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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 1  |  Page : 126-129
Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma

1 Department of Nephrology, Mediciti Hospitals, Aurangabad, Maharashtra; Department of Nephrology, Manik Hospital and Research Centre, Aurangabad, India
2 Department of Histopathology, Apollo Health City, Hyderabad, Andhra Pradesh, India
3 Department of Nephrology, Mediciti Hospitals, Aurangabad, Maharashtra, India

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Date of Web Publication30-Dec-2010


Deposit glomerulopathies are characterized by fibrillary deposits of various sizes, mainly in the mesangial area. Collagenofibrotic glomerulopathy is a rare type of such fibrillary glomerulopathies characterized by deposits of 60-80 nm fibrils in the sub-endothelial and mesan­gial areas. It is also associated with increased levels of serum pro-collagen type III peptide (PIIINP). Although most of the initial reports have emanated from Japan, many other scientists around the globe have later reported this disease. Possibility of systemic disease affecting metabolism of type III collagen is postulated but so far no such association has been identified. We report a 26-year­old male patient who presented with insidious onset of febrile illness associated with lympha­denopathy and proteinuria. Lymph node biopsy revealed features of Hodgkin's lymphoma while percutaneous renal biopsy showed features of collagenofibrotic glomerulopathy.

How to cite this article:
Soni SS, Gowrishankar S, Nagarik AP, Barnela SR, Padua MD, Adikey GK, Raman A. Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma. Saudi J Kidney Dis Transpl 2011;22:126-9

How to cite this URL:
Soni SS, Gowrishankar S, Nagarik AP, Barnela SR, Padua MD, Adikey GK, Raman A. Collagenofibrotic glomerulopathy in association with hodgkin's lymphoma. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2021 Jul 25];22:126-9. Available from: https://www.sjkdt.org/text.asp?2011/22/1/126/74385

   Introduction Top

Collagenofibrotic glomerulopathy is an unco­mmonly described deposit glomerulopathy cha­racterized by deposition of type III collagen fibrils within the mesangial matrix and sub­endothelial space and increase in serum pro­collagen type III peptide (PIIINP) levels. This rare glomerulopathy was first discussed in 1979 by Masaaki Arakawa at the meeting of the Japanese Society of Nephrology. [1] Subsequently, Arakawa and Yamanaka published a collection of ten cases from Japan. [2] We report a case of collagenofibrotic glomerulopathy in association with Hodgkin's lymphoma.

   Case Report Top

A 26-year-old male patient presented with history of intermittent fever of six weeks dura­tion, abdominal distension, nausea, anorexia and easy fatigability since one month and swelling over feet since two weeks. Clinical examina­tion revealed marked pallor, bilateral pedal edema and normal blood pressure (120/70 mm Hg in sitting position). He also had multiple small posterior cervical lymph nodes (LNs) and a large right axillary LN, moderate splenome­galy and ascitis.

Laboratory investigations revealed severe anemia (hematocrit 24.6 vol%), leukocytosis (13400/mm 3 ), normal platelet count (3.81 lakhs/ mm 3 ), erythrocyte sedimentation rate of 124 mm at first hour, severe hypoalbuminemia (1.8 g/dL) and normal serum creatinine (1.3 mg/dL). Urine examination revealed 3+ dipstick protei­nuria and bland urine sediment and 24-hour proteinuria was 1.6 g. Computed tomography (CT) scan of the abdomen revealed generalized intra-abdominal lymphadenopathy, renomegaly and splenomegaly with infiltration. His collagen profile and serological tests for Hepatitis B, Hepatitis C, Ebstein Barr virus and HIV were negative.

He underwent excision biopsy of the right axi­llary LN and percutaneous renal biopsy. His­topathology and immunohistochemistry of LN [Figure 1] confirmed it to be a case of mixed cellularity variant of Hodgkin's lymphoma.
Figure 1: Lymph node biopsy (high power view) (a) showing classical Reid Sternberg cell occupying center of the image; (b) immunohistochemistry showing positivity for CD30.

