Table 1 (abstract P323). See text for description
From: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Mean Age at Onset | 8.5 years (2-17years) |
Sex Ratio M:F | 1:1 |
Eye Involvement | 3 / 4 (75%) |
Recurrent scleritis & lacrimal gland involvement | 1 |
B/l orbital inflammation (MRI evidence) | 2 |
Tumour-like mass – mediastinal mass | 1 / 4 (25%) |
Renal (tubulointerstitial nephritis) | 1 / 4 ( 25%) |
Respiratory System ( Chronic Cough) | 1 / 4 (25%) |
Elevated IgG4 level > 200 mg / dl | 4 / 4 (100%) |
Supportive Histopathological evidence | 3 / 4 (75%) |
Response to steroids | 4 /4 (100%) |
Flare on stopping steroids | 4 / 4 (100%) |
Response to Methotrexate | 0 / 2 (0%) |
Response to MMF | 2 / 3 (75%) |
Response to Azathioprine | 1 / 2 (50%) |