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Table 1 Outcomes reported in case reports on IgG4-RD in pediatrics

From: IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

Reference Age Sex Organ manifestation Serum IgG4 Therapy Comments
Miglani 2010 [24] 13y M AIP-1
El (603 mg/dl) Pred 20 mg/d Initially suspected of malignancy. Pred tapered and stopped in 4 months.
Ibrahim 2010 [25] 3y F IgG4-R cholangitis
El (258 mg/dl) Pred 2 mg/kg/d and Aza 1.5 mg/kg Relapse after tapering pred and required a low (2 mg/d) maintenance dose of pred and Aza.
Mannion 2010 [14] 13y F AIP-1 and IgG4-R fibrosing mediastinitis, renal and hepatic manifestation
El (73.4 mg/dl) Pred and MMF Good results by MMF, pred tapered and stopped successfully.
Zakeri 2011 [20] 17y M Riedel’s thyroiditis
H + a
NM Pred 40 mg/d Pred tapered and stopped in 3 months.
Melo 2012 [28] 11y M IgG4-R sialadenitis
NM Pred  
Griepentrog 2013 [7] 10y F IgG4-ROD
N (L U) Lateral orbitotomy No further treatment was required.
Griepentrog 2013 [7] 14y F IgG4-ROD
N (L U) Pred, dosage unknown, and MMF MMF because of relapse after tapering pred, successful.
Kalapesi 2013 [10] 5y F IgG4-ROD
El (1.52 g/l) Pred 1 mg/kg and MMF (600 mg/m2) Weaned off pred and maintained on MMF successfully.
Naghibi 2013 [15] 16y F IgG4-related colitis, in the past AIP-1
El (210 mg/dl) Adalimumab Refractory disease to pred 0.5 mg/kg, Aza and infliximab. Adalimumab successful.
Pifferi 2013 [26] 15y M IgG4-R pulmonary disease
El (1090 mg/dl) Pred 0.6 mg/kg/d Treatment for 4 weeks.
Sane 2013 [11] 12y F IgG4-ROD and nephrotic syndrome
N (L U) Methylpred and rituximab The nephrotic syndrome also resolved. Initial good response to pred 40 mg, but relapse occured.
Pasic 2013 [12] 10y F Mikulicz disease/IgG-ROD
EL 9.02 g/l NM  
Caso 2013 [16] 17y M IgG4-R lymphad and scleritis
El (4.43 g/l) Rituximab and pred 10 mg daily Refractory to MMF, good results with rituximab.
Hasosah 2014 [17] 7y F IgG4-R mesenteritis and pericarditis
El (149 mg/dl) Pred, aza and colchicine (doses unknown) Relapsed despite aza, further treatment with 5 mg prednisone as maintenance therapy.
Jariwala 2014 [5] 7y M IgG4-ROD
El (109.3 mg/dl) Pred 1 mg/kg/d and Aza 2 mg/kg/d Good clinical results.
Mittal 2014 [6] 14y M IgG4-ROD
El (4.3 g/l) Pred 0.6 mg/kg/d Initial improvement, but lost to follow-up.
Notz 2014 [29] 13y F IgG4-R dacryoadenitis
N (23.9 mg/dl) Pred 40 mg/d for 3 months  
Prabhu 2015 [8] 15y F IgG4-ROD and sinonasal disease
El (579 mg/dl) Rituximab Insufficient response to prednisone.
Prabhu 2015 [8] 15 y F IgG4-R sinonasal disease
El (206 mg/dl) Pred (dosage unknown)  
Batu 2015 [9] 14y F IgG4-ROD
N (7.5 g/l)
(0-12.5 g/l)
Pred (dosage unknown) Pred was tapered and stopped, MTX as maintenance therapy.
Batu 2015 [9] 9y F IgG4-ROD
N (3.7 g/l) Methylpred and cyclophosphamide No response to pred, MTX or MMF. Now stable disease.
Corujeira 2015 [18] 22Mo F IgG4-R pulmonary disease and IgG4-R lymphad
El (805 mg/dl) Pred 2 mg/kg/d Pred tapered over period of 6 months.
Gillispie 2015 [13] 7y F IgG4-ROD, nerve and renal disease
N (L U) Pred and rituximab Refractory to pred, responsive to rituximab.
Nada 2015 [19] 10y M IgG4-R hepatic mass and coagulopathy
El (420 mg/dl) Pred 2 mg/kg/d Coagulopathy also resolved after treatment.
Rosen 2015 [27] 17y M IgG4-R cholangitis
El (242 mg/dl) Pred 30 mg/d Pred weaned in 3 months.
  1. Y year, IgG4-ROD IgG4-related orbital disease, Mo months, H+ histology performed, Mikulicz disease IgG4-related orbital and submandibular disease, M male, AIP-1 autoimmune pancreatitis type 1, IgG-R IgG4-related disease, F female, Pred prednisone, Aza azathioprine, EL elevated, MMF mycophenolate mofetil, L U level unknown, N normal, NM not measured, Methylpred Methylprednisolone, Lymphad Lymphadenopathy
  2. aHistology without IgG4 staining