Publications by authors named "N Schleinitz"
Background: Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample-size and/or follow-up duration are available.
Methods: The COHESion study is a national prospective multicenter multidisciplinary cohort recruiting both adults or children with the spectrum of eosinophilic disorders (including reactive HE/HES [HE/HES-R], idiopathic HES [HES-I], lymphocytic HES [HES-L], neoplastic HE/HES [HE/HES-N], HE of unknown significance [HE-US], as well as IgG4-related disease [IgG4RD] or ANCA-negative eosinophilic granulomatosis with polyangiitis [EGPA] overlaps).
Purpose: CTLA4 deficiency is an inborn error of immunity (IEI) due to heterozygosity for germline loss-of-function variants of the CTLA4 gene located on chromosome 2q33.2. CTLA4 deficiency underlies pleiotropic immune and lymphoproliferation-mediated features with incomplete penetrance.
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- IgG4-related disease is a chronic immune disorder with no current approved treatment, and inebilizumab, which targets CD19+ B cells, is being tested as a potential therapy.
- In a phase 3 trial, 135 adults with active IgG4-related disease were randomly assigned to receive either inebilizumab or a placebo, and the primary measure was time until the first disease flare.
- Results showed that patients receiving inebilizumab had significantly fewer disease flares (10% vs. 60% in the placebo group), lower annual flare rates, and higher rates of complete remission without treatment compared to those in the placebo group.
Article Synopsis
- A study on European FMF patients found that 20% experienced a diagnostic delay of over 10 years, with affected individuals being significantly older.
- Women were more likely to have this diagnostic delay compared to men, and specific symptoms like erysipelas-like erythema were more prevalent in delayed diagnosis cases.
- Delayed-diagnosis patients also had higher occurrences of AA amyloidosis and were more frequently treated with biotherapy, indicating more severe disease consequences.
Aims: Hypophysitis is defined as an inflammation of the pituitary gland and/or infundibulum. Our aim was to characterize the initial course and evolution of patients with hypophysitis according to the different etiologies.
Patients And Methods: Retrospective observational study conducted in a universitary referral hospital center.