Objective
To analyze the factors associated with response to anti–tumor necrosis factor (anti‐TNF) treatment and compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in patients ...with refractory noninfectious uveitis.
Methods
This was a multicenter observational study of 160 patients (39% men and 61% women; median age 31 years interquartile range 21–42) with uveitis that had been refractory to other therapies, who were treated with anti‐TNF (IFX 5 mg/kg at weeks 0, 2, 6, and then every 5–6 weeks n = 98 or ADA 40 mg every 2 weeks n = 62). Factors associated with complete response were assessed by multivariate analysis. Efficacy and safety of IFX versus ADA were compared using a propensity score approach with baseline characteristics taken into account. Subdistribution hazard ratios (SHRs) and 95% confidence intervals (95% CIs) were calculated.
Results
The main etiologies of uveitis included Behçet's disease (BD) (36%), juvenile idiopathic arthritis (22%), spondyloarthropathy (10%), and sarcoidosis (6%). The overall response rate at 6 and 12 months was 87% (26% with complete response) and 93% (28% with complete response), respectively. The median time to complete response was 2 months. In multivariate analysis, BD and occurrence of >5 uveitis flares before anti‐TNF initiation were associated with complete response to anti‐TNF (SHR 2.52 95% CI 1.35–4.71, P = 0.004 and SHR 1.97 95% CI 1.02–3.84, P = 0.045, respectively). Side effects were reported in 28% of patients, including serious adverse events in 13%. IFX and ADA did not differ significantly in terms of occurrence of complete response (SHR 0.65 95% CI 0.25–1.71, P = 0.39), serious side effects (SHR 0.22 95% CI 0.04–1.25, P = 0.089), or event‐free survival (SHR 0.55 95% CI 0.28–1.08, P = 0.083).
Conclusion
Anti‐TNF treatment is highly effective in refractory inflammatory uveitis. BD is associated with increased odds of response. IFX and ADA appear to be equivalent in terms of efficacy.
Objective
The nature and impact of food and other external triggers in recurrences of Behçet's disease (BD)‐related oral ulcers (OUs) remain unknown. This survey investigated dietary and nondietary ...triggers of BD‐related OU recurrences.
Methods
Patients with BD who were followed in 7 French hospital departments completed a self‐administered patient questionnaire. General and specific dietary triggering factors were sought in open questions. The questionnaire also included closed questions, notably to evaluate the effect of 6 general triggering situations and 24 selected foods. The results were expressed as number (percentage) of positive responses.
Results
Among the 101 questionnaires distributed, 81 were usable. Among the 81 patients, 96% fulfilled the International Criteria for Behçet's Disease classification criteria, and 53% qualified their OU recurrences during the previous 12 months as very discomforting or discomforting. For the 6 general situations suggested, 50 patients (62%) declared ≥1 as a “sure” trigger of OU recurrences. In both open and closed questions, the most frequent triggers were fatigue/stress (37–47% of patients) and food (32–35%). Among the 24 suggested foods, nuts (48%), pineapple (42%), peanuts (32%), Emmental cheese (30%), almonds (23%), lemons (22%), and other cheeses (21%) were the most frequently reported. The corresponding open question gave consistent findings but with lower frequencies.
Conclusion
Most patients can identify triggers of recurring BD‐related OUs, with fatigue/stress and food representing the most frequent triggers. The management of OU must consider such external factors. The histamine‐rich or ‐liberating properties of the commonly cited OU‐triggering foods suggest a hyperreactivity mechanism.
Renal involvement is a rare event during primary SS (pSS). We aimed to describe the clinico-biological and histopathological characteristics of pSS-related nephropathy and its response to treatment.
...We conducted a French nationwide, retrospective, multicentre study including pSS patients fulfilling American-European Consensus Group criteria or enlarged American-European Consensus Group criteria, and with biopsy-proven renal involvement.
A total of 95 patients were included (median age 49 years). An estimated glomerular filtration rate (eGFR) of <60 ml/min was found in 82/95 patients (86.3%). Renal biopsy demonstrated tubulointerstitial nephritis (TIN) in 93 patients (97.9%), and frequent (75%) plasma cell infiltrates. Glomerular lesions were found in 22 patients (23.2%), mainly related to cryoglobulin. The presence of anti-SSA (76.8%) and anti-SSB (53.8%) antibodies was particularly frequent among patients with TIN and was associated with a worse renal prognosis. Eighty-one patients (85.3%) were treated, with CSs in 80 (98.8%) and immunosuppressive agents (mostly rituximab) in 21 cases (25.9%). Despite marked interstitial fibrosis at initial biopsy, kidney function improved significantly during the 12-month period following diagnosis (final eGFR 49.9 vs 39.8 ml/min/1.73 m 2 at baseline, P < 0.001). No proven benefit of immunosuppressive agents over steroid therapy alone was found in this study.
