Association between idiopathic inflammatory myopathies (IIMs) and malignancy is well known and has been extensively reported in the literature. However, in the recent years, several new studies were published allowing us to better understand the clinical characteristics and pathophysiology of cancer-associated IIMs. We conducted a literature review of cancer-associated IIMs focusing on new data that was published in the recent years.
Anti-HMGCR myopathy is a subtype of immune-mediated necrotizing myopathy, typically associated with exposure to statins, although a sizable minority in some cohorts are statin-naive. Although the clinical features of acute- or subacute-onset symmetrical proximal muscle weakness mimic those of other idiopathic inflammatory myopathies, necrotizing myopathy is distinguished by the histopathological findings of muscle fiber necrosis and regeneration with little to no accompanying inflammation. Several recent studies of patients with anti-HMGCR myopathy have identified a slightly increased risk of cancer. Most patients require aggressive immunotherapy, usually as a combination of 2 or 3 immunosuppressant drugs. We report a case of a statin-naive paraneoplastic anti-HMGCR myopathy, who unlike other reported cases, responded to a single dose of 1000 mg of intravenous rituximab and subsequent chemoradiation therapy for an underlying lung cancer, despite failing to completely respond to prior high-dose oral prednisone and methotrexate.
Aim To review the incidence of renal involvement and the natural course of Henoch Schönlein Purpura (HSP) over a 5 year period at a tertiary paediatric hospital. Method Patients were followed-up over a 1 year period, with frequency of visits dependent on proteinuria. At each visit a minimum of blood pressure and urinalysis was performed. Using a standardised data collection pathway all clinical data was collected prospectively. The primary outcome measured was the need for a consultant referral either during or after completing a year of follow-up. Results 165 patients were identifi ed presenting over 69 months, giving an incidence of 14.5 HSP cases per 100 000 children per year attending our hospital. The mean age at presentation was 7.2 years, with those requiring a renal referral signifi cantly older group.bmj.com on June 19, 2015 -Published by http://adc.bmj.com/ Downloaded from
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