|Year : 2018 | Volume
| Issue : 3 | Page : 113-115
Collapsing glomerulopathy with IgA nephropathy
Arcot Thanjan Maasila, Jeyachandran Dhanapriya, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, V Murugesan, T Balasubramaniyan, Natarajan Gopalakrishnan
Institute of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||17-Dec-2018|
Institute of Nephrology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Primary IgA nephropathy (IgAN) is the most common primary glomerulonephritis globally. Its epidemiology, clinical presentation, histology, and prognosis vary widely. Focal segmental glomerulosclerosis is frequently seen in IgAN and is an indicator of poor prognosis. Collapsing glomerulopathy (CG) associated with IgAN is rarely seen, and its treatment and prognosis are largely unknown. We present here, two male patients presented with renal failure and nephrotic syndrome. Renal biopsy in the first patient revealed CG with IgAN with significant interstitial fibrosis and tubular atrophy and hence treated with supportive therapy. In the second patient, concomitant CG and IgAN were diagnosed and started on oral steroids and had partial improvement.
Keywords: Collapsing glomerulopathy, IgA nephropathy, renal biopsy, steroids
|How to cite this article:|
Maasila AT, Dhanapriya J, Dineshkumar T, Sakthirajan R, Murugesan V, Balasubramaniyan T, Gopalakrishnan N. Collapsing glomerulopathy with IgA nephropathy. J Integr Nephrol Androl 2018;5:113-5
|How to cite this URL:|
Maasila AT, Dhanapriya J, Dineshkumar T, Sakthirajan R, Murugesan V, Balasubramaniyan T, Gopalakrishnan N. Collapsing glomerulopathy with IgA nephropathy. J Integr Nephrol Androl [serial online] 2018 [cited 2020 Feb 26];5:113-5. Available from: http://www.journal-ina.com/text.asp?2018/5/3/113/247696
| Introduction|| |
The most common pathological finding in primary IgA nephropathy (IgAN) is the mesangial proliferative glomerulonephritis, but the spectrum is varied. Focal segmental glomerulosclerosis (FSGS) is a histological pattern resulting from a variety of mechanisms, due to damage to and/or loss of podocytes. The presence of FSGS in IgAN was studied previously, and association of collapsing glomerulopathy (CG) with IgAN is not widely known. We report such concomitant lesions in two male patients.
| Case Reports|| |
A 42-year-old male presented with bilateral leg swelling and oliguria of 1-week duration. His blood pressure (BP) was 100/80 mmHg and pulse rate (PR) was 72/min. Investigations revealed urine proteinuria: 3+; urine protein creatinine ratio (PCR): 6.1; blood hemoglobin: 11.8 g/dL; total count: 7900/mm3; platelet count: 170,000/mm3; blood urea: 66 mg/dL; and serum creatinine 2.9 mg/dL. Ultrasonogram of abdomen revealed normal-sized kidneys. Viral markers (HIV/HBSAg/anti-hepatitis B virus) were negative. Renal biopsy revealed 4 of 11 was globally sclerotic, collapse of glomerular capillary tuft with podocyte hyperplasia [Figure 1] and hyaline globules in one glomerulus, remaining glomeruli showed increase in mesangial matrix and cellularity, interstitial fibrosis, and tubular atrophy involving about 50% of the core. Immunofluorescence (IF) showed IgA (+3) and C3 (+2) positivity over the mesangium [Figure 2]. He was started on angiotensin-converting enzyme inhibitors (ACEIs) and atorvastatin (10 mg/day). At the last follow-up at 10 months, his urine PCR was 0.23 and serum creatinine was 3.9 mg/dL.
|Figure 1: Renal biopsy showing partial collapse of capillary tuft with hyperplasia of overlying podocytes (PAS stain)|
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A 19-year-old-male presented with edema legs and facial puffiness on off for 2 years. His BP was 130/80 mmHg and PR was 80/min. Investigations revealed urine PCR: 7.1 and serum creatinine: 2.9 mg/dL. Renal biopsy showed mesangial hypercellularity and matrix expansion and one glomerulus showing collapse of the capillary tuft with hyperplasia of the overlying podocytes without chronic changes. IF showed evidence of IgA (+3) positive over the mesangium. He was initiated on oral prednisolone (60 mg/day) for 2 months and tapered over the next 4 months along with ACEIs. On the last follow-up at 8 months, his urine PCR was 2 and serum creatinine was 2.9 mg/dL.
| Discussion|| |
IgAN may present with variable histologic patterns. Haas et al. in 1999 studied 18 of 244 patients (7.4%) showing FSGS along with IgAN. The mean age was 34.8 years, and 72% were males. The mean serum creatinine was 1.6 mg/dL, and urine protein was 5.5 g/day presentation. At mean follow-up of 70 months, only one who progressed to end-stage renal disease.
