Context:
Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction is a rare and life-threatening manifestation of the antiphospholipid syndrome (APLS). Data on the ...long-term outcome are scarce.
Objective:
The aims of the present study were to analyze the long-term outcome related to APLS per se and to characterize the course of adrenal involvement.
Design:
We conducted a retrospective study of patients with bilateral adrenal hemorrhage-adrenal infarction secondary to APLS seen in the Department of Internal Medicine of Pitié-Salpêtrière Hospital in Paris (France) between January 1990 and July 2010.
Results:
Three patients died during the acute phase related to APLS manifestations. Sixteen patients (7 males; 9 females) were followed up during a median period of 3.5 years (range 0.3–28.1 years). Three episodes of recurrent thrombosis were noted. One patient died from cerebral hemorrhage 3 months after the onset of adrenal insufficiency. Repeated Synacthen tests showed complete absence of response in 8 of the 10 patients assessed; cortisol and aldosterone increased appropriately in one patient and to some extent in another one. Dehydroepiandrosterone levels and 24-hour urinary epinephrine levels remained abnormally low in all evaluated patients. Adrenal imaging performed more than 1 year after the initial event revealed completely atrophic glands in 9 of 11 patients.
Conclusions:
This particular subset of APLS patients who survive the acute phase has a rather favorable long-term outcome. Although adrenal dysfunction is generally irreversible, adrenocortical function may, at least partially, recover in rare cases. In this view, measurement of early morning cortisol during follow-up is indicated to detect these patients.
Renal biopsy is the cornerstone of systemic lupus erythematosus (SLE) nephritis and antiphospholipid syndrome (APS) nephropathy management. However, transcutaneous renal biopsy (TCRB) is hampered by ...the antithrombotic treatment frequently prescribed for those diseases. Transjugular renal biopsy (TJRB) offers an attractive alternative for patients at increased risk of bleeding. The primary objective of the study was to describe the safety profile and diagnostic performance of TJRB in SLE and APS patients.
All SLE and/or APS patients who underwent a renal biopsy in our department (between January 2004 and October 2016) were retrospectively reviewed. Major complications were death, haemostasis nephrectomy, renal artery embolization, red blood cell transfusion, sepsis and vascular thrombosis; macroscopic haematuria, symptomatic perirenal/retroperitoneal bleeding and renal arteriovenous fistula without artery embolization were considered as minor complications.
Two hundred and fifty-six TJRBs-119 without antithrombotics (untreated), 69 under aspirin and 68 on anticoagulants and 54 TCRBs without antithrombotics-were analysed. Their major and minor complication rates, respectively, did not differ significantly for the four groups: 0 and 8% for untreated TJRBs, 1 and 6% for aspirin-treated, 6 and 10% for anticoagulant-treated and 2 and 2% for TCRBs. The number of glomeruli sampled and the biopsy contribution to establishing a histological diagnosis was similar for the four groups.
TJRBs obtained from SLE and APS patients taking antithrombotics had diagnostic yields and safety profiles similar to those of untreated TCRBs. Thus, TJRB should be considered for SLE and APS patients at risk of bleeding.
Churg-Strauss syndrome (CSS) is characterized by systemic vasculitis and blood and tissue eosinophilia. Blood eosinophilia correlates with disease activity, and activated T cells from CSS patients ...are predominantly T helper 2 (Th2). Interleukin (IL)-25 has been shown to link innate and adaptive immunity by enhancing Th2 cytokine production. We sought to determine the involvement of IL-25 and its receptor IL-17RB in the pathogenesis of CSS. We found increased levels of IL-25 in the serum of active CSS patients (952 ± 697 vs 75 ± 49 pg/mL in inactive patients and 47 ± 6 pg/mL in healthy donors). IL-25 was correlated with disease activity and eosinophil level. Eosinophils were the main source of IL-25, whereas activated CD4+ memory T cells were the IL-17RB–expressing cells in CSS. IL-25 enhanced the production of IL-4, IL-5, and IL-13 by activated peripheral blood mononuclear cells. IL-25 and IL-17RB were observed within the vasculitic lesions of patients with CSS, and IL-17RB colocalized with T cells. Increased expression of IL-17RB, tumor necrosis factor receptor–associated factor 6, and JunB in vasculitic lesions of CSS underscored the IL-25–mediated activation, whereas up-regulation of GATA3 and IL-10 supported Th2 differentiation. Our findings suggest that eosinophils, through the production of IL-25, exert a critical role in promoting Th2 responses in target tissues of CSS.
Aim: Severe uveitis is potentially associated with visual impairment or blindness in young patients. Therapeutic strategies remain controversial. The efficacy of interferon alpha-2a (IFN-α2a) in ...severe uveitis, refractory to steroids and conventional immunosuppressive agents, was evaluated. Patients and methods: Patients were included after a major relapse of uveitis following corticosteroids and immunosuppressants. IFN-α2a (3 million units three times a week) was administered subcutaneously. Efficacy was assessed by improvement in visual acuity, decrease in vitreous haze, resolution of retinal vasculitis and macular oedema, assessed by fundus examination and fluorescein angiography, and decrease in oral prednisone threshold. Results: 45 patients were included. Median age was 32.3 years (range 8–58) and sex ratio (F/M) was 0.66. Uveitis was associated with Behçet’s disease in 23 cases (51.1%) and with other entities in 22 cases (48.9%). Median duration of uveitis before interferon therapy was 34.9 months (range 3.4–168.7) and an average of 3.26 relapses following corticosteroids and immunosuppressants was noted. Uveitis was controlled in 82.6% of patients with Behçet’s disease and 59% of patients with other types of uveitis (p = 0.07). During a mean follow-up of 29.6 months (range 14–55), median oral prednisone threshold decreased significantly from 23.6 mg/day (range 16–45) to 10 mg/d (range 4–14) (p<0.001). Interferon was discontinued in 10 patients (22.2%) with Behçet’s disease and in four patients without Behçet’s disease. Relapses occurred in four and one cases, respectively. Conclusions: Interferon therapy appears to be an efficient strategy in severe and relapsing forms of Behçet’s disease but also in other uveitic entities. However, it seems to act more to suspend rather than cure the disease. Therefore, IFN-α2a may be proposed as a secondline strategy after failure of conventional immunosuppressants.
