1 Department of Medicine, 2 Department of Nephrology, 3 Department of Urology, Christian Medical College, Ludhiana, Punjab, India.
Sir,
Lipodystrophy is a rare and unusual condition characterized by partial or total atrophy of subcutaneous adipose tissue. Partial lipodystrophy (Barraquer-Simons disease) [1] is seen to be associated with mesangiocapillary glomerulonephritis (MCGN) type II [2,3]. We are reporting one such patient who developed progressive renal failure and later underwent successful renal transplantation.
Case.
A 31-year-old male presented with pedal, periorbital oedema and hypertension. Physical examination revealed severe lipoatrophy involving the upper limbs, thorax and abdomen with complete loss of buccal fat. There was extensive and uniform fat deposition below the gluteal region. His blood pressure was 150/110 mmHg. His haemoglobin was 7.5 g/dl, urinalysis revealed 3+ proteinuria with 45 RBCs/high power field, and 24 h urinary protein losses were 2 g. Serum cholesterol was 302 mg/dl, triglycerides 819 mg/dl, and low density lipoprotein 1020 mg/dl. Blood urea was 47 mg/dl and creatinine 2 mg/dl. Serum C3 was low at 7.9 mg/dl (normal 85170 mg/dl) while C4 was 60.0 mg/dl (normal 1545 mg/dl). Magnetic resonance imaging demonstrated complete loss of subcutaneous fat in the upper half of the body with excessive fat deposition in the gluteal and thigh region. Percutaneous kidney biopsy showed features of MCGN type II characterized by hypercellular glomeruli which showed lobular accentuation with capsular adhesions in some tufts, capillary walls were thickened with deposits in the basement membrane which were enhanced by PAS staining. There was focal tubular atrophy with moderately dense focal cell infiltrate in the interstitium.
His renal function progressively deteriorated and he became haemodialysis dependent 3 years after diagnosis. He received a 1-hapto identical renal allograft from his father 2 months after starting dialysis. His immunosuppression comprised of cyclosporin, prednisolone and azathioprin. A renal biopsy done 20 weeks after transplantation for worsening renal allograft functions had evidence of cyclosporin nephrotoxicity, there was no evidence of recurrence of glomerulonephritis or rejection in the biopsy. The dose of cyclosporin was reduced, which resulted in improvement in renal function.
Comment.
Barraquer-Simons disease (partial lipodystrophy) is a rare syndrome. Renal disease is common in these patients, the most common being MCGN Type II (dense deposit disease) [2,3]. These patients are usually found to have asymptomatic proteinuria and microscopic haematuria on routine screening and nephrotic syndrome is occasionally present. A diminished serum concentration of the third component of complement in association with C3 nephritic factor is frequently present. The course of Type II MCGN is variable, the GFR remains stable in some patients and declines gradually to end-stage renal disease over 510 years in others [4]. At presentation or during the course of disease, heavy proteinuria, macroscopic haematuria and high quantitatively assessed urinary red cell or white cell counts characterize patients with progressive disease. Patients with crescents on their initial renal biopsy or with large number of polymorphs in glomerular capillaries corresponding with sterile pyuria are more likely to have deterioration of renal function [5]. Recurrent disease in the transplanted kidney is common, perhaps universal in type II MCGN [6]. Intramembranous dense deposits have been noted in 50100% of allograft biopsy specimens taken several weeks to many years after transplantation [7]. In a study by Bennett et al. (1989) the interval from transplantation to biopsy with proof of recurrence varied from 2 months to 8 years [5].
This patient who had Barraquer-Simons disease and MCGN Type II and underwent successful renal transplantation did not have evidence of recurrence of this disease. We have come across one similar case of MCGN type II and partial lipodystrophy who received a renal transplant which was rejected [8]. The patient, however, received a second renal transplant with lasting success. No recurrence was reported in either the first or the second transplant.
References