ESPN 50th Annual Meeting

ESPN 2017


 
IGA NEPHROPATHY AND IgG4 RELATED ORBİTAL DİSEASE: A CHİLD CASE
KADRIYE ÖZDEMIR 1 CEMALIYE BASARAN 1 ÖZLEM BARUT SELVER 3 MELIS PALAMAR 3 NECIL KÜTÜKÇÜLER 2 AYSE YAGCI 3 ERKIN SERDAROGLU 1

1- DR BEHCET UZ CHILDRENS HOSPITAL, PEDIATRIC NEPHROLOGY
2- EGE UNIVERSITY, SCHOOL OF MEDICINE, DEPARTMENT OF IMMUNOLOGY AND ROMATOLOGY
3- EGE UNIVERSITY FACULTY OF MEDICINE, DEPARTMENT OF OPHTHALMOLOGY
 
Introduction:

IgG4-related disease (IgG4-RD) is recognized systemic autoimmune disorder characterized by high levels of serum IgG4 and infiltration of IgG4-positive plasma cells in multiple organs. Co-existence between IgG4-RD and IgA nephropathy (IgAN) is very rare.Orbital involvement in IgG4-RD includes lacrimal glands, extra-ocular muscles, trigeminal nerve and other parts of the orbit.Herein we presenta child with IgAN and IgG4-RD with orbital findings.

Material and methods:

CASE REPORT

A 10 year old female was admitted with fever, macroscopic hematuria (4 weeks),proteinuria (15 mg/m2/hr)and decreasing GFR (59 ml/sc/1.73m2).NormalC3,C4,ANA,Anti-ds-DNAand elevated serum IgA (284 mg/dl) were found in laboratory.Renal biopsy was done for prolonged hematuria and showed positive segmental granular IgA+, IgM++ staining and minimal mesenchymal cell proliferation,fibro-necrotizing and segmental sclerosing (5/25 glomerulus) and focal chronic tubulointerstitial inflammation.Improvement in renal function, macroscopic hematuria and proteinuria were observed in 2 weeks.The case was diagnosed as IgAN and ramipril was started.Two years later,patient admitted again with swelling redness, ptosis on left eye lid.MRI revealed a supero temporal orbital mass (29x11x31mm) extending to lacrimal gland.Incisional biopsy of the mass revealed lympho-plasmocytic infiltration and fibrolipomatosis.With the existing findings IgG4-RD was suspected and elevated serum IgG (1910 mg/dl), IgG4 (137mg dl),acute phase reactants supported this diagnosis.Proteinuria (4.5 mg/mg creatinine)was also present. 


Results:

 Systemic steroid (40 mg/day) and mycophenolate mofetil (500 mg/day) were started.The orbital mass regressed rapidly,elevated acute phase reactants returned to normal,but proteinuria resisted. 

Conclusions:

 IgG4-RD is rare in adults,and very rare in childhood.To the best of our knowledge this is the first report on coexisting IgAN and IgG4-RD in childhood.