ESPN 53rd Annual Meeting

ESPN 2021


 
A Rare Cause of Non-Lupus Full-House Nephropathy In An Adolescent Boy: C3 Glomerulopathy
GÖKÇEN ERFIDAN 1 ÖZGÜR ÖZDEMIR ŞIMŞEK 1 CEMALIYE BAŞARAN 1 SEÇIL ARSLANSOYU ÇAMLAR 2 DEMET ALAYGUT 1 FATMA MUTLUBAŞ 2 BELDE KASAP DEMIR 3

1- UNIVERSITY OF HEALTH SCIENCES, TEPECIK TRAINING AND RESEARCH HOSPITAL, DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY, IZMIR, TURKEY
2- UNIVERSITY OF HEALTH SCIENCES, IZMIR FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY, IZMIR, TURKEY
3- İZMIR KATIP CELEBI UNIVERSITY, FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY AND RHEUMATOLOGY, IZMIR, TURKEY
 
Introduction:

“Full-house” pattern is a histological definition to describe glomerular immune complex deposits of IgG, IgA, IgM, C3 and C1q on Immunofluorescent (IF) microscopy. It is well known as a characteristic feature of lupus nephritis. However, full-house nephropathy in a patient without any extrarenal signs and symptoms of systemic lupus erythematosus (SLE) and/or presence of autoantibodies may become a diagnostic challange. 

Material and methods:

A sixteen-year-old boy, admitted with periorbital edema, which was intermittently prominent in last two months. He had no history of hematuria, hypertension, infection, drug use or family history of kidney disease. On the physical examination, he had normal development with a weight of 73 kg (50-75th percentile), height of 181 cm (75-90th percentile) and blood pressure of 110/75 mmHg. He had normal urine output and no edema. The laboratory examination showed microscopic hematuria, nephrotic range proteinuria (95 mg/m2/h), low serum albumin (2.1 g/dL) with elevated lipid levels. He had low C3 level of 0.14 g/L (0.9-1.8 g/L) and normal C4 level of 0.27 g/L (0.1-0.4 g/L). The Anti-Streptolysin-O, Anti-Nuclear Antibody, Anti-double stranded DNA, Anti-Neutrophil Cytoplasmic Antibody, Anti-Phospholipase A2 Receptor Antibody were all negative. Due to low C3 level and the presentation age, a renal biopsy was performed.

Results:

 The light microscopy showed diffuse thickening of glomerular basement membrane, mild mesangial hypercellularity, endocapillary proliferation and segmental sclerosis. Immunofluorescent microscopy revealed “full-house” pattern with C3 prominancy. Electron microscopic examination revealed basement membrane thickening with irregular borders, granular electron dense deposits in subepithelial, intramembranous, subendothelial areas and mesengium with matrix expansion. No cytoplasmic tubuloreticular inclusions were identified.

Conclusions:

In this case, the histopathological findings were suggestive for lupus nephritis with full-house pattern on IF. Nevertheless, he had only microscopic hematuria and nephrotic proteinuria with no other manifestations of SLE. Considering light-IF-electron microscopic examination, this case is diagnosed with C3-glomerulopathy.