ESPN 53rd Annual Meeting

ESPN 2021


 
IgG4-related disease with isolated renal involvement in childhood
ÖZGÜR ÖZDEMİR ŞİMŞEK 1 GÖKÇEN ERFİDAN 1 NESLİHAN GÜNEY 2 SEÇİL ARSLANSOYU ÇAMLAR 3 DEMET ALAYGUT 1 FATMA MUTLUBAŞ 3 BELDE KASAP DEMİR 4

1- UNIVERSITY OF HEALTHY SCIENCES TEPECIK TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY, IZMIR, TURKEY
2- UNIVERSITY OF HEALTHY SCIENCES TEPECIK TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF PATHOLOGY,IZMIR, TURKEY
3- UNIVERSITY OF HEALTH SCIENCES, IZMIR FACULTY OF MEDICINE DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY, IZMIR, TURKEY
4- IZMIR KATIP ÇELEBI UNIVERSITY MEDICAL FACULTY, DEPARTMENT OF PEDIATRICS, DIVISION OF NEPHROLOGY AND RHEUMOTOLOGY, IZMIR, TURKEY
 
Introduction:

 

IgG4-related disease is a fibroinflammatory disorder characterized by IgG4-positive plasma cell infiltration and fibrosis. We present a case of isolated IgG4-related kidney disease (RKD) with tubulointerstitial nephritis (TIN) and several glomerular lesions.

Material and methods:

 A 6-year-old boy was admitted to the emergency room with nausea, vomiting and fatigue that started 5 days ago. He had a body weight of 21.4kg (25–50p), a height of 118.5cm (25-50p), blood pressure of 158/100mmHg (>95p+12), and (+1) pretibial pitting edema. Laboratory tests revealed urea 198mg/dL(N:10-38); serum creatinine 6.9mg/dL(N:0.5-1.2); uric acid 6.4mg/dL(N:2-5.5); albumin 2.3g/dL(N:3.5-5.5); sodium 130mmol/L(N:134-150), potassium 4.91mmol/L(N:3.5-5.5), calcium 7.7mg/dL(N:8.8-10.8), phosphorus 6.6mg/dL(N:4-7), erythrocyte sedimentation rate(ESR) 108mm/h(N:0-20); parathormone 248ng/L(N:18.5-88); total IgE 24600IU/mL(N:0-90), total IgG:8.44g/dL(N:7-16), IgG4:2.91g/dL(N:0.012-1.699), pH 7.13, HCO3 11.6mmol/L, and BE-16.7mmol/L. His urine output was 0.3 cc/kg/h. Urinalysis revealed specific gravity 1018; glucose+2; protein+3; erythrocyte+2. Spot urine protein/creatinine was 24.4. Serum C3-C4 were normal; ANA and anti-dsDNA were negative. The kidney biopsy was consistent with chronic TIN. There was global sclerosis in 3, cellular/fibrocellular crescents in 4, ischemic collapse in 10-11 of the 43 glomeruli. IgG (+2) and IgM (+1) staining were observed in immunofluorescence microscopy. There were ≥10 IgG4 stained cells/hpf. The patient was diagnosed with IgG4-RKD and USG, CT, MRI and PET-CT were performed to detect other organ involvements. There were no signs of malignancy or extrarenal symptoms. Hemodialysis was initiated. 

Results:

 

He received 6 doses of pulse methylprednisolone followed by 2 doses of intravenous cyclophosphamide 15 days apart and Rituximab. Serum creatinine decreased to 1.7 mg/dL and he had no need for dialysis anymore. The treatment was supposed to be personalized due to laboratory tests in the follow-up.

Conclusions:

 

IgG4-RKD is should be kept in mind in patients with high IgE levels and chronic TIN even in childhood. Our case is the smallest patient with IgG4-RKD and the first child with isolated IgG4-RKD.