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Light microscopy of renal tissue [Figure 2] showed mild basement membrane thickening with mesangial widening. There was no evi­dence of tubular atrophy or interstitial fibrosis. Immunofluorescence study was negative for IgG, IgM, IgD, C3, kappa and lambda light chains. Electron microscopy showed flattening of foot processes, mild thickening of basement membrane in some capillaries and fibrillary deposits of 60-80 nm in size in mesangial area forming a fascicle [Figure 2]. Serum PIIINP levels could not be estimated due to lack of testing facility. Thus, a diagnosis of collageno­fibrotic glomerulopathy in association with Hodgkin's lymphoma was made. This is the first case of this association being reported in literature.
Figure 2: Renal biopsy: (a) Light microscopy (high power) showing mild thickening of the basement membrane with mesangial widening; (b) electron microscopic image (×80,000 magnification) showing deposit in the mesangium forming a fascicle.

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   Discussion Top

Collagenofibrotic glomerulopathy is a disor­der of unknown etiology characterized by accu­mulation of atypical type III collagen fibrils within the mesangial matrix and sub-endothe­lial space and increased serum PIIINP levels. [3] Type III collagen is a structural protein of the extracellular matrix. In the human kidney, it is present only in the interstitium and blood ve­ssels and not in the glomerulus.

The disease is rare, with around 40 published cases in the English literature. [4] Most of the ini­tial reports were from Japan. However, Dombros from Canada, [5] Imbasciati from Italy, [6] Berna­sovska from Slovakia [7] and Reis from Brazil [8] have reported isolated cases of this disease. In 1993, Gubler et al, reported 10 French children of this disorder, who had evidence for familial renal disease with an autosomal recessive trans­mission. [9]

The etiopathogenesis of this glomerulopathy is not well established. Clustering of the cases from Japan points to environmental or racial factors. Occurrence of the disease in siblings points to genetic etiology. [9],[1] Increased circu­lating levels of PIIINP and an autopsy report of collagen III deposition in all organs in a known case of collagenofibrotic glomerulopathy [11] sug­gest the possibility of systemic disorder with abnormal metabolism of type III collagen.

However, no disorder has been identified so far. Collageno-fibrotic glomerulopathy is being reported in association with a systemic disease (Hodgkin's lymphoma) for the first time. Many other glomerular diseases are reported in asso­ciation with Hodgkin's lymphoma, namely, minimal change, membranous, membranopro­liferative and crescentric glomerulonephritis as well as amyloidosis. [12] Mixed cellularity Hodg­kin's disease is the histologic variety most fre­quently associated with the nephrotic syn­drome, [13] as in our case.

Histologically, collagenofibrotic glomerulopa­thy presents as deposit glomerulopathy with eosinophilic sub-endothelial and mesangial de­posits. The deposits are periodic acid-Schiff (PAS) and Congo red stain negative but positive for aniline blue and acid fuschin orange. [14] Immunofluorescence study for immunoglobu­lins, complement components and light chains is negative and characteristically positive for type III collagen. Positive immunostaining for type V collagen is also reported. [15] Electron microscopy is imperative to establish a defi­nitive diagnosis which shows marked accumu­lation of fibrillar material in the mesangium and sub-endothelial space of size around 60 nm, as in our case. The fibers form irregularly arranged bundles and sometimes impart flower like appearance on cross section. [4]

The clinical presentation includes edema and/ or persistent proteinuria (nephrotic range in up to 60%), hypertension (in two-third of the ca­ses) and anemia. Renal function test results usually are normal or slightly increased at pre­sentation. [4] The case described also presented with edema and proteinuria leading to severe hypoalbuminemia and normal renal function.

No specific therapy is available. Steroid the­rapy is reported to be effective in temporarily treating the renal dysfunction, anemia and pro­teinuria. [16] The natural history is variable, but the disease is progressive, with progression to end-stage renal disease in 5-12 years. [17] Suc­cessful kidney transplantation has been repor­ted with no recurrence at three years. [17] It will be interesting to see whether successful treat­ment of the systemic disease (Hodgkin's lym­phoma) leads to resolution of renal disease in this case.

   Acknowledgments Top

Dr. Arun Chitale from Jaslok Hospital, Mumbai, India, for electron microscopic and Dr. Sonali Saboo and Dr. M.Ch. Balaji Reddy from Focus Diagnostic Centre, Hyderabad, India, for radiologic assistance are acknowledged.

