Experience from the first UK inter-regional specialist multidisciplinary meeting in the diagnosis and management of IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is a complex multisystem fibro-inflammatory disorder, requiring diagnostic differentiation from malignancy and other immune-mediated conditions, and careful management to minimise glucocorticoid-induced toxicity and prevent progressive organ dysfunction. W...

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Published inClinical medicine (London, England) Vol. 20; no. 3; pp. e32 - e39
Main Authors Goodchild, George, Peters, Rory JR, