Opportunistic Infections in Medium and Small-Sized Vessel Vasculitis: Based on a Retrospectivestudy on 108 Patients

Raphaele Mestiri¹, Lafarge Antoine², Jean-Jacques Tudesq³, Marc Pineton de Chambrun⁴, Melanie Roriz⁵, Stéphane Bally⁶, Laurent Alric⁷, Damien Sène⁸, Thomas Quémeneur⁹, Cécile-Audrey Durel¹⁰, Brigitte Gilson¹¹, Alice Berezne¹², Anne-Marie Ruppert¹³, Raphaele Seror¹⁴, Philippe Guilpain¹⁵, Xavier Puéchal¹⁶, Luc Mouthon¹⁷, Loïc Guillevin¹⁶ and Benjamin Terrier¹⁸, ¹Internal Medicine, Cochin hospital, Paris, France, ²Medecine Interne, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France, ³Internal Medicine, CHU Montpellier, Montpellier, France, ⁴Université Pierre et Marie Curie, Paris, France, ⁵Université Paris-Diderot, Paris, France, ⁶Nephrology, CH Chambery, Chambery, France, ⁷Internal Medicine, CHU Toulouse, Toulouse, France, ⁸Lariboisière Hospital, Paris Diderot University, Paris, France, ⁹Department of Internal Medicine, CH of Valenciennes, France, Valenciennes, France, ¹⁰Internal Medicine, Hôpital Edouard Herriot, lyon, France, ¹¹Internal Medicine, CH Verdun, Verdun, France, ¹²Internal Medicine, CH Annecy, Annecy, France, ¹³Pulmonology, Tenon hospital, Paris, France, ¹⁴Hopitaux Universitaires Paris Sud, Kremlin Bicetre, France, Rheumatology, Université Paris Sud, Le Kremlin Bicetre, France, ¹⁵Montpellier, Montpellier, France, ¹⁶Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, Hôpital Cochin, Université Paris Descartes, Sorbonne Paris Cité, Paris, France, ¹⁷Department of Internal Medicine, INSERM Unité 1016, Centre de Référence pour les Maladies Auto-immunes Rares, National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France, ¹⁸Centre de Référence des Maladies Auto-immunes et Systémiques Rares, Vascularites Nécrosantes et Sclérodermie Systémique, Service de Médecine Interne, Hôpital Cochin, APHP, INSERM U1016, Université Paris Descartes, Paris, France

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: ANCA, opportunistic infections and vasculitis, Wegener's granulomatosis

Session Information

Date: Tuesday, October 23, 2018

Title: Vasculitis – ANCA-Associated Poster II

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Overall survival has been markedly improved during the last decades in systemic vasculitis. In contrast, the use of immunosuppressive agents led to an increased risk of infectious complications, ranging from common to opportunistic infections (OI). While the former have been well described, data on the latter are scarce. This study aimed to describe characteristics of patients having OI during systemic vasculitis.

Methods:

We conducted a retrospective, observational, multicenter study, including patients with medium and small-sized vessel vasculitis according to Chapel Hill definitions and presenting with OI. Clinico-biological findings and therapies were recorded at diagnosis and follow-up of vasculitis and diagnosis of OI.

Results:

A total of 108 patients (men 51%, mean age at infection 59.3±15.4 years), experiencing 132OI between 1985 and 2018, were included. Twenty-five (19%) patients had multiple OI. Vasculitis diagnoses were: ANCA-associated vasculitis (AAV) in 86 (80%) patients, i.e. granulomatosis with polyangiitis in 51, microscopic polyangiitis in 18 and eosinophilic granulomatosis with polyangiitis in 17, polyarteritis nodosa in 10 (9%), and immune complex vasculitis in 12 (11%).

Comorbidities included chronic kidney failure with eGFR <60 ml/min/1.73m²in 33 (31%), chronic respiratory disease in 22 (20%) and diabetes in 14 (13%). Before OI, 30 (28%) patients experienced ³1 common infections. Treatments received before OI were glucocorticoids in 106 (99%) patients, cyclophosphamide in 75 (73%) with a median cumulative dose of 7.2 g (IQR 3.6-18), rituximab in 31 (32%), and both drugs in 14 (13%). Median interval from diagnosis of vasculitis to OI was 11 (3-62) months.

Opportunistic infections were: invasive fungal infections in 75 (including pneumocystis in 40, invasive aspergillosis in 19, severe candidiasis in 12, cryptococcosis in 4, mucormycosis in 1), viral infections in 30 (including cytomegalovirus in 24), bacterial infections in 24 (including tuberculosis in 12, atypical mycobacteria in 5, nocardiosis in 7), and parasitic infections in 3. OI were disseminated in 24 (18%%) cases, and organ-limited in 108 (82%), the latter involving the lungs in 87 (81%).

At diagnosis of OI, patients were under glucocorticoids in 75%, at a median dose of 20 mg/day (5-50). Lymphopenia <1000/mm³was noted in 34%, with a median CD4 T cell count of 226/mm³(143-578), respectively, and hypogammaglobinemia <6 g/L in 31%. Only 5 patients with pneumocystis infection had prophylaxis.

OI required hospitalization in intensive care unit in 24 (18%) and led to death in 19 (14%). Among all death (n=37), OI were the leading cause of death (51%).

Twelve OI (9%) were diagnosed concomitantly to a vasculitis flare, and OI led to a modification of immunosuppressive therapy in 42% of cases, mainly through a decrease of immunosuppression.

Conclusion:

Opportunistic infections mainly occur during AAV, especially granulomatosis with polyangiitis receiving high doses of glucocorticoids. Pneumocystis was the most frequent infection, followed by cytomegalovirus infection and invasive aspergillus. Prognosis was severe, with high number of patients requiring ICU and/or dying.

Disclosure: R. Mestiri, None; L. Antoine, None; J. J. Tudesq, None; M. Pineton de Chambrun, None; M. Roriz, None; S. Bally, None; L. Alric, None; D. Sène, None; T. Quémeneur, None; C. A. Durel, None; B. Gilson, None; A. Berezne, None; A. M. Ruppert, None; R. Seror, None; P. Guilpain, None; X. Puéchal, None; L. Mouthon, None; L. Guillevin, None; B. Terrier, None.

To cite this abstract in AMA style:

Mestiri R, Antoine L, Tudesq JJ, Pineton de Chambrun M, Roriz M, Bally S, Alric L, Sène D, Quémeneur T, Durel CA, Gilson B, Berezne A, Ruppert AM, Seror R, Guilpain P, Puéchal X, Mouthon L, Guillevin L, Terrier B. Opportunistic Infections in Medium and Small-Sized Vessel Vasculitis: Based on a Retrospectivestudy on 108 Patients [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/opportunistic-infections-in-medium-and-small-sized-vessel-vasculitis-based-on-a-retrospectivestudy-on-108-patients/. Accessed .

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