The clinical phenotype and evolution of antisynthetase syndrome (ASS) are heterogeneous.
This study was therefore undertaken to identify subgroups of ASS patients with similar …

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It
has been mainly described in Asia. There are few data available on this disease in Europe. …

Seven autoantibodies directed against synthetases have been identified to date, the best
known being anti-Jo1. Synthetases play a vital role in protein synthesis by catalyzing the …

Background The combination of cyclophosphamide and glucocorticoids leads to remission
in most patients with antineutrophil cytoplasm antibody (ANCA)–associated vasculitides. …

Objective Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg‐Strauss) (EGPA),
with limited patient numbers and followup durations, demonstrated that clinical …

Background Current standard therapy for Wegener's granulomatosis and microscopic
polyangiitis combines corticosteroids and cyclophosphamide to induce remission, followed by a …

Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA).
In this context, the early identification of a severe, antibody-mediated, ADAMTS13 …

X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies characterized
by a particular vulnerability toward Epstein-Barr virus infection, frequently resulting in …

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with
noncodified therapeutic management and high mortality. No treatment has yet been shown to …

Objective. To describe characteristics and outcomes of vasculitides associated with
malignancies. Methods. The requirement for inclusion in this retrospective, 10-year study was …