Hill et al. noted that FSGS was found in 78.9% of biopsies. The study cohort of the Oxford classification showed FSGS in 76%. The possible pathomechanisms of segmental sclerosis in patients with IgAN were (1) post-inflammatory scarring of segmental proliferative or necrotizing lesions, (2) compensatory hemodynamic changes following nephron loss, and (3) primary podocyte damage due to mediators released from mesangial cells. Fogo et al. showed that the prognosis is different in 50% of those who had ended up with dialysis, compared with 4.1% of those without segmental sclerosis.
In a study by El Karoui et al. which included 128 patients IgAN, 101 had FSGS of which 11 had CG. Of 11 cases, 8 had associated extracapillary proliferation. All showed advanced parenchymal damage, with numerous vacuolated cells in the tubular lumens. All presented with advanced renal insufficiency and heavy proteinuria. Of 10 patients with follow-up, 9 became dialysis dependent at 22 months.
Another study which included 114 patients, 35 had coexistent IgAN and FSGS. About 50% had not-otherwise specified, 30% had tip and 20% perihilar variant of FSGS. None had collapsing or cellular variants.
Both patients presented to us with renal failure and nephrotic proteinuria and were males. The first patient was treated with only supportive therapy as he had significant chronic changes in biopsy and the second patient with oral steroid showed partial improvement though long term follow-up is needed to conclude on the use of steroids in coexistent CG and IgAN.
It is well-known fact that the presence of FSGS-like lesions in IgAN is associated with the poor outcome, but its association with CG makes the prognosis grave almost with 100% dialysis dependency.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to thank Dr. Anila Abraham Kurien renopath, Chennai, for her help in evaluating renal biopsy.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
D'Amico G. Natural history of idiopathic IgA nephropathy and factors predictive of disease outcome. Semin Nephrol 2004;24:179-96.
D'Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: A working proposal. Am J Kidney Dis 2004;43:368-82.
Hill GS, Nochy D, El Karoui K. Comments on the oxford classification of IgA nephropathy. Kidney Int 2009;76:1207.
Haas M. IgA nephropathy histologically resembling focal-segmental glomerulosclerosis: A clinicopathologic study of 18 cases. Am J Kidney Dis 1996;28:365-71.
Hill GS, Karoui KE, Karras A, Mandet C, Van Huyen JD, Nochy D, et al.
Focal segmental glomerulosclerosis plays a major role in the progression of IgA nephropathy. I. Immunohistochemical studies. Kidney Int 2011;79:635-42.
Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, et al.
The oxford classification of IgA nephropathy: Rationale, clinicopathological correlations, and classification. Kidney Int 2009;76:534-45.
Mubarak M, Nasri H. Significance of segmental glomerulosclerosis in IgA nephropathy: What is the evidence? J Renal Inj Prev 2013;2:113-5.
Fogo AB, Alpers CE, D'Agati VD. FSGS lesions in IgA nephropathy. Kidney Int 2011;80:319.
El Karoui K, Hill GS, Karras A, Moulonguet L, Caudwell V, Loupy A, et al.
Focal segmental glomerulosclerosis plays a major role in the progression of IgA nephropathy. II. Light microscopic and clinical studies. Kidney Int 2011;79:643-54.
Nasri H, Mubarak M. Focal segmental glomerulosclerosis in IgA nephropathy with regard to Oxford classification: Does it matter? Port J Nephrol Hypert 2014;28:210-8.
Cook HT. Focal segmental glomerulosclerosis in IgA nephropathy: A result of primary podocyte injury? Kidney Int 2011;79:581-3.
[Figure 1], [Figure 2]