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Background Adult Henoch-Schonlein purpura (HSP) has been associated with poor outcome and end-stage renal disease in >20% of cases. Although the benefit of adding another immunosuppressant to steroids in severe adult HSP has not been shown, the benefit of plasma exchange (PE) therapy has been poorly evaluated. Study Design Case series. Setting & Participants 11 consecutive patients with severe and newly diagnosed HSP since 1988 who were treated with steroids and PE. Outcome & Measurement Patients' characteristics and outcome were analyzed. Birmingham Vasculitis Activity Score (BVAS), estimated glomerular filtration rate (eGFR), and proteinuria were measured at baseline, at the end of PE treatment, at months 6 and 12, and at the last visit. Side effects of corticoid treatment and PE were recorded. Results 11 patients were identified in 1988-2010. Patients received intravenous corticoid pulses in 64% of cases, followed by oral prednisone for a median of 6.6 months. They received a median of 12 PE sessions. BVAS, eGFR, and proteinuria improved significantly between baseline and the last PE at a median of 2 months. PE sessions were well tolerated, except in one patient who developed central catheter–associated septicemia. One patient required dialysis therapy 15 days after HSP diagnosis and did not recover kidney function. At the last medical evaluation at a mean follow-up of 6 years, median eGFR and proteinuria were 83 ± 22 mL/min/1.73 m 2 and protein excretion of 140 ± 10 mg/d, respectively. 3 women had pregnancy without complications. Limitations This case series did not have a control group. Conclusions The combination of PE and corticoid therapy in severe forms of HSP was associated with fast improvement and good long-term outcome.

Introduction Les echanges plasmatiques (EP) peuvent etre proposes comme traitement de certaines Neuropathies Peripheriques Dysimmunitaires Chroniques (NPDC). Toutefois, plusieurs points concernant leur utilisation dans ces indications restent encore a preciser. Objectifs Nous avons mene une etude retrospective de 10 ans au CHU d’Angers afin d’evaluer l’efficacite et la tolerance des echanges plasmatiques dans le traitement des NPDC. Patients et methodes Les dossiers des patients traites par EP entre 2006 et 2016 au CHU d’Angers ont ete etudies, tous les patients traites pour une NPDC ont ete inclus. Les donnees cliniques et les procedures d’echange ont ete analysees. La reponse aux EP a ete evaluee en definissant 2 categories de patients « repondeurs » et « non repondeurs » en fonction de l’evolution clinique et de la decision de poursuivre ou non le traitement. Les scores cliniques de Hughes calcules avant et apres initiation des echanges pour chaque patient ont egalement ete compares. Resultats Parmi les 206 patients traites par EP, 30 l’ont ete pour une NPDC. Huit des 11 patients (73 %) traites pour une PIDC ont repondu aux EP, avec une amelioration significative du score Hughes (p = 0,01). Quatre des 5 patients (80 %) atteints de neuropathie dysglobulinemique et 6 des 9 patients (67 %) atteints de neuropathie a anti-MAG ont repondu aux EP avec pour les seconds une tendance a l’amelioration du score Hughes (p = 0,07). Aucun des 5 patients atteints d’un Lewis-Sumner ou d’un POEMS n’ont repondu aux EP. Discussion Les taux de reponse des patients traites pour une PIDC et une neuropathie dysglobulinemique sont similaires a ceux des essais cliniques ayant valide l’utilisation des EP dans ces deux indications. Les deux tiers des patients atteints de neuropathie a anti-MAG ont repondu traitement ; a notre connaissance aucune etude n’a a ce jour evalue l’efficacite des EP dans cette indication. Les EP semblent peu efficaces dans le Lewis-Sumner et le POEMS. Conclusion L’efficacite des EP observee chez les patients traites pour une neuropathie a anticorps anti-MAG semble prometteuse, des essais cliniques complementaires sont necessaires pour conforter ces premiers resultats.

A 26-year-old woman presented to a local community hospital with fever and abdominal pain. Clinical examination showed mild abdominal and bilateral lumbar tenderness. Blood analysis showed C-reactive protein of 401 mg/l, leukocyte count of 15,340/mm3, and serum creatinine of 71 μmol/l. Chest radiograph and abdominal ultrasound were normal. She was diagnosed with pyelonephritis despite negative urinalysis and was discharged. Soon after, her condition worsened with 5 kg weight loss, myalgia, and arthralgia. Two months later, she was readmitted with severe left flank pain. Contrast-enhanced abdominal computed tomography (CT) showed a large subcapsular hematoma surrounding the left kidney (Figure 1). Multiple bilateral intrarenal aneurysms of variable diameter (1–14 mm) were detected during the arterial phase (Figure 1) with the appearance of bunch of grapes. Multiple bilateral wedge-shaped low-attenuation involving cortex and medulla suggesting kidney infarcts were also detected (Supplementary Figure 1 online). Urinalyses showed microscopic hematuria and proteinuria of 0.9 g/24 h. Comprehensive autoimmune including Anti-neutrophil cytoplasmic antibodies (ANCA) research and viral screenings were negatives.