Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion

Collagenofibrotic glomerulopathy, a very rare glomerular disease, characterised by abnormal deposition of Type III collagen in the mesangium and subendothelial space. This disease may have an indolent course clinically but is capable of finally progressing to end stage renal failure. Thus, it is imperative for clinicians and pathologists to be mindful of this entity due to its rarity, nonspecific presenting signs and symptoms and varied differentials on histopathology which must be ruled out. A definite diagnosis depends on the awareness of this entity, a high index of suspicion and confirmation by immunohistochemistry and electron microscopy. Here, the present authors report a case of a 41-year-old hypertensive female who came with persistent pedal oedema and proteinuria. The biopsy showed nodular expansion of the mesangium by a homogenous, Congo red negative, eosinophilic material. A diagnosis of Collagenofibrotic glomerulopathy was confirmed on immunohistochemistry and electron microscopy.