Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy

Matthai, Smita Mary; Mohapatra, Anjali; Duhli, Neelaveni; David, Vinoi George; Varughese, Santosh

doi:10.4103/ijpm.ijpm_341_18

Cited by 2 publications

(2 citation statements)

References 9 publications

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“…NPS is an autosomal dominant disorder in which mesangial and mottled glomerular basement membrane (GBM) deposits are noted, described as moth-eaten lamina densa. CG deposits are found in mesangial and subendothelial spaces, but not in the lamina densa [ 14 ]. This atypical glomerular deposition of collagen type III can occur in proliferative, non-proliferative, immune, and non-immune glomerular diseases [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

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Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Aloudah,

Moalwi,

Alnofal

2024

Am J Case Rep

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Patient: Male, 35-year-old Final Diagnosis: Glomerulonephritis Symptoms: Arthralgia • body aches • hematuria • swelling Clinical Procedure: Kidney biopsy • ultrasonography Specialty: Nephrology • Pathology Objective: Rare disease Background: Collagen type III glomerulopathy (CG) is a rare disease with poorly understood pathogenesis, usually identified by abnormal collagen type III accumulation in glomeruli and manifesting as progressive deterioration of kidney function with nephrotic-range proteinuria. Immunoglobulin A nephropathy (IgAN) is the most prevalent glomerulopathy worldwide and is a leading cause of end-stage renal disease as a result of progressive fibrotic changes. Fibrosis is primarily caused by collagen type III deposition, which may explain the simultaneous occurrence of IgAN and CG. Case Report: A young man presented with clinical and laboratory evidence of chronic kidney injury, including long-term nephrotic-range proteinuria and microscopic hematuria. Partial improvement in proteinuria was achieved with steroid therapy and conservative management. As the non-invasive workup was inconclusive, and a complete recovery of kidney function was not achieved, a kidney biopsy was done. Histopathological microscopic examination revealed advanced IgA nephropathy, Oxford classification M0E1S1T2C0, with features highly suggestive of type III collagen glomerulopathy. Conclusions: We described a case of collagen type III glomerulopathy, also known as collagenofibrotic glomerulopathy, and its association with concurrent immunoglobulin A nephropathy in a healthy man presenting with chronic proteinuria and microscopic hematuria. As the number of reported cases in the Middle East is rising, we present this report to improve understanding and greater recognition of such cases.

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Section: Discussionmentioning

confidence: 99%

“…Also, podocyte effacement is usually present. Additionally, a crucial difference detectable in EM distinguishing NPS from CG is the lamina densa of the GBM, which is spared from collagen depositions in CG but not in NPS [ 1 , 2 , 14 ].…”

Section: Discussionmentioning

confidence: 99%

Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Aloudah,

Moalwi,

Alnofal

2024

Am J Case Rep

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Telmisartan alleviates collagen type III glomerulopathy: A case report with literature review

et al. 2020

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Collagen type III is commonly detected in the renal interstitium and vasculature; however, it is absent in healthy glomeruli. Deposition of collagen type III in the glomerular mesangium and capillary basement membranes may arise in two rare diseases, namely collagen type III glomerulopathy (CG) and nail patella syndrome. CG is a rare glomerular disease with no specific treatment, although supportive measures for control of hypertension and edema may help to relieve symptoms. With progression to end-stage renal disease, patients with CG may come to require dialysis and/or renal transplantation. The present study reported on a 59-year-old male who was diagnosed with CG nephrotic syndrome by immunohistochemical and electron microscopic examination of biopsy material. To the best of our knowledge, this is the first case reported in northeastern China. The angiotensin II blocker telmisartan was successfully used to alleviate renal symptoms and a literature review was performed. The present case supports the use of telmisartan as a first choice of treatment for CG.

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Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy

Cited by 2 publications

References 9 publications

Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Telmisartan alleviates collagen type III glomerulopathy: A case report with literature review

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Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy

Cited by 2 publications

References 9 publications

Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Simultaneous Occurrence of Collagen Type III Glomerulopathy and Immunoglobulin A Nephropathy: A Rare Case Report

Telmisartan alleviates collagen type&nbsp;III glomerulopathy: A case report with literature review

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Telmisartan alleviates collagen type III glomerulopathy: A case report with literature review