Aim Immunoglobulin‐associated mesangiocapillary glomerulonephritis is currently the most common biopsy‐confirmed glomerulonephritis in Cape Town, South Africa. We aimed to determine the outcome of patients with a biopsy‐confirmed diagnosis of immunoglobulin‐associated mesangiocapillary glomerulonephritis at our centre. Methods A retrospective cohort study of adult patients was conducted from January 1, 2000 to December 31, 2016. The endpoint was a composite of doubling of creatinine and/or end‐stage renal disease and/or death. Cox univariable and multivariable proportional hazards models were used to examine the association between the composite endpoint and predictor variables. Survival curves were made with the use of Kaplan‐Meier estimates. Results A total of 70 patients were included in the study and their median duration of follow‐up was 30.4 months. Forty‐eight (68.6%) patients reached the composite endpoint. The proportion reaching this endpoint at 1, 3 and 5 years were 37.5%, 64.6% and 81.3%, respectively. Cox multivariable proportional hazards model identified a serum creatinine concentration > 200 μmol/L at the time of biopsy, moderate to severe interstitial fibrosis, ≥50% crescents and cyclophosphamide therapy as predictors of the composite endpoint. Conclusion Immunoglobulin‐associated mesangiocapillary glomerulonephritis remains a common glomerular pathological diagnosis in our setting and has poor outcomes. This may partially be explained by late presentation. Future research needs to focus on identifying the possible cause(s) of this common glomerular disease so that more targeted therapeutic approaches can be offered.
Laboratory showed a nephrotic syndrome with proteinuria at 5.87 g/24h and serum albumin at 9g/l, serum creatinine at 70 umol/ l, urea at 3,8mmol/l. Immunoassay showed positive rheumatoid factor and anti-CCP positive. Electrophoresis of serum proteins showed a narrow band of monoclonal aspect migrating into the gamma zone ( Figure 1). Immunoelectrophoresis has demonstrated monoclonal gammopathy of IgG Lambda type. Radiography of the whole spine showed vertebral fractures with diffuse deregulation.Radiological exams showed diffuse mediacalcosis on Pelvic and lower limb X-ray (Figure 2 and4). and bilateral carotid atherosclerotic calcifications predominant in the carotid bulbs, atheromatous infiltration with mediacalcosis of the thoracoabdominal aorta and its branches of bifurcation on thoraco-abdomino-pelvic CT (Figure 3).Biopsy of accessory salivary glands showed chronic sialadenitis with amyloid deposits. The myelogram showed a 17.4% plasmocytosis with an estimated macrophage excess of 9.8%, an estimated 6.9% macrophage excess of myeloblast, and hemophagocyte images. Erythroblasts displayed dysplasia on 21% of the binuclear lineage, carryorhexis and laminated cytoplasm. We established the diagnosis of MM and renal amyloidosis. Before starting treatment, follow up was marked by infectious complication of CUA lesions. The patient died as a result of septic shock. Conclusions: CUA is a rare and severe condition which can often lead to death due to infectious complications and comorbidities, the association with MM is rare leading to dramatic outcomes.
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