crescentic glomerulonephritis ( cgn ) is the most aggressive structural phenotype in the continuum of injury that results from glomerular inflammation . the term rapidly progressive glomerulonephritis ( rpgn ) is also interchangeably used with the term cgn . picgn can lead to end - stage renal disease ( esrd ) in 20% of cases despite treatment , and anca - associated vasculitis ( aav ) accounts for 4% of case of esrd in the uk . however , it can recur in the post - transplant period and can lead to graft loss . hence , nephrologists should be aware of this potentially disastrous complication of picgn in the post - transplant period . a 16-year - old adolescent male had complaints of weakness , malaise , anorexia , nausea , fever and vomiting for a period of 4 weeks . the patient was investigated for the above problems at his primary health centre , and the investigation revealed severe anaemia and deranged renal functions . there was no history of skin rash , joint pains , oral ulcers or native / herbal medicinal intake . general physical examination revealed pallor , pitting pedal edema in both feet with a blood pressure of 160/90 mmhg . the investigations revealed a hemoglobin level of 90 g / l ( 9 g / dl ) , total leukocyte count of 6600/cu mm and platelet count of 2  10 /cu mm . urine examination showed a dipstick albumin of 3 + with a 24-h urine protein of 2.4 g and 1520 red blood cells ( rbcs)/high power field with rbc casts . renal functions revealed a blood urea of 46.4 mmol / l ( 130 mg / dl ) and a serum creatinine of 265.2 mol / l ( 3 mg / dl ) , which had rapidly progressed to 884 mol / l ( 10 mg / dl ) after 7 days . antinuclear antibody and anca were negative ( by both enzyme - linked immunosorbent assay ( elisa ) and immunofluorescence ) . the patient 's serum tested negative for hbsag , anti - hepatitis c antibody and human immunodeficiency virus . ultrasonography of the kidney , ureter and bladder revealed normal - sized kidneys ( 10 cm on either side ) with a normal pelvicalyceal system . with a provisional diagnosis of rapidly progressive renal failure , the underlying glomerular tuft shows a segmental necrotising lesion with disruption of the glomerular basement membrane , fibrin exudation and presence of karyorrhectic debris indicating small vessel vasculitis ( h / e , 400 ) . the underlying glomerular tuft shows a segmental necrotising lesion with disruption of the glomerular basement membrane , fibrin exudation and presence of karyorrhectic debris indicating small vessel vasculitis ( h / e , 400 ) . a contrast - enhanced computerized tomogram of chest and abdomen , which was done to look for evidence of extra renal vasculitic involvement , was normal . an ocular fundus examination carried by an ophthalmologist was unremarkable . with the diagnosis of anca - negative picgn methylprednisolone pulse 15 mg / kg for three consecutive days followed by 1 mg / kg / day of oral prednisolone ) and monthly intravenous pulse cyclophosphamide therapy ( 12.5 mg / kg / pulse ) . his renal functions did not improve and he continued to be dialysis dependent for more than 3 months , so further cyclophosphamide pulse was not given and was declared esrd . the patient underwent 12 months of hemodialysis before undergoing a live - related renal transplantation from his father . the patient received no induction and immunosuppression was comprised of tacrolimus ( c0 was 11 ng / ml for first 3 months and then was maintained at 7 ng / ml ) , mycophenolate mofetil ( 2 g / day for first 3 months and then reduced to 1.5 g / day ) and prednisolone ( started at 0.5 mg / kg / day and was tapered and continued at 5 mg / day ) . his immediate postoperative period was unremarkable but for a slow fall in serum creatinine , which remained at 176.8 mol / l ( 2 mg / dl ) . a graft biopsy was done for the same , which revealed acute t - cell - mediated rejection . the patient was pulsed with methylprednisolone ( 500 mg for 3 days ) and his serum creatinine came down to a baseline of 88.4 mol / l ( 1 mg / dl ) . he was doing well with a creatinine level of around 88.4 mol / l ( 1 mg / dl ) till 8 months post transplant when he developed nausea , vomiting and loss of appetite . his investigations revealed a serum creatinine level of 176.8 mol / l ( 2 mg / dl ) and his urine analysis revealed albumin 2 + with 24-hour urine protein of 1.6 g and 1520 rbcs with rbc casts . serum creatinine progressed to 530.4 mol / l ( 6 mg / dl ) over 1 week . direct immunofluorescence and electron microscopy did not reveal any immune complexes , thus confirming a recurrence of picgn . 2.one of the glomeruli shows cellular crescent with the presence of fibrin within the crescent . the underlying tuft shows segmental necrosis with fibrin exudation and presence of nuclear debris , thus confirming small vessel vasculitis ( h / e , 400 ) . one of the glomeruli shows cellular crescent with the presence of fibrin within the crescent . the underlying tuft shows segmental necrosis with fibrin exudation and presence of nuclear debris , thus confirming small vessel vasculitis ( h / e , 400 ) . his treatment with mycophenolate mofetil ( mmf ) was stopped and he was started on cyclophosphamide pulses ( 15 mg / kg every 2 weeks for three doses followed by three doses every 3 weeks ) after giving pulse methylprednisolone ( 1 g / day for 3 days ) . his oral steroids were increased to 1 mg / kg / day for a period of 2 months and then tapered to 7.5 mg / day over next 4 months . seven sessions ( 60 ml / kg / session ) of plasmapheresis were given over a 2-week period . however , his renal functions worsened gradually and the serum creatinine had increased to 8 mg / dl over a period of 3 months . most cases of pauci - immune cgn ( picgn ) can be attributed to systemic small vessel vasculitides , including granulomatosis polyangitis , microscopic polyangiitis and churg  strauss syndrome and renal - limited vasculitis . these primary small vessel vasculitides are collectively known as aav , in reference to their serological hallmark . however , a subgroup of patients with picgn remain persistently negative for anca . in a large cohort of 213 