Chronic urticaria and mesangial proliferative glomerulonephritis: a case report
Monika Merkle1, Max Weiss2 and Markus Wörnle3
Keywords: chronic urticaria; hypocomplementemia; mesangial proliferative glomerulonephritis; nephrotic syndrome
We hereby report on a patient with chronic urticaria who presented with an acute nephrotic syndrome caused by a mesangial proliferative GN and that had the particular feature of persistently depressed serum complement C4 levels.
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A 34-year-old white female first presented with fatigue, oedema and a Raynaud's phenomenon. She was found to be hypertensive and had a nephrotic syndrome with elevated serum creatinine. The medical history was unremarkable except for chronic urticaria and hypothyroidism of unknown origin. She denied any arthralgias, myalgias, dysphagia, dyspnoea or abortions. Immunoserology showed an elevated ANA, positive SS-A antibodies and a profound hypocomplementemia. Kidney biopsy was performed, which showed some intracapillary glomerular precipitations, an irregular contour of glomerular capillaries and a moderate mesangial cell proliferation as well as a mild, focal interstitial nephritis. Immunofluorescence staining showed mesangial deposits of IgM with some IgG as well as C3 and C1q in the glomerular capillary loops. EM revealed immunoprecipitations in the mesangial cells including scarce deposits in the subepithelial space.
ReplyDeleteLupus nephritis was considered to be the most likely differential diagnosis, because patient did not fulfil the diagnostic criteria for SLE. Oral prednisone, cyclophosphamide and an ACE inhibitor were started because of the impaired kidney function and the nephrotic range proteinuria. After 6 weeks of therapy, serum creatinine and complement C3 levels were within normal limits, and proteinuria regressed below 1 g/gCrea after only 3 months. C4 remained suppressed. Cyclophosphamide was given over a total of 6 months and prednisone over 9 months. At 3 months following discontinuation of the oral steroid the patient was referred back because of an increase in proteinuria. Concomitantly, a microhematuria was found in the otherwise asymptomatic patient. Serum creatinine was normal. Although the response to oral steroid therapy was prompt, any prednisone dose reduction <7.5 mg/day was followed by the development of urticarial skin lesions and a relapse of proteinuria (Figure 1). Because the patient preferred not to switch to an alternative immunosuppressive regimen, she was kept on low-dose steroid treatment.