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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 324-326
C1q nephropathy presenting as acute renal failure


Department of Nephrology, Lilavati Hospital and Research Centre, Mumbai, India

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Date of Web Publication18-Mar-2011
 

   Abstract 

We describe a 42-year-old male patient who presented with high grade fever asso­ciated with acute renal failure requiring hemodialysis. Renal biopsy revealed that he had focal proliferative glomerulonephritis on light microscopy, dominant mesangial deposition of C1q by immunofluorescent staining, and electron dense deposits on electron microscopy, with no evi­dence of systemic lupus erythematosus, compatible with the diagnosis of C1q nephropathy. Intensive treatment with a combination of methyl prednisolone pulse therapy and oral predni­solone was successful in achieving complete remission and disappearance of proteinuria in our patient.

How to cite this article:
Malleshappa P, Ranganath R, Chaudhari AP, Ayiangar A, Lohitaksha S. C1q nephropathy presenting as acute renal failure. Saudi J Kidney Dis Transpl 2011;22:324-6

How to cite this URL:
Malleshappa P, Ranganath R, Chaudhari AP, Ayiangar A, Lohitaksha S. C1q nephropathy presenting as acute renal failure. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2014 Oct 25];22:324-6. Available from: http://www.sjkdt.org/text.asp?2011/22/2/324/77620

   Introduction Top


C1q nephropathy is an immune complex glo­merulonephritis defined by the presence of me­sangial immunoglobulins and complement de­posits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. [1] C1q nephropathy often manifests as steroid-resistant asymptomatic pro­teinuria or nephrotic syndrome. It can also pre­sent with asymptomatic hematuria. Patients presenting primarily with acute renal failure requiring dialysis have not been reported in C1q nephropathy thus far.


   Case Report Top


A 42-year-old male patient presented to us with high-grade fever with chills, nausea and vomiting for the preceding five days, and de­creased urine output for the last two days. He denied any history of dysuria, hematuria, skin rash, joint pains and pain abdomen. He was a known hypertensive which was under good control with medications and was also suffe­ring from alcoholic liver disease. On examina­tion, he was febrile, icteric and breathless at rest with a respiratory rate of 32 beats/min. His blood pressure was 170/110 mmHg. He had bilateral pitting edema of the legs and sinus tachycardia. Auscultation of the lungs revealed pulmonary congestion.

Laboratory data confirmed advanced renal failure. His laboratory tests revealed serum creatinine of 9 mg/dL, blood urea nitrogen of 75 mg/dL, hemoglobin of 13.3 g/dL, white blood cell count of 5,830/mm 3 , platelet count of 99,000/mm 3 , serum sodium of 133 mEq/L, potassium of 4.2 mEq/L, chloride of 102 mEq/ L and bicarbonate of 12 mEq/L. Urine analysis revealed specific gravity of 1.020, albumin 2+, RBC 40-50/hpf, pus cells 80-100/hpf and spot urine for protein-creatinine (PCR) ratio was 1.6. The patient was negative for malarial para­site, dengue and Leptospira. The serum was negative for HIV 1 and 2, hepatitis B surface antigen and hepatitis C virus antibodies. Com­plement 3 and 4 levels were within normal limits. Vasculitis work-up was negative for ANA, ds DNA, p and c-ANCA, anti-GBM an­tibody and ASO titer. Ultrasonography of the abdomen revealed chronic liver disease, bila­teral bulky kidneys with normal echotexture and cortical reflectivity and minimal ascites. The blood and urine cultures were negative.

In view of oliguria, fluid overload, azotemia and metabolic acidosis, he was initiated on he­modialysis (HD). Kidney biopsy was planned in view of the acute onset of unexplained renal failure. He underwent kidney biopsy after four sessions of HD. On light microscopy, there were eight enlarged glomeruli showing thin basement membranes and patent capillary lumina; three glomeruli showed proliferation of mesangial cells (3-5 per region) with neu­trophilic infiltration and sparse endothelial pro­liferation. The tubules revealed foci of necrosis and atrophy. The interstitium was stippled with lymphocytes, eosinophils and plasma cells. The blood vessels showed luminal narrowing due to myointimal thickening, suggestive of focal proliferative glomerulonephritis. Immunofluo­rescence study revealed prominent deposits of C1q along with C3 in the mesangium and along the capillary loops [Figure 1]. Electron microscopy showed numerous mesangial, sub­endothelial and rare intra-membranous electron dense deposits, confirming the diagnosis of C1q nephropathy [Figure 2].
Figure 1: Immunoflurescence with prominent deposits of C1q along with C3 in the mesangium and along the capillary loops.

