Myeloperoxidase-positive ANCA-associated vasculitis presenting as myalgia, proximal weakness and a normal CK.

We describe an unusual presentation of myeloperoxidase positive antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis managed by a multidisciplinary approach. A 75-year-old man gave a 3-week history of proximal lower limb weakness and exertional myalgia. His serum creatine kinase was normal and many of his non-specific symptoms suggested small vessel vasculitis. His investigations for common causes of muscle weakness were normal, and renal biopsy was normal despite haemoproteinuria. CT scan of the chest identified a pulmonary nodule of uncertain significance, not amenable to biopsy. MR scan of the thighs showed muscle oedema, and muscle biopsy confirmed typical features of vasculitis. Following high-dose corticosteroids his exertional myalgia quickly resolved and his normal mobility returned. Early immunosuppression is essential to improving clinical outcomes in ANCA-associated vasculitis, but diagnostic investigations often lack sensitivity.

Assessment of living kidney donors and adherence to national live donor guidelines in the UK.

BACKGROUND: As in most industrialized countries, living kidney donation has increased considerably in the UK and now amounts to 38% of the total UK kidney transplant activity. Living kidney donation guidelines have been formulated by the relevant national societies. We were interested to study whether or not renal units across the UK adhere to those guidelines and to delineate areas of inconsistency and controversy. METHODS: Twenty-four adult kidney transplant centres and 50 adult non-transplant renal units across the UK were contacted by a postal questionnaire from January to April 2011. RESULTS: Twenty-one of 24 (88%) transplanting units and 23 of 50 (46%) non-transplanting units responded. Eighty-one per cent of the responding transplanting units and only 30% of the non-transplanting units have a dedicated live donor clinic. Eighty-six per cent of all units are without a set upper age limit for donors, whereas 7% of units excluded all potential donors older than 70 years. Twenty per cent of units accept donors with body mass indices (BMIs) up to 35, whereas 9% of units did not have an upper limit for BMI. Thirty-two per cent of centres exclude hypertensive donors on more than one antihypertensive drug, whereas 64% of units exclude donors only if they are on more than two anti-hypertensive drugs. of units rely on a spot urine sample to assess proteinuria, while 30% of units still perform 24 h urine collection. Sixty one per cent of units perform computed tomography (CT) angiography to assess the renal vessels prior to donation, while 32% use magnetic resonance (MR) angiography. Seventy-five per cent of centres assess split kidney function by radionuclide testing in all cases, whereas 25% perform this test only if there is a discrepancy in kidney size. The practice of suspension of recipients with potential living donor from the deceased waiting list is also remarkably inconsistent, whereby some centres suspend once a decision for living donation has been made in principle, whereas others suspend the recipient only once a date for surgery has been agreed. CONCLUSIONS: We demonstrate significant variability in accepting living kidney donors, particularly regarding age, BMI, and hypertension. Infrastructure setting for living donation and the live donor assessment are also remarkably inconsistent across the UK. It remains unclear as to why nephrologists decide not to adhere to established guidelines and further research should aim to find the lacking evidence in areas causing inconsistency in living donor assessment.