Collagenofibrotic Glomerulopathy with Thrombotic Microangiopathy: A Rare Case Report
ED07-ED09
Correspondence
Dr. Khushbu Agarwal,
D-104 Vishwanath Sopan Shela, Near Club 07, Ahmedabad, Gujarat, India.
E-mail: khushbuagarwal82@gmail.com
Collagenofibrotic Glomerulopathy (CG) is a very rare glomerular disease characterised by the massive accumulation of atypical type III collagen fibrils within the mesangial matrix and subendothelial space, as well as a marked increase in serum type III procollagen peptide levels. Owing to its rarity, the cause and pathogenesis of CG remain uncertain. While initial reports of CG were primarily in patients of Asian descent, subsequent cases in patients of European descent have also been published. Clinically, patients present with oedema and hypertension, often progressing to end-stage renal disease. Proteinuria is a cardinal manifestation of this disease. A definitive diagnosis is contingent upon recognising this entity, maintaining a high index of suspicion, and confirming it through immunohistochemistry and Electron Microscopy (EM), due to its rarity, non specific presenting signs and symptoms, and various differential diagnoses on histology. Here, a case of a 44-year-old non diabetic, hypertensive female who presented with pedal oedema and nephrotic range proteinuria. The biopsy showed nodular expansion of the mesangium by homogeneous, congo red-negative, eosinophilic material, along with vascular changes indicative of acute thrombotic microangiopathy. Many conditions, such as amyloidosis, fibrillary glomerulonephritis, diabetic nephropathy, fibronectin glomerulopathy, light chain deposition disease, and thrombotic microangiopathy, can cause nodular or membranoproliferative glomerular changes. The biopsy was initially reported as nodular glomerulosclerosis, which, upon EM, showed mesangial and subendothelial space expansion by banded fibrillary material. Further immunohistochemistry for type III collagen was performed, which yielded positive results. Thus, the diagnosis of CG was confirmed.