Undetectable mannose binding lectin and corticosteroids increase serious infection risk in Rheumatoid Arthritis

Background: Infection is the leading cause of death in rheumatoid arthritis (RA). Corticosteroid (CS) use is a known and important risk factor for serious infections (SIs). Mannose binding lectin (MBL) is a genetically determined component of the innate immune system implicated in neonatal infections. Objective: Our aim was to determine whether MBL deficiency is a risk factor for SIs in RA and to compare it with CS use and also synthetic and biologic disease-modifying antirheumatic drug (DMARD) therapy. Methods: Data on 228 patients with RA were collected for up to 7 years (median = 5.9 years). Serum MBL concentrations were determined in all patients receiving synthetic (n = 96) or biologic (n = 132) DMARD therapy. Results: High rates of SIs were observed in RA irrespective of treatment (17%). Similar rates of SIs were observed in synthetic and biologic DMARD users. The rates of single and multiple Sis were similar, irrespective of the use of a biologic agent. Undetectable MBL (\u3c56 ng/mL) concentrations and maintenance prednisolone at 10 mg per day or higher were associated with an increased risk for an SI, with incident risk ratio of 4.67 (P = .001) and 4.70 (P \u3c .001), respectively. Conclusions: Undetectable MBL and prednisolone confer a high risk for an SI. The use of biologic DMARDs did not confer substantial SI risk in this observational study. MBL deficiency is hitherto an unrecognized risk factor for an SI in RA

An unusual cause of granulomatous disease

BACKGROUND: Chronic granulomatous disease (CGD) is an inherited disorder of phagocytic cells caused by an inability to generate active microbicidal oxygen species required kill certain types of fungi and bacteria. This leads to recurrent life-threatening bacterial and fungal infections with tissue granuloma formation. CASE PRESENTATION: We describe a case of X-linked Chronic granulomatous disease (CGD) diagnosed in an 18-year-old male. He initially presented with granulomatous disease mimicking sarcoidosis and was treated with corticosteroids. He subsequently developed Burkholderia cepacia complex pneumonia and further investigation confirmed a diagnosis of CGD. CONCLUSION: Milder phenotypes of CGD are now being recognised. CGD should be considered in patients of any age with granulomatous diseases, especially if there is a history of recurrent or atypical infection

Clinical experience in T cell deficient patients

T cell disorders have been poorly understood until recently. Lack of knowledge of underlying molecular mechanisms together with incomplete data on long term outcome have made it difficult to assess prognosis and give the most effective treatment. Rapid progress in defining molecular defects, improved supportive care and much improved results from hematopoietic stem cell transplantation (HSCT) now mean that curative treatment is possible for many patients. However, this depends on prompt recognition, accurate diagnosis and careful treatment planning

Increasing recognition of dermatomyositis with subcutaneous edema – is this a poorer prognostic marker?

Subcutaneous edema as a presenting feature of dermatomyositis has infrequently been described and is thought to signify a more aggressive disease course. We report a case involving a 38-year-old man who presented with significant subcutaneous edema involving his neck and upper body; he later developed clinical features and biopsy results consistent with dermatomyositis. Only sixteen previous cases of dermatomyositis with subcutaneous edema involving adults have been published in the literature and we aim to review disease progression, prognosis, and optimal treatment of the condition

Falsely normal C4 in a case of acquired C1 esterase inhibitor deficiency

A 59‐year‐old lady presented with recurrent angioedema without urticaria. The clinical history and examination were consistent with an acquired C1 esterase deficiency secondary to lymphoproliferative disease. Despite a low C1 esterase level, the C4 level assayed by nephelometry on our automated analyser was normal. Analysis using different nephelometric analysers revealed consistently low C4, despite consistent normal readings in our analyser. Further investigation revealed an IgM‐κ paraprotein that seemed to interfere with both this and haematology coagulation assays. Splenic marginal zone lymphoma was confirmed on bone marrow biopsy. Monoclonal paraproteins may interfere with nephelometric, turbidimetric and immunological assays in a non‐antibody‐specific manner and should be considered when there are unusual or unexpected results, particularly in a patient with lymphoproliferative disease

Formation of the Australia and New Zealand Vasculitis Society to improve the care of patients with Vasculitis in Australian and New Zealand

The term vasculitis encompasses a group of disorders that result in the inflammation of blood vessels. These disorders can lead to irreversible damage in many organ systems. In Australia and New Zealand, practitioners from numerous specialties including rheumatology, immunology, nephrology, respiratory medicine, dermatology, ophthalmology, neurology and ear nose and throat (ENT) surgery manage patients with vasculitis. Not surprisingly, the patient’s experience of medical care can become fragmented. Australia and New Zealand centres have been active in vasculitis research over many years. Antineutrophil cytoplasmic antibodies (ANCA) were first described in Melbourne in 1982 by Davies et al., helping to define a major category of small vessel vasculitis – ANCAassociated vasculitis (AAV).1,2 This discovery led to further local research into ANCA testing and input into international testing guidelines.3 More recently, mechanistic studies have examined the loss of tolerance to ANCA antigens and the involvement of T cells in tissue injury,4 as well as the relationship between human leukocyte antigen (HLA) and disease.5 In addition, there is ongoing collaborative research into the contribution of genetic variation in the initiation and outcome of vasculitis