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AB0749 Immuglobuline G4 (IGG4) deposit disease and orbital pseudotumor as first manifestations of wegener’s granulomatosis (WG) in child
  1. D.S. Lazarevic,
  2. J. Vojinovic
  1. Pediatric Rheumatology, Clinic of Pediatrics, Clinical Center Nis, Nis, Serbia


Background WG is rare severe systemic disease characterized by necrotizing granulomatous vaculitis that primary affects the upper and lower respiratory tracts and kidneys. Immunoglobulin G4 (IgG4)-related disease is an evolving concept of clinicopathological entity that has not yet been fully established. The entity is characterized clinically by high levels of serum IgG4 or pathologically by predominant infiltration of plasmacytic cells with IgG4 expression. Orbital pseudo tumor, with immunoglobulin G4 (IgG4)-related diseases have been reported only in adult systemic diseases until now.

Objectives We report case of child with orbital pseudo tumor with immunoglobulin G4 (IgG4) deposit diseases as a first clinical presentation of WG.

Results At her age of 3 years girl PP started to have recurrent fever episodes. Because of recurrent, rhinitis and sore throat, tonsillectomy was done at age of 8 years. Ten days later unilateral right eyelid ptosis, swelling and redness appeared. MRI has confirmed orbital pseudo tumor and 20 mg/day prednisolone course with slow tapering was initiated with steroid dependent clinical response. One month later disease worsened with fever attacks (lasting for two months) and septic appearance, nasal obstruction with mucous and purulent secretion and recurrent nasal bleeding why triple different antibiotics and antimycotics were administrated even though serology for infections tests were negative. Only elevated ESR and CRP were persistent. Due to worsening of ocular symptoms ophthalmologists performed tumor extirpation. Biopsy has shown granulomatous lesion and vasculitic infiltration of blood vessels with the lack of eosinophilia in both blood and biopsy samples. There was also appearance of a fibrosing chronic inflammatory process rich in plasma cells which appear to be considerably restricted to expression of IgG4. Laboratory investigations have showed marginal elevation of IgG4 (1.4 g/l) and association with systemic IgG4 positive disease was considered despite the fact it was not reported in children. She was treated only with vitamin supplements. Year later, when first time referred to our unit, ocular manifestations relapsed on the same eye. Arthritis of left ankle and two granulomatous skin changes with central induration and spider nevus were present. Chest X-ray has shown bilateral hilar adenopathy (no elevation of ACE). Control MRI has revealed right orbital pseudo tumor and pan sinusitis. There was ANCA positivity (p-ANCA=7.8 U/ml), ANA and anti-ds DNA were negative, LAC (1.41) was moderately positive and ESR and CRP highly elevated. Renal exploration has shown presence of glomerulonephritis (proteinuria 1.5 g/24h, microalbuminuria and hematuria) and serum level of β2-microglobuline 2.15 mg/L. Kidney biopsy was performed and has shown the necrotizing pauci-immune glomerulonephritis so diagnose of WG was finally established. High doses of methyl-prednisolon and cyclophosphamide were commenced monthly for six months with good clinical response.

Conclusions To diagnose WG when orbital pseudo tumor is only present can be challenging. To our knowledge this first report of immunoglobulin IgG4 related disease in child with WG. This case indicates orbital involvement associated with patohistologicaly IgG4 lymphoid lesions could help early diagnosis of WG.

Disclosure of Interest None Declared

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