossRefPubMedGoogle Scholar