Objective
To report the long‐term outcome of neurologic involvement in patients with Behçet's disease (BD).
Methods
We performed a retrospective analysis of 115 patients who fulfilled the ...international criteria for BD (57% male; median age 37 years interquartile range (IQR) 30–46 years) and had neuro‐BD (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapse of NBD, inability to perform activities of daily living, and mortality were assessed.
Results
Seventy‐eight patients (68%) presented with acute NBD and 37 (32%) presented with a progressive course. The HLA–B51 allele was carried by 49% of the patients. Overall, 46 of 115 patients (40%) had severe disability at baseline, represented by a Rankin score of ≥3. The 5‐ and 7‐year event‐free survival rates were 65% and 53%, respectively. In multivariate analysis, a positive HLA–B51 status was independently associated with the risk of NBD relapse, with an odds ratio (OR) of 3.6 (95% confidence interval 95% CI 1.5–9.1). After a median followup of 73 months (IQR 59–102 months), 29 patients (25.2%) became dependent (were unable to perform activities of daily living) or died. Factors independently associated with poor outcome were paresis at onset (OR 6.47 95% CI 1.73–24.23) and location of inflammatory lesions at the brainstem on magnetic resonance imaging (OR 8.41 1.03–68.43). All 115 patients were treated with corticosteroids; 53 (46.1%) also took cyclophosphamide and 40 (34.8%) also took azathioprine. A trend toward longer event‐free survival was observed in patients with severe NBD (i.e., with a Rankin score of ≥3 at onset) receiving intravenous cyclophosphamide compared with those receiving azathioprine (P = 0.06).
Conclusion
Our findings indicate that NBD is a severe condition in which patients with the HLA–B51 allele appear to experience a worse prognosis.
Objective
The determinants of vessel targeting are largely unknown in vasculitides. This study was undertaken to identify patterns of vascular involvement in Takayasu arteritis (TA), using objective ...classification of vascular beds. We postulated that cluster analysis could unveil preferential associations between vascular beds commonly affected by TA.
Methods
Peripheral vascular Doppler, computed tomography angiography, and angio–magnetic resonance imaging data from 82 patients with TA (according to the American College of Rheumatology criteria) were studied between January 1995 and May 2006. Cross‐relationships of involvement between 24 main arteries were assessed using the phi correlation coefficient. Identification of patterns of vascular involvement was performed using agglomerative hierarchical cluster analysis.
Results
Data were obtained from 82 patients (68 women 82.9% and 14 men 17.1%). The median duration of followup was 5.1 years (range 1 month to 30 years). For 16 (80%) of 20 paired arteries, the highest correlation of involvement was observed with the contralateral artery. Conversely, disease extension was contiguous in the aorta. Cluster analysis further confirmed that all paired arterial beds, except for the internal and external carotid arteries, clustered with their contralateral counterpart and that the aortic arch, the descending thoracic aorta, and the abdominal aorta clustered together.
Conclusion
Our findings reveal that TA lesions mostly develop in a symmetric manner in paired vascular territories and that disease extension is contiguous in the aorta. This may prove useful for improving the radiologic followup of patients with TA and for providing a pattern for further investigations focusing on the mechanisms of vessel specificity in vasculitides.
Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary ...arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.
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.
To describe the pseudotumoural presentation of neuro-Behçet's disease (NBD).
We report here the main characteristics, treatment and outcome of 23 patients (5 personal cases and 18 patients from the ...literature) with a pseudotumoural presentation of NBD. Pseudotumoural NBD patients were compared with 69 consecutive patients, with a classical form of NBD.
The median age was 39 (range 27-48 years) years, with a male predominance (65.2%). Clinical features of the pseudotumoural NBD included hemi- or tetra-pyramidal symptoms (n = 20), headache (n = 17), cerebellar syndrome (n = 3), sphincter impotence (n = 3) and pseudobulbar signs (n = 2). CNS imaging showed pseudotumoural lesions mainly in the capsulo-thalamic area (69.6 vs 11.6% for classical NBD; P < 0.01). Histological analysis revealed necrotic lesions with perivascular inflammatory infiltrate without signs of tumoural or infectious lesions. Patients with pseudotumoural NBD had more severe initial disability status (Rankin's score ≥3 in 65.2 vs 24.7%; P < 0.01) and had a 3 years' longer duration between neurological signs and BD diagnosis (P = 0.01) compared with patients with classical NBD. Treatment consisted of CSs (n = 21, 95.5%) and immunosuppressive agents (n = 10, 35.7%) that led to complete clinical and imaging remission in 60.9% of patients. Two (8.7%) of the 23 patients with pseudotumoural NBD died of bedridden state complications.
The pseudotumoural form of NBD is a rare and life-threatening condition.
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