First reported case of collagenofibrotic glomerulopathy with a full-house pattern of immune deposits

Collagenofibrotic glomerulopathy is a very rare glomerular disease characterized by the deposition of Type III collagen fibrils within the subendothelial and mesangial areas, and by elevated serum levels of pro-collagen Type III peptide. We reported here an elderly patient representing the first case of collagenofibrotic glomerulopathy with a "full-house" pattern of glomerular immunoglobulin and complement deposits by immunofluorescence. A 79-year-old Japanese woman was admitted to our hospital for clinical examinations of leg edema. A renal biopsy specimen showed a remarkable enlargement of the glomerular tufts due to the deposition of periodic acid-Schiff- and Masson's trichrome-positive material. All three immunoglobulins, complements, and light chains were detected in the subendothelial space and capillary walls of the glomeruli. Electron microscopy of tannic acid staining showed spiraled and frayed fibers in the subendothelial areas, which were positive for Type III collagen staining. Serum levels of pro-collagen Type III peptide were increased. Therefore, even in cases where the renal biopsy sample displays a "full-house" immunofluorescence pattern of glomerulopathy, as in systemic lupus erythematosus, we may not always rule out the diagnosis of collagenofibrotic glomerulopathy.