VEXAS is a newly recognised adult-onset autoinflammatory syndrome resulting from a somatic mutation in the UBA1 gene. Herein, we present three cases of VEXAS syndrome in Sydney, Australia, that capture key clinical features and the refractory nature of the condition. They highlight the importance of multidisciplinary collaboration for early diagnosis and the need for new therapeutic options.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal condition characterized by inappropriate immune system activation leading to a “cytokine storm”, and ultimately resulting in end‐organ damage. Causes include primary defects in genes involved in immune‐mediated cytolytic pathways, or secondary triggers such as infection or malignancy. We describe a case of HLH precipitated by fungal infection which occurred as a consequence of immunosuppression for management of systemic lupus erythematosus (SLE) and necrotizing myopathy. The patient presented with immune‐mediated disease of the muscles and lung which was treated with high‐dose corticosteroids and aggressive immunosuppression. HLH emerged in the context of confirmed candidiasis and features of severe sepsis. The patient responded rapidly to antifungal therapy and high‐dose anakinra, which was administered subcutaneously and progressively weaned over 4 weeks. She completed HLH treatment as an outpatient and remains well at 12 months with controlled SLE and no recurrence of HLH.

ObjectiveTo analyse the Multidimensional Health Assessment Questionnaire (MDHAQ) in screening for anxiety in patients with rheumatoid arthritis (RA) and psoriatic arthritis (PsA), in comparison to the Hospital Anxiety and Depression Scale (HADS) as the reference standard.MethodsPatients with a physician diagnosis of RA or PsA were invited to complete the MDHAQ and HADS at their routine Rheumatology Clinic visit. Sensitivity, specificity, percent agreement and kappa statistics were used to evaluate agreement between two MDHAQ items for anxiety and HADS-anxiety (HADS-A) score of 8 or more. The first is a question asked on a 4-point scale (0-3.3) and the second is a "yes"/"no" (blank) item asked within a 60-symptom checklist (ROS).ResultsThe study included 183 participants, of whom 126 (68.9%) had RA and 57 (31.1%) had PsA. The mean age was 57.3 years and 66.7% were female. Positive screen for anxiety according to a HADS-A score of 8 or more was seen in 39.3%. Compared to a HADS-A score of 8 or more, an MDHAQ score of 2.2 or more OR positive on ROS had a sensitivity of 69.9%, specificity of 73.6% and substantial agreement (percent agreement 80.9%, kappa statistic 0.59).ConclusionThe MDHAQ provides information similar to the HADS to screen for anxiety in patients with RA and PsA. The use of this single questionnaire which can also be used to monitor clinical status, screen for fibromyalgia and depression without requiring multiple questionnaires, may present a valuable tool in routine clinical practice.