TY - JOUR
T1 - In search of a disease
AU - Savino, Gustavo
AU - Campana, Md, Andrea
AU - Petrone, Gianluigi
AU - Grimaldi, Gabriela
AU - Murchison, Ann P.
PY - 2016
Y1 - 2016
N2 - A 10-year-old child presented with right eyelid swelling and a palpable orbital mass. Diagnostic evaluation with the aid of excisional biopsy revealed a pseudoencapsulated mass associated with high levels of serum perinuclear antineutrophil cytoplasmic antibodies. Family history of rheumatic diseases, response to treatment with corticosteroids and immunosuppressant drugs, and clinical and histopathologic features suggested an orbital autoimmune response in which perinuclear antineutrophil cytoplasmic antibodies were detected, and a diagnosis of probable IgG4-related disease was made.
AB - A 10-year-old child presented with right eyelid swelling and a palpable orbital mass. Diagnostic evaluation with the aid of excisional biopsy revealed a pseudoencapsulated mass associated with high levels of serum perinuclear antineutrophil cytoplasmic antibodies. Family history of rheumatic diseases, response to treatment with corticosteroids and immunosuppressant drugs, and clinical and histopathologic features suggested an orbital autoimmune response in which perinuclear antineutrophil cytoplasmic antibodies were detected, and a diagnosis of probable IgG4-related disease was made.
KW - Intravenous steroids
KW - Ophthalmology
KW - Orbital IgG4-related disease
KW - Pediatric orbital inflammatory disease
KW - Perinuclear antineutrophil cytoplasmic antibodies (p-ANCA)
KW - Subcutaneous methotrexate
KW - Intravenous steroids
KW - Ophthalmology
KW - Orbital IgG4-related disease
KW - Pediatric orbital inflammatory disease
KW - Perinuclear antineutrophil cytoplasmic antibodies (p-ANCA)
KW - Subcutaneous methotrexate
UR - http://hdl.handle.net/10807/103162
UR - http://www.sciencedirect.com/science/journal/00396257
U2 - 10.1016/j.survophthal.2016.12.006
DO - 10.1016/j.survophthal.2016.12.006
M3 - Article
SN - 0039-6257
SP - N/A-N/A
JO - Survey of Ophthalmology
JF - Survey of Ophthalmology
ER -