The Netherlands journal of medicine
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We present a case with five auto-immune phenomena, including Sjögren's syndrome, for which we also diagnosed a tip lesion of focal segmental glomerulosclerosis (FSGS).v About one-third of Sjögren's syndrome patients have renal involvement, but FSGS is rarely reported. FSGS is thought to involve T-cell dysfunction and in this patient with multiple auto-immune phenomena, it may reflect a severe dysregulation of cellular immunity.
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Case Reports
Case series of three adult patients with exceptional clinical presentations of haemophagocytic lymphohistiocytosis.
Macrophage activation syndrome (MAS) is a secondary form of haemophagocytic lymphohistiocytosis (HLH). MAS-HLH is an underrecognised and life-threatening condition associated with a heterogeneous group of diseases including connective tissue disease and inflammatory disorders. Here, we report three cases of adult patients with MAS-HLH triggered by different entities, including systemic lupus erythematosus, Griscelli syndrome type 2, and Adult onset Still's disease.
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Despite the availability of several guidelines on the diagnosis and treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), clinical routine practice will only improve when an implementation strategy is in place to support clinical decision making and adequate implementation of guidelines. We describe here an initiative to establish national and multidisciplinary consensus on broad aspects of the diagnosis and treatment of AAV relevant to daily clinical practice in the Netherlands. ⋯ We present a national, multidisciplinary consensus on a diagnostic strategy and treatment algorithm for AAV patients as part of the implementation of (inter)national guideline-derived recommendations in the Netherlands. Future studies will focus on evaluating local implementation of treatment protocols for AAV, and assessments of current and future clinical practice variation in the care for AAV patients in the Netherlands.