patients with cgn , the likelihood of anca negativity was 2030% when the intensity of staining for immunoglobulins was 0 and 1 + , respectively ( on a scale of 04 + ) . anca - negative picgn tends to present at a much younger age than its anca - positive counterpart [ 57 ] . two studies compared the extra renal manifestations of patients with anca - negative picgn with anca - positive counterparts in detail . in the study by hedger et al . chen et al . reported anca - negative picgn had fewer constitutional and lesser extrarenal involvement than patients with anca positivity . renal involvement tends to be more severe in anca - negative picgn than anca - positive picgn across different ethnicities [ 58 ] . this may explain the poorer prognosis often documented in anca - negative picgn . despite advances in the diagnosis and treatment , 2040% of patients with picgn develop esrd and have to be treated with renal replacement therapy . our index patient also presented at a younger age , had no extra renal manifestations and had a severe renal disease progressing to end - stage renal failure despite the treatment offered . an antibody against human lysosome membrane protein-2 ( lamp-2 ) was reported in the pathogenesis of vasculitis . however , a recent large study failed to show any mechanistic relationship between anti - lamp-2 antibodies and glomerulonephritis . kidney transplantation is considered the treatment of choice in patients with esrd due to pauci - immune glomerulonephritis . it was shown in a large retrospective analysis of 59 aav patients with esrd from a single center that transplanted patients had better survival than those who remained on dialysis . recurrence of vasculitis post transplant , particularly in the graft , can occur and can be graft threatening . in 1983 , curtis et al . described the first biopsy - proven recurrence of focal necrotizing glomerulonephritis in the graft . although no prospective data are available to assess the likelihood of recurrent picgn after kidney transplantation , there are a few retrospective case series ( table  1 ) which have looked into the post - transplant renal recurrence and they have revealed a frequency of recurrent pauci - immune necrotizing gn varying from 4% to as high as 37% depending on different series . table  1.recurrence of picgn in the renal allograftstudynfollow - up ( months)recurrence n ( % ) time at relapse ( months)treatmentoutcomeanca statuslionel rostaing et al . 181 ( 100)1mp , cyp , ppgraft lossnegat the time of transplantation , mp , methylprednisolone ; cyp , cyclophosphamide ; pp , plasmapheresis ; nr , not related ; nm , not mentioned ; nc , not clear ; neg , negative ; aza , azathioprine ; mmf , mycophenolate mofetil.at the last follow - up , the mean levels of serum creatinine and proteinuria were more elevated in patients with recurrence than in the other vasculitic patients . recurrence of picgn in the renal allograft at the time of transplantation , mp , methylprednisolone ; cyp , cyclophosphamide ; pp , plasmapheresis ; nr , not related ; nm , not mentioned ; nc , not clear ; neg , negative ; aza , azathioprine ; mmf , mycophenolate mofetil . at the last follow - up , the mean levels of serum creatinine and proteinuria were more elevated in patients with recurrence than in the other vasculitic patients . however , recurrence of anca - negative picgn is restricted to a single case report . kai ming et al .   had reported a 41-year - old chinese woman who developed anca - negative picgn along with systemic manifestations in the form of cutaneous , ocular and neural involvement . she received a cadaveric transplant a year later and in the first post - transplant month she developed renal , cutaneous and ocular relapse which showed partial response to plasmapheresis , cyclophosphamide and high - dose steroids . our index patient had recurrence of the disease at 8 months post transplant , but unlike the above - mentioned patient the recurrence was limited to the graft kidney without any extrarenal involvement . though there is not much evidence to delay transplantation in picgn as far as the graft outcomes are concerned , it has been shown in multivariate analysis that kidney transplantation within 12 months of achieving remission was associated with increased mortality . the optimal therapeutic management of recurrent picgn is not clear , but the present day practice is similar to that of severe aav and includes a combination of cyclosphosphamide , steroids and plasmapheresis . recurrent picgn has been associated with poor long - term graft and patient survival . in conclusion , we report a rare case of recurrence of anca - negative renal - limited vasculitis manifesting as picgn in the grafted kidney . delaying transplantation for a period of at least 1 year of extrarenal remission and early diagnosis of recurrence by a graft biopsy may help to deal with this potentially graft - threatening complication of picgn .
renal transplantation is the treatment of choice for end - stage renal disease ( esrd ) due to pauci - immune crescentic glomerulonephritis ( picgn ) . 
 a small subgroup of patients with picgn are anti - neutrophil cytoplasmic antibody ( anca ) negative . 
 we report a case of a patient with anca - negative renal - limited form of picgn who developed esrd despite treatment . 
 he underwent live - related renal allograft transplantation after 12 months on haemodialysis . in the eighth post - transplant month , he developed graft dysfunction , which on evaluation turned out to be a graft recurrence of the basic disease in the form of picgn . 
 he received treatment with methylprednisolone , cyclophosphamide and plasmapheresis . 
 however , his renal functions did not improve and he developed graft loss in the 11th post - transplant month and was started on continuous ambulatory peritoneal dialysis . 
 we report a rare recurrence of renal - limited picgn in the allograft . 
 patients with picgn undergoing renal transplantation should be followed up carefully , and an early biopsy should be performed in the case of graft dysfunction to deal with this potentially graft - threatening complication .