Renal involvement of pSS is mostly due to TIN with marked T, B and especially plasma cell infiltration. Renal dysfunction is usually isolated but can be severe. Use of CSs can improve the eGFR, but further studies are needed to define the best therapeutic strategy in this disease.
Abstract Objective Kawasaki disease (KD) is a vasculitis that mostly occurs in young children and rarely in adults. We analyzed the characteristics of adult-onset KD (AKD) in France. Methods We ...collected retrospective and prospective data for patients with a diagnosis of KD occurring after the age of 18 years. Cases were obtained via various French medical networks and identified from the international literature. Results We included 43 patients of AKD at 26 institution from 1992 to 2015, with mean (SD) age 30 (11) years (range 18–68) and sex ratio (M/F) 1.2; 34 patients met the American Heart Association criteria and 9 were incomplete AKD. The median time to diagnosis was 13 days (interquartile range 8–21). The main symptoms were fever (100%), exanthema (98%), changes in the extremities (91%), conjunctivitis (77%), oral cavity changes (89%), cervical adenitis (55%) and cardiac abnormalities (45%). Overall, 35% of patients showed large-vessel vasculitis: coronary vasculitis (26%) and coronary aneurysm (19%). Treatment was mostly intravenous immunoglobulins (79%) and aspirin (81%). Four patients showed myocardial infarction due to coronary vasculitis, but none were treated with IVIg because of late diagnosis. After a median follow-up of 5 months (range 1–117), persistent aneurysm was noted in 9% of cases. Damage was significantly lower with early treatment than late or no treatment (p = 0.01). Conclusion Given the high frequency of cardiac involvement and complications in this series of AKD, diagnosis and treatment should not be delayed, and early IVIg treatment seems to improve the outcome.
Biotherapies targeting TNFα were proven to be effective in the most severe cases of Crohn's Disease, a chronic granulomatous inflammatory bowel disease that can involve any portion of the digestive ...tract. The tolerance of anti-TNFα therapy is usually good, although several infectious complications have been reported with these drugs.
We report a case of a Crohn's disease patient who developed pulmonary cryptococcosis following chicken manure exposition while he received adalimumab and azathioprine.
A 54-year-old man, with history of severe Crohn's disease and ankylosing spondylitis, was admitted for diarrhea and abdominal pain under azathioprine treatment. In December 2010, he was treated with oral prednisone (1mg/kg/day), but Crohn's disease relapsed when prednisone dose was lower than 30mg/a day. The patient was then treated with adalimumab, but six weeks later he developed severe pulmonary cryptococcosis. The patient experienced a good outcome under antifungal therapy. We retrospectively found a high exposure to chicken manure in the last weeks.
Cryptococcosis is an opportunistic infection that can occur under anti-TNFα therapy. The environmental exposure to Cryptococcus spp. (in particular in chicken manure) is a source of contamination. Avoiding exposition to bird manure should be a recommendation for patients who are living in rural areas.
Abstract Objectives This study aimed to estimate the prevalence of ANCA-associated vasculitis (AAV). That is, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic ...granulomatosis with polyangiitis (EGPA), in Southern France in 2018, and evaluate differences among Europeans and non-Europeans. Methods This population-based, cross-sectional study used four sources (hospitals, community-based physicians, laboratories, National Health Insurance) to identify adults ≥15 years diagnosed with GPA, MPA or EGPA, living in Hérault and Gard in 2018. Cases were defined using the ACR/EULAR classification criteria, and if necessary, the European Medicines Agency algorithm. Prevalence estimates were standardised to the world population and capture-recapture analysis was used to assess the comprehensiveness of the estimation. The influence of geographical origin was evaluated. Results A total of 202 patients were selected, with 86 cases of GPA (42.6%), 85 cases of MPA (42.1%) and 31 cases of EGPA (15.3%). The standardised prevalence estimates per million inhabitants for 2018 were: 103 (95%CI 84–125) for AAV, 48 (95%CI 35–64) for GPA, 39 (95%CI 28–53) for MPA and 16 (95%CI 9–26) for EGPA, 36 (95%CI 25–50) for anti-PR3 positive AAV, 46 (95%CI 34–61) for anti-MPO positive AAV, and 16 (95%CI 9–26) for ANCA-negative AAV. The global estimation of comprehensiveness by capture-recapture analysis was 80.5%. The number of AAV cases was higher for non-European residents (P = 0.001), particularly for MPA (P < 0.0001). Conclusion We provide a new estimate of AAV prevalence in France and show a higher prevalence of MPA in non-European patients.
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