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2017 ; 96
(10
): e6205
Nephropedia Template TP
gab.com Text
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English Wikipedia
Ophthalmic manifestations in IgG4-related disease: Clinical presentation and
response to treatment in a French case-series
#MMPMID28272212
Ebbo M
; Patient M
; Grados A
; Groh M
; Desblaches J
; Hachulla E
; Saadoun D
; Audia S
; Rigolet A
; Terrier B
; Perlat A
; Guillaud C
; Renou F
; Bernit E
; Costedoat-Chalumeau N
; Harlé JR
; Schleinitz N
Medicine (Baltimore)
2017[Mar]; 96
(10
): e6205
PMID28272212
show ga
IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ
involvements sharing common pathological findings. Orbital or orbital adnexa
involvement of the disease has been reported in a few case series. The aim of our
study was to characterize and analyze ophthalmic manifestations from a nationwide
French case-series.Patients with IgG4-RD and orbital or orbital adnexa
involvement included in the French multicentric IgG4-RD case-registry were
identified. Only patients fulfilling "modified" comprehensive diagnostic criteria
with pathological documentation were retained for the study. Clinical,
biological, pathological, radiological findings and data regarding the response
to treatment were retrospectively analyzed.According to our data registry, the
frequency of IgG4-related ophthalmic disease (IgG4-ROD) was 17%. Mean age at
diagnosis was 55.1?±?7.1 years with a male/female ratio of 2.2. The 19 cases of
IgG4-ROD consisted of lacrimal gland (68.4%), soft tissue (57.9%), extra-ocular
muscles (36.8%), palpebral (21.1%), optical nerve (10.5%), orbital bone (10.5%),
and mononeuritis (V1?and/or V2, 10.5%) involvements. IgG4-ROD was bilateral in
57.9% of cases. Extra-ophthalmic manifestations were reported in 78.9% of cases.
All patients responded to prednisone but two-thirds of patients relapsed within a
mean (SD) of 9.8 (3.5) months and 72.2% required long-term glucocorticoids and/or
immunosuppressive agents. Eight patients were treated by rituximab with a
favorable response in 87.5% of cases.Lacrimal involvement is the most frequent
ophthalmic manifestation of IgG4-RD and is frequently associated with
extra-orbital manifestations. Despite initial favorable response to steroids, the
long-term management of relapsing patients needs to be improved.