J. Ninet

Aseptic abscesses: a study of 30 patients with or without inflammatory bowel disease and review of the literature.

Aseptic abscesses (AA) are characterized by deep, sterile, round lesions consisting of neutrophil that do not respond to antibiotics but improve dramatically with corticosteroids. We report the clinical, laboratory, and radiologic characteristics and the associated conditions of 29 patients from the French Register on AA plus 1 patient from the Netherlands. The mean age of patients at AA diagnosis was 29 years (SD = 14). The main clinical manifestations of AA were fever (90%), abdominal pain (67%), and weight loss (50%). Duration of symptoms was 4.7 months on average until abscesses were discovered. The abscesses involved the spleen in 27/29 patients (93%; the thirtieth patient had a personal history of splenectomy after a trauma). In 7 they were unifocal and in the others they were multifocal, involving in addition the abdominal lymph nodes in 14 (48%), liver in 12 (40%), lung in 5 (17%), pancreas in 2 (7%), and brain in 2 (7%). They were not splenic in 3, including 2 with abdominal lymph nodes and 1 with superficial lymph nodes and testicle and lung involvement. Twenty-two patients (70%) had elevated white blood cell and neutrophil count; antineutrophil cytoplasmic autoantibodies with a perinuclear, cytoplasmic or atypical pattern (negative for antiproteinase 3 and negative for antimyeloperoxidase except for 1) were positive in 21% of the 24 patients tested. Twenty-one patients had inflammatory bowel disease (IBD), which preceded the occurrence of abscesses in 7, was concomitant in 7, and appeared secondarily in 7. Six patients had neutrophilic dermatosis (20%), 3 had relapsing polychondritis as an associated condition, and 3 others had monoclonal gammopathy of undetermined significance. Three patients had no associated condition. Splenectomy was performed in 15 (52%) patients. All patients received steroid therapy. Thirteen (43%) were given additional immunosuppressive therapy, 1 immediately and the others after a relapse, of whom 3 were also treated by anti-tumor necrosis factor-alpha agents. Mean follow-up was 7 years. Eighteen (60%) patients experienced 1 or several relapses, but there was no death related to AA. Relapses occurred on immunosuppressive therapy in 2 patients and off immunosuppressive therapy in the others while corticosteroids were being tapered. We surveyed the literature and analyzed 19 additional cases. AA is an emergent and probably underrecognized entity. It represents an apparently noninfectious inflammatory disorder involving neutrophils that responds to corticosteroid therapy. AA mainly affects patients with IBD but also affects those with other conditions, or with no other apparent disease.Abbreviations: AA = aseptic abscess, AFB = acid-fast bacillus, ANCA = antineutrophil cytoplasmic antibodies, ASCA = anti-Saccharomyces cerevisiae antibodies, BOOP = bronchiolitis obliterans organizing pneumonia, CD = Crohn disease, CRMO = chronic recurrent multifocal osteomyelitis, CRP = C-reactive protein, CT = computed tomography, EED = erythema elevatum diutinum, ESR = erythrocyte sedimentation rate, HES = hematoxylin-eosin-saffron stain, IBD = inflammatory bowel disease, IC = indeterminate colitis, MGUS = monoclonal gammopathy of undetermined significance, PCR = polymerase chain reaction, PG = pyoderma gangrenosum, PMN = polymorphonuclear neutrophils, RA = rheumatoid arthritis, RP = relapsing polychondritis, SAPHO = synovitis, acne, pustulosis, hyperostosis, and osteomyelitis syndrome, SS = Sweet syndrome, TNF = tumor necrosis factor, UC = ulcerative colitis, WBC = white blood cell count.

Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: A french multicenter study

The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5years (range: 1.6-35). Two patients developed Waldenströms macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown.

Treatment of Systemic Necrotizing Vasculitides in Patients Aged Sixty‐Five Years or Older: Results of a Multicenter, Open‐Label, Randomized Controlled Trial of Corticosteroid and Cyclophosphamide–Based Induction Therapy

To investigate a new therapeutic strategy, with rapid corticosteroid dose tapering and limited cyclophosphamide (CYC) exposure, for older patients with systemic necrotizing vasculitides (SNVs; polyarteritis nodosa [PAN], granulomatosis with polyangiitis [Wegneners] [GPA], microscopic polyangiitis [MPA], or eosinophilic GPA [Churg‐Strauss] [EGPA]).