Table 1 (abstract P162). See text for description
From: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Mean age at Onset | 5.2 years |
Sex Ratio (F:M) | 1.5:1 |
Livedo Reticularis | 5/5(100%) |
Nodular Skin lesions | 3/5(60%) |
Myalgia | 2/5 (40%) |
Hypertension | 5/5 (100%) |
Severe Abdominal Pain | 5/5(100%) |
Gastrointestinal Ulcers | 3/5(60%) |
Gastrointestinal Perforation | 3/5(60%) |
Splenic or Renal Infarcts | 3/5(60%) |
Central Nervous System Infarcts | 1/5(20%) |
Peripheral Neuropathy | 3/5 (60%) |
Angiographic Evidence of Medium Vessels Vasculitis | 5/5(100%) |
Hematological involvement , Evidence of Immunodeficiency & Type 1 Interferonopathy | 0/5 |
Average delay between diagnosis of PAN and DADA2 deficiency | 7 years |
Intravenous Cyclophospahmide | 5/5(100%) |
Oral Azathioprine | 5/5(100%) |
Intravenous Immunoglobulin | 3/5(60%) |
Subcutaneous Adalimumab | 1/5 (20%) |
Intravenous Infliximab | 1/5(20%) |
Inactive disease | 3/5(60%) |
Resistent Disease | 1/5(20%) |
Death | 1/5(20%) |