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Figure 2: E.M. showing numerous mesangial, subendothelial and rare intra-membranous electron dense deposits of C1q nephropathy.

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The patient was initiated on intravenous me­thylprednisolone 1 g/day for three days, follo­wing which his serum creatinine dropped sig­nificantly from 6.2 to 3.9 mg/dL. He was taken off HD, and he was put on oral prednisolone 1 mg/kg/day (60 mg/day). His renal functions were monitored on a daily basis. The serum creatinine gradually decreased from 3.9 to 1.9 mg/dL within a span of 10 days. He was con­tinued on oral prednisolone in a dose of 60 mg/day for one month and gradually tapered to the current levels of 30 mg/day. The renal functions returned to normal (creatinine 1.1 mg/dL) with no significant proteinuria (spot urine PCR-0.2).


   Discussion Top


Jennette and Hipp proposed to define the cli­nical entity of C1q nephropathy by the follo­wing criteria: (a) the presence of predominant or co-dominant deposition of C1q in the me­sangium on immunofluorescence, (b) corres­ponding mesangial or para-mesangial electron­dense deposits under electron microscopy, and (c) lack of clinical and pathological evidence of systemic lupus erythematosus (SLE). [1] Ge­nerally, C1q deposition is caused by the acti­vation of C1 by immunoglobulin G (IgG) and IgM; therefore, C1q nephropathy is considered as an immune complex glomerulonephritis. However, in C1q nephropathy, it remains un­clear whether the deposition of C1q in the glo­meruli is in response to the deposition of im­munoglobulin or immune complex, or whether deposition is non-specific trapping that accom­panies increased glomerular protein trafficking associated with proteinuria. Since both the pa­thogenesis of C1q deposition in the glomeruli as well as its significance are still uncertain, it has not yet been established as an independent disease.

A variety of histological findings have been reported on light microscopy. They include minor glomerular abnormalities, mesangial pro­liferative glomerulonephritis with or without segmental sclerosis, and focal segmental glo­merulosclerosis (FSGS). [2] There are few case reports wherein the patients had presented with rapidly progressive glomerulonephritis. [3]

Clinical outcomes of C1q nephropathy are diverse. Patients with the nephrotic syndrome and C1q nephropathy often show a poor res­ponse to corticosteroid treatment. Methylpred­nisolone pulse therapy has been shown to be effective in steroid resistant cases. Aggressive treatment with a combination of cyclosporine, prednisolone and methylprednisolone pulse the­rapy could be a treatment of choice for C1q nephropathy with FSGS. Sequential therapy with cyclophosphamide, azathioprine, mycophe­nolate mofetil and tacrolimus used separately or in combination with steroids has shown good clinical response in different studies. [4]

Our patient presented with acute renal failure requiring HD. Such a presentation has not yet been reported in patients with C1q nephro­pathy. The fact that the response to steroids is immediate and complete prompts us to be­lieve that this is an immune mediated problem causing a proliferative disease.


   Acknowledgment Top


The authors would like to acknowledge Dr. Shaila Khubchandani, Histopathologist, Jaslok Hospital, Mumbai, India, for her contribution in preparing this case report.

 
   References Top

1.Jennette JC, Hipp CG. C1q nephropathy: A distinct pathologic entity usually causing neph­rotic syndrome. Am J Kidney Dis 1985;6:103-10.  Back to cited text no. 1
[PUBMED]    
2.Alenka V, Dusan F. Pathology, clinical presen­tations, and outcomes of C1q nephropathy. J Am Soc Nephrol 2008;19(11):2237-44.  Back to cited text no. 2
    
3.Srivastava T, Chadha V. C1q nephropathy pre­senting as rapidly progressive crescentic glo­merulonephritis. Clin Exp Nephrol 2009;13(4): 263-74.  Back to cited text no. 3
    
4.Satoshi H, Yuko F. Clinicopathologic corre­lation and outcome of C1q nephropathy. Clin J Am Soc Nephrol 2008;3:1637-43.  Back to cited text no. 4
    

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Correspondence Address:
Pavan Malleshappa
Senior Registrar, Department of Nephrology, A-791, Lilavati Hospital and Research Centre, Bandra Reclamation, Bandra-West, Mumbai400050, Maharashtra State
India
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PMID: 21422635

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    Abstract
    Introduction
    Case Report
    Discussion
    Acknowledgment
    References
    Article Figures
 

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