IgG4-related disease: Evidence from six recent cohorts

Purpose To better characterize IgG4‐related disease (RD) in the setting of idiopathic orbital inflammation syndrome (IOIS). Methods National, multicentre, prospective, observational cohort study. Among the patients consecutively included in the French multicentre SIOI cohort, we selected those who underwent orbital and/or adnexal biopsy. Clinical, morphological and pathological findings at diagnosis were blindly analysed. Serum IgG4 levels at inclusion were measured and all available biopsy specimens were immunostained for IgG4 and IgG. Biopsy samples with more than 10 IgG4‐positive plasma cells per high‐power field and a IgG4+/IgG+ plasma cell ratio above 40% were scored as positive. IgG4‐positive patients were then screened for comprehensive diagnostic criteria for IgG4‐RD. Results Of the 87 patients included, 35 had histologically documented IOIS. Thirteen patients (37%) with a mean age at onset of 27 years (range 21–78) had IgG4‐positive biopsies, among which 10 patients (77%) and 3 (23%, with IgG4 serum levels >1.35 g/L) were considered as having probable and definite IgG4‐RD, respectively. The latter 13 patients more frequently fulfilled histological criteria for IgG4‐RD (including plasmacytic infiltrate (p = 0.006), fibrosis (p = 0.0025) and periphlebitis (p = 0.075)) than IgG4‐negative patients. Storiform fibrosis was exclusively found in orbital tissues from IgG4‐positive patients (n = 3, 23%). Eosinophilia associated with recurrent sinusitis or asthma was a prominent feature in patients with definite IgG4‐RD. Conclusions More than one‐third of patients with biopsy‐proven IOIS satisfied criteria for IgG4‐RD, but only a few had a definite type.

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“…; Martínez‐Valle et al. ). Most of these patients originated from Asia or North America and had primarily orbital disease (Masaki et al.…”

Section: Discussionmentioning

confidence: 99%

IgG4‐related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort

Abad

Martin

Héran³

et al. 2018

Acta Ophthalmologica

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“…After combination of our patients with all other published cases of IgG4-RD from Hong Kong, we analysed 104 cases comprising predominantly older men (mean age, 62 ± 13 years; male-to-female ratio=3:1), which is consistent with other reports. [19][20][21][22] Over 95% of patients had serum IgG4 level of >135 mg/dL and an IgG4:IgG ratio of >8%. Although these cut-offs are often quoted in the diagnostic criteria for IgG4-RD, 4,23 it is important to note that elevated serum IgG4 levels can be seen in a variety of other conditions such as malignancies, infections, or autoimmune disorders.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

et al. 2017

Hong Kong Med J

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“…Uchida et al [66] estimated the annual incidence and prevalence of IgG4-RD in Japan to be 0.28-1.08/100,000 and 6/100,000, respectively. IgG4-RD affects middle- to old-age (median age: 50.3-67) subjects, with a male/female ratio ranging from 1.6:1 to 4:1 [63,67,68]. IgG4-RD immunopathogenesis remains incompletely elucidated.…”

Section: Igg4-related Respiratory Diseasementioning

confidence: 99%

“…Since the initial report of a patient with sclerosing cholangitis associated with an elevated serum IgG4 concentration [62], IgG4-related disease (IgG4-RD) has been defined as a multiorgan chronic fibroinflammatory condition characterized by lymphoplasmacytic infiltrates with abundant IgG4 plasma cells, associated with storiform fibrosis and often high serum IgG4 concentrations [63,64]. Fibroinflammatory lesions may virtually affect every organ [63].…”

Section: Igg4-related Respiratory Diseasementioning

confidence: 99%

See 1 more Smart Citation

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

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In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

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IgG4-related disease: Evidence from six recent cohorts

Cited by 53 publications

References 25 publications

IgG4‐related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort

IgG4‐related disease in patients with idiopathic orbital inflammation syndrome: data from the French SIOI prospective cohort

Immunoglobulin G4–related disease in Hong Kong: clinical features, treatment practices, and its association with multisystem disease

The Lung in Dysregulated States of Humoral Immunity

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