Post-transplantation Presentation of ANCA-associated Vasculitis: Granulomatosis with Polyangitis.

Granulomatosis with polyangitis (GPA) is characterized by necrotizing granulomatosis of the upper and lower respiratory tract and glomerulonephritis. If GPA does not respond to appropriate management, it might result in end-stage renal disease, which may remit the disease severity. The overall impression is that immunosuppression following renal transplantation would further subside the vasculitis. However, several studies have shown that systemic vasculitis recur in 25% of patients following renal transplantation. This may indicate the perplexing nature of the immune system. One of the key factors in prevention of relapse of GPA is following up of patients by careful immunosuppressive dose adjustment and regular measurement of biomarkers for vasculitis. Herein, we describe an interesting case of biopsy-proven GPA who had a complex long history of several post-transplantation relapses in different organs with anti-neutrophil cytoplasmic antibodies seroconversion. This case emphasizes that vasculitis in particular GPA can mimic various diseases depending on which vessels and organs are affected by the inflammation and is one of the reversible causes of failure of transplanted kidney. Bearing the diagnosis in mind as one of the potential differential diagnoses of failure of renal transplantation will lead to early diagnosis and treatment of recurrent GPA.

Socioeconomic status of Iranian living unrelated kidney donors: a multicenter study.

Renal transplantation is the best treatment modality for end-stage renal disease (ESRD) patients. In Iran, a living unrelated donor (LURD) transplant program was started in 1988, because of the shortage of living related and cadaver donors. In this study, we evaluated the socioeconomic status of LURD in Iran. We enrolled 478 LURDs from 30 transplant centers between October 2005 and March 2006. The demographic data, education, employment, motivation, and satisfaction status were asked. Economic status was assessed using criteria of the statistics center of Iran. Ninety-six percent of donors were referred from the Kidney Foundation of Iran (KFI). The mean age of the donors was 27 +/- 4.8 years and 85% were men (n=408). Fifty-one percent were smokers, 82% married, and 79% the breadwinner; 29% were unemployed; 2.7%, 90.8%, and 6.5% were illiterate, school graduates, and university graduates, respectively. Coercion was documented for only one donor. Financial issues were the most frequent motive. Among them, 91% were satisfied with the donation. Finally, 53% suggested kidney donation to others. Of donors, 62% were living below the poverty line. In the Iran model, the KFI is a bridge that connects recipients and donors. As a result, there is no middle man or broker introducing donors to recipients. The transplantation team knows nothing about money transactions between recipient and donor. Most donors were satisfied with the donation. However, establishment of a government-regulated program for social support of donors, such as lifelong health insurance may be a compensation for donors.

A study of membranoproliferative glomerulonephritis in Iran.

BACKGROUND: The aim of this study was to review the morphologic patterns of membranoproliferative glomerulonephritis (MPGN) in 100 Iranian patients using light microscopy (LM) and electron microscopy (EM), and to compare the treatment and outcome in 13 patients with two biopsies. PATIENTS AND METHODS: A retrospective study of 713 kidney biopsies of Iranian patients received between 1981 to 1994 was carried out. Of the 713 kidney biopsies, MPGN (n=106) and membranous glomerulopathy (n=112) made up the highest numbers of cases. RESULTS: Among 100 MPGN patients, 55 (55%) were MPGN type I, 10 were type II (10%), and 35 type III (35%). Eighty-three (83%) had nephrotic proteinuria, 39 (39%) had hematuria, and 52 (52%) were hypertensive. Complement levels were estimated in 58, with low C3 in 10. The glomerular involvement was irregular, with focal hypercellularity in 47 patients (47%), widely patent capillaries in 50 (50%), arteriosclerosis in 48 (48%), and with hyaline change in 25 (25%). Follow-up data (22-130 months) was available in 61 (61%) patients: 6 (10%) died after 14-56 months, 27 (44%) were on maintenance hemodialysis for 15-110 months, and three received transplants. Thirteen patients had detailed follow-up and a second biopsy after 24-120 months. All 13 presented with edema and nephrotic range proteinuria, with hematuria and hypertension in five and azotemia in four. Seven of the 13 patients received initial steroids, followed by antiplatelet or antihypertensive drugs. Four (type III) patients received antiplatelet and antihypertension drugs, and two (type III) received only antihypertensive drugs. In the first biopsy, glomerular changes by light microscopy were non-uniform in 7 of 10 (70%) type III MPGN cases. Vascular changes were absent or mild in 11, and moderate in two. In the second biopsies, 10 showed decrease in cellularity, with many open capillaries, persistence of deposits by EM in all, and progression of vascular sclerosis in eight, and tubulointerstitial changes in 10. Among the 13, six were clinically stable, another six received dialysis followed by transplant in three, and one had relapses with episodes of cryoglobulinemia. Three patients died. CONCLUSION: There is a high incidence of MPGN in Iranian patients, with a substantial number of type III MPGN cases. Second biopsies showed decreased cellularity, but increase in chronic tubulointerstitial and vascular cases. Steroids did not appear to benefit the outcome in types I and III MPGN patients compared to patients who received antihypertensive and antiplatelet treatment without steroids.

Suggested guidelines for treatment of acute renal failure in earthquake victims.

The 1990 Iran earthquake affected two states with a combined population of 2,300,000. It resulted in at least 13,888 deaths, 43,390 injured, and 33,616 hospitalized. The overall mortality among hospitalized patients was 0.17%. Acute renal failure (ARF) requiring dialysis support was diagnosed in 156 patients nationwide, with a mortality rate of 14%. Three teaching hospitals of Tehran University of Medical Sciences (UMS) admitted 495 patients, with total mortality of 7.5%. Of these, 30 patients (6%) required dialysis, with a mortality of 40%, accounting for 12 of the deaths. On admission, patients with ARF were more severely injured and had significantly higher incidence of multiple trauma; peripheral nerve injury; elevated muscle enzymes, potassium, and phosphorus; and abnormal urinalysis. In one of these hospitals, patients were treated with a specific hydration protocol coupled with a cautious approach to fasciotomy. The incidence of ARF, mortality associated with ARF, and fasciotomy were lower in this group. Based on these findings, guidelines are suggested that may be helpful in the treatment of ARF in earthquake victims.

Arteriovenous fistula following nephrectomy.

UNLABELLED: Arteriovenous fistula (AVF) of the renal pedicle is a rare complication of nephrectomy. Since the 1st case report in the literature, 62 cases have been reported in the world literature. A continuous abdominal or lumbar bruit is diagnosed. They may also present with symptoms of congestive heart failure and/or hypertension. METHODS: 37 years following nephrectomy, a case of AVF of the right renal pedicle associated with gross proteinuria is described. RESULTS: The clinical diagnosis was confirmed by aortogram, and the proteinuria subsided after successful surgical management. CONCLUSION: The causes of proteinuria may be due to the hyperfiltration state. To our knowledge, there has been no previous report of this combination in the literature.

Successful renal transplantation in polyarteritis nodosa.

Hemodialysis and renal transplantation were performed in a patient with end stage renal disease caused by polyarteritis nodosa. Severe peripheral neuropathy developed during hemodialysis. Five years after transplantation, marked improvement of the neuropathy associated with adequate function of the allograft has been noted. There has been no detectable clinical or pathologic signs of recurrent disease. These observations favor the consideration of renal transplantation in patients with polyarteritis nodosa and renal failure, particularly in the absence of extensive organ involvement.