IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients

Campochiaro, Corrado; Ramirez, Giuseppe A.; Bozzolo, E.; Lanzillotta, Marco; Berti, Alvise; Baldissera, Elena; Dagna, Lorenzo; Praderio, Luisa; Scotti, Raffaella; Tresoldi, Moreno; Roveri, Luisa; Mariani, Alberto; Balzano, Gianpaolo; Castoldi, R.; Doglioni, Claudio; Sabbadini, M G; Della‐Torre, Emanuel

doi:10.3109/03009742.2015.1055796

Cited by 125 publications

(150 citation statements)

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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%

“…A summary of the demographics, clinical features, and treatment modalities in comparison to other cohorts is shown in Table 1. [8][9][10][11][12][13][14][15][16][17][18][19][20][21] The most common organ systems involved were HBP system (40.4%), salivary gland (33.7%), lymph node (29.8%), and eye (19.2%). A Venn diagram of these most common involved systems from the combined cohort is shown in the Figure. …”

Section: Demographics Clinical Features and Treatment Modalitiesmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Demographics Clinical Features and Treatment Modalitiesmentioning

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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“…In all studies, neurological disease was relatively rare, although cases of pituitary, meningeal and peripheral nervous system involvement were reported. In two smaller European studies, one from Spain and the other one from Italy, pachymeningitis was the only reported neurological manifestation—2/55 (4%) and 3/41 (7%), respectively [36, 37]. …”

Section: Epidemiologymentioning

confidence: 99%

Neurological Manifestations of IgG4-Related Disease

Baptista

Casian

Gunawardena

et al. 2017

Curr Treat Options Neurol

106

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Opinion statementIgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised—hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required. Wherever possible, a tissue diagnosis should be established. The characteristic histopathological changes include a lymphoplasmacytoid infiltrate, storiform fibrosis and obliterative phlebitis. IgG4-RD typically responds well to treatment with glucocorticoids, although relapse is relatively common and treatment with a steroid-sparing agent or rituximab may be required. Improved understanding of the pathogenesis of IgG4-RD is likely to lead to the development of more specific disease treatments in the future.

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“…Although the biopsy rate is relatively low, it is similar to other cohorts; for example, in an Italy cohort, 15/41 cases (36.6%) were diagnosed as possible IgG4-RD. 26 In conclusion, glucocorticoid is an effective drug in remission induction of IgG4-RD. Our results showed that there were no statistically significant differences in the efficacy and safety between high and medium doses of glucocorticoid in the remission treatment of IgG4-RD patients.…”

Section: -13mentioning

confidence: 89%

Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4‐related diseases: a preliminary randomized controlled trial

Chang

Chen

et al. 2017

Int J of Rheum Dis

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Aim: In order to evaluate the efficacy and safety of high versus medium doses of glucocorticoid therapy in remission induction of immunoglobulin G4-related disease (IgG4-RD), we set up a randomized controlled study.Method: Newly diagnosed IgG4-RD patients were randomly assigned to two groups: high doses of prednisone (0.8-1.0 mg/kg/day) and medium doses (0.5-0.6 mg/kg/day). Patients were assessed at weeks 0, 4, 12 and 24. The primary outcome was the remission rate at week 24. The secondary endpoints included IgG4-RD responder index (IgG4-RD RI), IgG and IgG4 levels.Results: Twenty cases in each group finished the study. At week 24, the total response rates were 95% and 80% in high and medium dose groups, respectively. There was no significant difference between the two groups. IgG4-RD RI reduced from 14.9 to 3.0 in the high dose group, while in the medium dose group it was from 13.1 to 3.2. At week 24, the average level of IgG4 reduced from 1576 to 324 mg/dL and from 1445 to 684 mg/dL in the two groups, respectively. Relapsed patients had higher baseline IgG4-RD RI. There was no severe adverse effect shown in both groups. Conclusion:The effect of remission induction was similar in high and medium glucocorticoid groups. However, patients with more organs involved and higher IgG4-RD RI score at baseline might get more benefit with high dose glucocorticoid for remission induction.

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IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients

Abstract: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

Cited by 125 publications

References 40 publications

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

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

Neurological Manifestations of IgG4-Related Disease

Efficacy between high and medium doses of glucocorticoid therapy in remission induction of IgG4‐related diseases: a preliminary randomized controlled trial

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