Accumulation of IgG4 positive plasma cells in tissues are hallmarks of the disease and CD4 + cytotoxic T lymphocytes have been shown to play a major role in formation of fibrosis. The exact pathogenesis of the IgG4-RD is unknown. She was diagnosed as a possible IgG4-RD according to diagnostic criteria and methylprednisolone and azathioprine were started.Ĭonclusion: To our knowledge, only one adult patient had ligneous conjunctivitis due to proven IgG4-RD was reported before. Auto-antibodies were negative, but IgG4 was high (>150 mg/dL). Heterozygous mutation on PRSS-1 gene was detected for hereditary pancreatitis. She experienced cholangitis, pneumobilia, hepatitis, duodenitis and interstitial lung disease.
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Besides, no differences were observed in laboratory data and clinical conditions including fever, neurological presentation, pulmonary hemorrhage, arthralgia and skin involvement (all P 3 times the upper limit of normal) were detected.
Patients with positive finding in ANCA level have tendency to hematuria ( P = 0.026) and initial presentation with anti-phospholipid ( P = 0.022). 3 patients with elevation of C-ANCA level and 6 patients had increase P-ANCA level. Median ages at diagnosis were 13.78 years and 12.67 years in the ANCA-positive and ANCA-negative group, respectively. Among these patients, 9 (13%) had ever positive in ANCA in follow-up. Results: A total of 70 children of pediatric-onset SLE patient were enrolled. Clinical characteristics, laboratory data, histological features, treatment and outcome were recorded. Method: We conducted a retrospective study of pediatric-onset SLE patients with ANCA data in a tertiary referral center over a 3-year-period (2015-2018). However, it is association with disease activity including clinical features, histological and outcomes with the presence of ANCA in pediatric-onset SLE patients is very limited. The presence of ANCA in SLE had been known for 20 years. Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies against cytoplasmic antigens within neutrophils and monocytes. Sunday, 2 June 2019 TPS 01 Clinical Immunology from Autoimmunity to Cancer TP0615 The impact of serum anti-neutrophil cytoplasmic antibody (ANCA) on clinical presentation and outcome in pediatric onset systemic lupus erythematosus (SLE) patientsĭivision of Allergy, Asthma and Rheumatology, Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan, Taiwanīackground: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by several immunological abnormalities.
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