Vaskulitiden mit pulmonaler Beteiligung

Zusammenfassung

Die vielfältigen pulmonalen Manifestationen von Vaskulitiden sind eine große diagnostische und therapeutische Herausforderung. Eine Vaskulitis sollte bei schnellen, schweren und ungewöhnlichen Verläufen von Lungenfunktionseinschränkungen in Betracht gezogen werden. Die klinische Untersuchung häufig ebenfalls betroffener Organsysteme, insbesondere Haut und Niere auf der einen und die spezifischen Antikörperuntersuchungen auf der anderen Seite, sind komplementäre Ansätze zur Diagnosesicherung.

Die Lunge ist häufig bei Kleingefäßvaskulitiden wie der anti-GBM-Vaskulitis/Goodpasture-Syndrom und den ANCA-assoziierten Vaskulitiden beteiligt. Lebensbedrohliche Blutungen und schnelle, irreversible Schäden anderer Organe, häufig der Niere, sind gefürchtete Komplikationen, sodass eine schnelle Diagnose nötig ist.

Angesichts der Seltenheit der Erkrankungen, vieler neuer Medikamente einschließlich Biologicals und häufig multipler betroffener Organe sind der Kontakt zu einem spezialisierten Zentrum und Kooperation der Fachabteilungen zentral, um das optimale Behandlungsergebnis für die betroffenen Patienten zu erreichen. Alle betreuenden Ärzte sollten die Nebenwirkungsspektren der immunsuppressiven Induktions- und Erhaltungstherapien kennen.

Abstract

The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation.

Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides. Life threatening pulmonary hemorrhage and irreversible damage of other organs, frequently the kidney, are important complications necessitating rapid diagnosis of these conditions.

Vasculitides are rare diseases of multiple organs and therapies including biologics are evolving rapidly, requiring cooperation of specialities and with specialized centres to achieve best patient care. All involved physicians should be aware of typical complications of immunosuppressive therapy.

• Literatur

• 1 Gutierrez-Gonzalez LA. Biological Therapy-Induced Systemic Vasculitis. Current rheumatology reports 2016; 18: 39
• 2 Jennette JC, Falk RJ, Bacon PA et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis and rheumatism 2013; 65: 1-11
• 3 Teng GG, Chatham WW. Vasculitis related to viral and other microbial agents. Best practice & research. Clinical rheumatology 2015; 29: 226-243
• 4 Ntatsaki E, Mooney J, Scott DG et al. Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy. Rheumatology 2014; 53: 145-152
• 5 Sharma A, Dhooria A, Aggarwal A et al. Connective Tissue Disorder-Associated Vasculitis. Current rheumatology reports 2016; 18: 31
• 6 Ramos-Casals M, Brito-Zeron P, Seror R et al. Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements. Rheumatology 2015; 54: 2230-2238
• 7 Greco A, Rizzo MI, De Virgilio A et al. Goodpastureʼs syndrome: a clinical update. Autoimmunity reviews 2015; 14: 246-253
• 8 Jennette JC, Falk RJ. Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nature reviews. Rheumatology 2014; 10: 463-473
• 9 Comarmond C, Crestani B, Tazi A et al. Pulmonary fibrosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a series of 49 patients and review of the literature. Medicine 2014; 93: 340-349
• 10 Kallenberg CG. Key advances in the clinical approach to ANCA-associated vasculitis. Nature reviews. Rheumatology 2014; 10: 484-493
• 11 McKinney EF, Willcocks LC, Broecker V et al. The immunopathology of ANCA-associated vasculitis. Seminars in immunopathology 2014; 36: 461-478
• 12 Unizony S, Villarreal M, Miloslavsky EM et al. Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Annals of the rheumatic diseases 2016; 75: 1166-1169
• 13 Homma S, Suzuki A, Sato K. Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist. Clinical and experimental nephrology 2013; 17: 667-671
• 14 Mueller A, Holl-Ulrich K, Gross WL. Granuloma in ANCA-associated vasculitides: another reason to distinguish between syndromes?. Current rheumatology reports 2013; 15: 376
• 15 West S, Arulkumaran N, Ind PW et al. Diffuse alveolar haemorrhage in ANCA-associated vasculitis. Internal medicine 2013; 52: 5-13
• 16 Haubitz M. Vaskulitiden. Der Nephrologe 2016; 11: 124-133
• 17 Walsh M, Merkel PA, Peh CA et al. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials 2013; 14: 73
• 18 Laudien M, Gadola SD, Podschun R et al. Nasal carriage of Staphylococcus aureus and endonasal activity in Wegener s granulomatosis as compared to rheumatoid arthritis and chronic Rhinosinusitis with nasal polyps. Clinical and experimental rheumatology 2010; 28 (Suppl. 57) 51-55
• 19 Tadema H, Heeringa P, Kallenberg CG. Bacterial infections in Wegenerʼs granulomatosis: mechanisms potentially involved in autoimmune pathogenesis. Current opinion in rheumatology 2011; 23: 366-371
• 20 Pendergraft 3rd WF, Cortazar FB, Wenger J et al. Long-term maintenance therapy using rituximab-induced continuous B-cell depletion in patients with ANCA vasculitis. Clinical journal of the American Society of Nephrology: CJASN 2014; 9: 736-744
• 21 Rajagopala S, Shobha V, Devaraj U et al. Pulmonary hemorrhage in Henoch-Schonlein purpura: case report and systematic review of the english literature. Seminars in arthritis and rheumatism 2013; 42: 391-400
• 22 Cacoub P, Comarmond C, Domon Ft et al. Cryoglobulinemia Vasculitis. The American journal of medicine 2015; 128: 950-955
• 23 Jachiet M, Flageul B, Deroux A et al. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis & rheumatology 2015; 67: 527-534
• 24 Schreiber J, Knolle J, Kachel R et al. [Differential diagnosis of diffuse pulmonary haemorrhage]. Pneumologie 2006; 60: 347-354
• 25 Fishbein GA, Fishbein MC. Lung vasculitis and alveolar hemorrhage: pathology. Seminars in respiratory and critical care medicine 2011; 32: 254-263
• 26 Chan E, Sangle SR, Coghlan JG et al. Pulmonary artery aneurysms in Behcetʼs disease treated with anti-TNFalpha: A case series and review of the literature. Autoimmunity reviews 2016; 15: 375-378
• 27 Bahmer T, Romagnoli M, Girelli F et al. The use of auto-antibody testing in the evaluation of interstitial lung disease (ILD) – A practical approach for the pulmonologist. Respiratory medicine 2016; 113: 80-92
• 28 Luqmani RA. State of the art in the treatment of systemic vasculitides. Frontiers in immunology 2014; 5: 471
• 29 Flossmann O, Berden A, de Groot K et al. Long-term patient survival in ANCA-associated vasculitis. Annals of the rheumatic diseases 2011; 70: 488-494
• 30 Holdsworth SR, Gan PY, Kitching AR. Biologics for the treatment of autoimmune renal diseases. Nature reviews. Nephrology 2016; 12: 217-231
• 31 Kronbichler A, Jayne DR, Mayer G. Frequency, risk factors and prophylaxis of infection in ANCA-associated vasculitis. European journal of clinical investigation 2015; 45: 346-368
• 32 Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrology, dialysis, transplantation: official publication of the European Dialysis and Transplant Association – European Renal Association. 2016 [ePub ahead of print]