Anthony J. Nicholls

Mesangiocapillary glomerulonephritis type 2 associated with familial partial lipodystrophy (Dunnigan-Kobberling syndrome)

The lipodystrophies are a heterogeneous group of disorders of adipose tissue associated with insulin resistance. The sporadic form of partial lipodystrophy, characterised by fat loss from the face and upper body, is associated with complement abnormalities and mesangiocapillary glomerulonephritis type 2 (MCGN II) and the conditions are thought to have a shared autoimmune aetiology. We present the first case of the rare familial form of partial lipodystrophy, caused by a mutation in the LMNA gene, associated with MCGN II. This suggests that partial lipodystrophy of both the sporadic and familial subtypes may predispose to this condition and that the observed renal and complement abnormalities may be secondary to other factors associated with lipodystrophy.

Renal Vasculitis: Increasingly a Disease of the Elderly?

Background/Aims: The majority of patients presenting to our district general hospital with renal vasculitis are elderly. Older patients respond less well to treatment and have a poorer prognosis. We investigated the relationship between age and outcome of renal vasculitis in our centre and examined the evidence regarding treatment of elderly patients with this condition. Methods: Patients presenting over a 2-year period with renal vasculitis were identified by clinical and histological features and by antineutrophil cytoplasmic autoantibody positivity. They were followed for a mean of 15 months and outcomes were recorded. Results were compared with published studies. Results: The mean age at presentation of 21 patients was 69 years. Forty-eight percent required dialysis and there was a 33% overall mortality. The mean age of patients in previous treatment studies has been between 50 and 55 years. Conclusions: The greater severity of disease at presentation and poorer outcome than previously described is likely to be due to the high proportion of elderly patients. The incidence of vasculitis is increasing in the elderly but as this group has been poorly represented in clinical trials in renal vasculitis, applying the findings of these trials to their treatment may be hazardous. Future research should determine which treatments are safe and effective in the elderly.

Systemic vasculitis and p-ANCA positivity developing in a patient with ulcerative colitis and the antiphospholipid syndrome.

Accessible online at: www.karger.com/journals/nef Dear Sir, Ulcerative colitis (UC) is typically associated with antineutrophil cytoplasmic antibodies of perinuclear type (p-ANCA). These antibodies are not usually considered to carry potential for the development of systemic vasculitis as they lack specificity for proteinase 3 (PR3) or myeloperoxidase (MPO). We report a p-ANCA-positive patient with UC who developed small vessel systemic vasculitis, renal involvement and the antiphospholipid syndrome. The p-ANCA were shown to be specific for PR3. This case emphasises the importance of determining the antigenic specificity of ANCA in patients with UC. PR3 reactivity carries potential for systemic vasculitis and appropriate monitoring is essential.