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 H+ | El (603Â mg/dl) | Pred 20Â mg/d | Initially suspected of malignancy. Pred tapered and stopped in 4Â months. |
Ibrahim 2010 [25] | 3y | F | IgG4-R cholangitis H+ | El (258Â mg/dl) | Pred 2Â mg/kg/d and Aza 1.5Â mg/kg | Relapse after tapering pred and required a low (2Â mg/d) maintenance dose of pred and Aza. |
Mannion 2010 [14] | 13y | F | AIP-1 and IgG4-R fibrosing mediastinitis, renal and hepatic manifestation H+ | El (73.4Â 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 H+ | NM | Pred | Â |
Griepentrog 2013 [7] | 10y | F | IgG4-ROD H+ | N (L U) | Lateral orbitotomy | No further treatment was required. |
Griepentrog 2013 [7] | 14y | F | IgG4-ROD H+ | N (L U) | Pred, dosage unknown, and MMF | MMF because of relapse after tapering pred, successful. |
Kalapesi 2013 [10] | 5y | F | IgG4-ROD H+ | El (1.52Â g/l) | Pred 1Â mg/kg and MMF (600Â mg/m2) | Weaned off pred and maintained on MMF successfully. |
Naghibi 2013 [15] | 16y | F | IgG4-related colitis, in the past AIP-1 H+ | El (210Â mg/dl) | Adalimumab | Refractory disease to pred 0.5Â mg/kg, Aza and infliximab. Adalimumab successful. |
Pifferi 2013 [26] | 15y | M | IgG4-R pulmonary disease H+ | El (1090Â mg/dl) | Pred 0.6Â mg/kg/d | Treatment for 4Â weeks. |
Sane 2013 [11] | 12y | F | IgG4-ROD and nephrotic syndrome H+ | N (L U) | Methylpred and rituximab | The nephrotic syndrome also resolved. Initial good response to pred 40Â mg, but relapse occured. |
Pasic 2013 [12] | 10y | F | Mikulicz disease/IgG-ROD H+ | EL 9.02Â g/l | NM | Â |
Caso 2013 [16] | 17y | M | IgG4-R lymphad and scleritis H+ | El (4.43Â g/l) | Rituximab and pred 10Â mg daily | Refractory to MMF, good results with rituximab. |
Hasosah 2014 [17] | 7y | F | IgG4-R mesenteritis and pericarditis H+ | El (149Â mg/dl) | Pred, aza and colchicine (doses unknown) | Relapsed despite aza, further treatment with 5Â mg prednisone as maintenance therapy. |
Jariwala 2014 [5] | 7y | M | IgG4-ROD H+ | El (109.3Â mg/dl) | Pred 1Â mg/kg/d and Aza 2Â mg/kg/d | Good clinical results. |
Mittal 2014 [6] | 14y | M | IgG4-ROD H+ | El (4.3Â g/l) | Pred 0.6Â mg/kg/d | Initial improvement, but lost to follow-up. |
Notz 2014 [29] | 13y | F | IgG4-R dacryoadenitis H+ | N (23.9Â mg/dl) | Pred 40Â mg/d for 3Â months | Â |
Prabhu 2015 [8] | 15y | F | IgG4-ROD and sinonasal disease H+ | El (579Â mg/dl) | Rituximab | Insufficient response to prednisone. |
Prabhu 2015 [8] | 15 y | F | IgG4-R sinonasal disease H+ | El (206Â mg/dl) | Pred (dosage unknown) | Â |
Batu 2015 [9] | 14y | F | IgG4-ROD H+ | N (7.5Â g/l) (0-12.5Â g/l) | Pred (dosage unknown) | Pred was tapered and stopped, MTX as maintenance therapy. |
Batu 2015 [9] | 9y | F | IgG4-ROD H+ | N (3.7Â 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 H+ | El (805Â mg/dl) | Pred 2Â mg/kg/d | Pred tapered over period of 6Â months. |
Gillispie 2015 [13] | 7y | F | IgG4-ROD, nerve and renal disease H+ | N (L U) | Pred and rituximab | Refractory to pred, responsive to rituximab. |
Nada 2015 [19] | 10y | M | IgG4-R hepatic mass and coagulopathy H+ | El (420Â mg/dl) | Pred 2Â mg/kg/d | Coagulopathy also resolved after treatment. |
Rosen 2015 [27] | 17y | M | IgG4-R cholangitis H+ | El (242Â mg/dl) | Pred 30Â mg/d | Pred weaned in 3Â months. |