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Background: Cryopyrin‐associated periodic syndrome (CAPS) is an autoinflammatory disorder with a spectrum ranging from the milder familial cold auto‐inflammatory syndrome (FCAS) phenotype to the more severe Muckle–Wells Syndrome. Although headaches are the most common neurological symptoms, neuroinflammatory diseases such as multiple sclerosis (MS) have only been reported in the more severe phenotypes. Case Presentation: We describe a 43‐year‐old male diagnosed with relapse‐remitting MS who developed cold‐induced arthralgias and urticaria. He initially presented with facial numbness and arthralgias in his late teenage years, and brain magnetic resonance imaging (MRI) demonstrated multiple lesions with periventricular white matter extension. Oligoclonal bands were detected in the cerebrospinal fluid (CSF). Symptoms gradually improved, and a decade later he presented with numbness at upper limbs and tongue. Brain and spine MRI showed new lesions, but CSF was unremarkable. He was started on interferon β‐1a with symptomatic improvement until another flare around 13 years later. Treatment was changed to dimethyl fumarate. During this hospitalization, urticaria was problematic and particularly associated with cold temperatures. This raised suspicion for possible CAPS and subsequently confirmed with an A439V mutation in the NLRP3 gene, consistent with FCAS. Anakinra was started but was not tolerated because of localized reactions despite desensitization. His serum amyloid A was mildly elevated at 9 mg/L (reference range 0–6). He remains stable from a neurological perspective, including imaging, and continues dimethyl fumarate. Conclusions: To date, FCAS and possible association with MS has not been described. The case raises challenges in the diagnosis of concurrent disease processes. It also highlights that neurological sequelae should be considered in all spectrums of CAPS, and distinctions with MS should be made where possible. [ABSTRACT FROM AUTHOR]

Giant cell arteritis is a medical emergency because of the high risk of irreversible blindness and cerebrovascular accidents. While elevated inflammatory markers, temporal artery biopsy and modern imaging modalities are useful diagnostic aids, thorough history taking and clinical acumen still remain key elements in establishing a timely diagnosis. Glucocorticoids are the cornerstone of treatment but are associated with high relapse rates and side effects. Targeted biologic agents may open up new treatment approaches in the future. [ABSTRACT FROM AUTHOR]

Background: IgG4 related disease (IgG4RD) is a newly described multisystem fibro‐inflammatory disorder. There is a paucity of literature describing the Australian experience of this rare condition. Aims: To characterise the Royal Adelaide Hospital IgG4RD cohort with biopsy‐proven disease. Methods: A search of the Frome Road SA Pathology database was performed for all tissue biopsies containing the phrase 'IgG4 positive'. Case notes were reviewed for clinical details, laboratory and radiology results. Histological features according to the Boston Criteria were used. Patients with available case notes, highly suggestive or probable histology and clinical features to suggest IgG4RD were included. Results: Twenty patients had definite or probable IgG4RD and suggestive clinical features; median age 59 (20–76), male : female 1.5:1. There was considerable delay in diagnosis (median diagnosis at 64 months). Organ involvement included: 11 exocrine gland, seven pancreatobiliary, seven nodal, seven soft tissue, five retro‐orbital, three retroperitoneal fibrosis and two renal. Systemic symptoms at diagnosis were seen in eight patients. Seven (35%) had an elevated serum IgG4 (>1.35 g/L) at diagnosis. Only 12 (60%) required immunosuppressive treatment (corticosteroids); of these, four (20%) required a steroid‐sparing agent and four (20%) required B‐cell depleting therapy (rituximab). The median duration of follow up was 18 months. Conclusions: This is the first characterised Australian cohort with generalised IgG4RD, a rare, relatively indolent and under‐recognised multisystem disorder. Diagnosis is difficult given lack of awareness of this rare condition among physicians, its presentation as a great disease mimic, challenges with histopathological assessment and the absence of a suitable serum biomarker. [ABSTRACT FROM AUTHOR]

Abstract: Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Although TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal. In all adults, urgent, empirical plasma exchange (PE) should be started within 4–8 h of presentation for a possible diagnosis of TTP, pending a result for ADAMTS13 (a disintegrin and metalloprotease thrombospondin, number 13) activity. A sodium citrate plasma sample should be collected for ADAMTS13 testing prior to any plasma therapy. In children, Shiga toxin‐associated haemolytic uraemic syndrome due to infection with Escherichia coli (STEC‐HUS) is the commonest cause of TMA, and is managed supportively. If TTP and STEC‐HUS have been excluded, a diagnosis of aHUS should be considered, for which treatment is with the monoclonal complement C5 inhibitor, eculizumab. Although early confirmation of aHUS is often not possible, except in the minority of patients in whom auto‐antibodies against factor H are identified, genetic testing ultimately reveals a complement‐related mutation in a significant proportion of aHUS cases. The presence of other TMA‐associated conditions (e.g. infection, pregnancy/postpartum and malignant hypertension) does not exclude TTP or aHUS as the underlying cause of TMA. [ABSTRACT FROM AUTHOR]

Urticaria is a common condition that occurs in both children and adults. Most cases have no specific allergic trigger and the aetiology of urticaria remains idiopathic and occasionally spontaneous in nature. Inappropriate advice such as avoidance of foods (milk, egg, prawn, and brinjal) is common place in certain sections of India mostly by nonspecialists that should not be routinely recommended. It is important to look for physical urticarias such as pressure urticaria in chronic cases, which may be present either alone or in combination with other causes. Autoimmune causes for chronic urticaria have been found to play an important role in a significant proportion of patients. Long-acting nonsedating antihistamines at higher than the standard doses is safe and effective.Quality of life is affected adversely in patients with chronic symptomatic urticaria and somemay requiremultidisciplinary management. [ABSTRACT FROM AUTHOR]

In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis ( IIM) were treated with intravenous immunoglobulin ( IVIg). We reviewed disease characteristics to determine predictors of response to therapy and IVIg dosing and duration to identify opportunities to rationalise IVIg use. Patients with dermatomyositis/polymyositis had a response rate of 77% and were more likely than inclusion body myositis to respond to therapy. Consideration should be given to the use of the lowest possible dose of IVIg and to the undertaking of trials of cessation of IVIg in patients with stable IIM. [ABSTRACT FROM AUTHOR]

An abstract is presented of an article on the case study of a man diagnosed with drug induced hypersensitivity syndrome which was existing secondary to allopurinol.

An abstract is presented of an article featuring case study of a woman suffering from cytophagic histiocytic panniculitis.

The article presents an abstract on the study which investigates the use of basophil activation test (BAT) for the diagnosis of type 1 hypersensitivity reaction mediated by beta-lactam antibiotics.