   References Top

1.Arakawa M, Hueki H, Sato M, Yamashita K, Nakashima S. Idiopathic mesangiodegenerative glomerulonephropathy: A proposal of a new glomerular disease. Jpn J Nephrol 1979;21:1404.  Back to cited text no. 1
2.Ikeda K, Yokoyama H, Tomosugi N, Kida H, Ooshima A, Kobayashi K. Primary glomerular fibrosis: A new nephropathy caused by diffuse intra-glomerular increase in atypical type III collagen fibers. Clin Nephrol 1990;33(4):155-9.  Back to cited text no. 2
3.Abt AB, Cohen AH. Newer glomerular di­seases. Semin Nephrol 1996;16:501-10.  Back to cited text no. 3
4.Alchi B, Nishi S, Narita I, Gejyo F. Collageno­fibrotic glomerulopathy: clinicopathologic over­view of a rare glomerular disease. Am J Kidney Dis 2007;49(4):499-506.  Back to cited text no. 4
5.Dombros N, Katz A. Nail-patella like renal lesion in the absence of skeletal abnormalities. Am J Kidney Dis 1982;1(4):237-40.  Back to cited text no. 5
6.Imbasciati E, Gheradi G, Morozumi K, et al. Collagen Type III glomerulopathy: A new idiopathic glomerular disease. Am J Nephrol 1991;11(5):422-9.  Back to cited text no. 6
7.Bernasovska G, Demes M, Oksa A, et al. Collagenofibrotic glomerulopathy-rare glome­rulonephritis. Vnitr Lek 2006;52(12):1200-4.  Back to cited text no. 7
8.Bichuette-Custodio F1, Castro E1, Teixeira V, Reis MA. Collagenofibrotic glomerulopathy: A description of two cases. Abstract book ofWorld Congress of Nephrology. 2007. p. 327.  Back to cited text no. 8
9.Gubler MC, Dommergues JP, Foulard M, et al. Collagen type III glomerulopathy: A new type of hereditary nephropathy. Pediatr Nephrol. 1993;7(4):354-60.  Back to cited text no. 9
10.Tamura H, Matsuda A, Kidoguchi N, Matsu­mura O, Mitarai T, Isoda K. A family with two sisters with collagenofibrotic glomerulonephro­pathy. Am J Kidney Dis 1996;27(4):588-595.  Back to cited text no. 10
11.Yasuda T, Imai H, Nakamoto Y, et al. Colla­genofibrotic glomerulopathy: A systemic di­sease. Am J Kidney Dis 1999;33(1):123-7.  Back to cited text no. 11
12.Davison AM, Hartley B. Malignancy associated glomerular disease. In: Davison AM, Cameronn JS, Grunfeld JP, et al, (eds). Textbook of Cli­nical Nephrology. 3rd ed. Oxford. Oxford Uni­versity Press, 2005,634.  Back to cited text no. 12
13.Moorthy AV, Zimmennan SW, Burkholder PM. Nephrotic syndrome in Hodgkin's disease: Evi­dence for pathogenesis alternative to immune complex deposition. Am J Med 1976;61(4): 471-7.  Back to cited text no. 13
14.Kern WF, Silva FG, Laszik ZG, Bane BL, Nadasdy T, Pitha JV. Miscellaneous conditions affecting the kidney. In: Kern WF, Silva FG, Laszik ZG, Bane BL, Nadasdy T, Pitha JV, (eds). Atlas of Renal Pathology. 1 st ed. Phila­delphia, PA. Saunders, 1999, 194-207.  Back to cited text no. 14
15.Morita H, Hasegawa T, Minamoto T. Colla­genofibrotic glomerulopathy with a widespread expression of type-V collagen. Virchows Arch 2003;442(2):163-8.  Back to cited text no. 15
16.Hisakawa N, Yasuoka N, Nishiya K. Collageno­fibrotic glomerulonephropathy associated with immune complex deposits. Am J Nephrol 1998;18(2):134-41.  Back to cited text no. 16
17.Suzuki T, Okubo Y, Ikezumi Y. A favorable course of collagenofibrotic glomerulopathy after kidney transplantation and questionnaire sur­vey about the prognosis of collagenofibrotic glomerulopathy. Jpn J Nephrol 2004;46(4): 360-4.  Back to cited text no. 17

Correspondence Address:
Anuradha Raman
Director of Nephrology, Mediciti Hospitals, 5-9-22, Secretariat Road, Hyderabad 500063, Andhra Pradesh
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PMID: 21196628

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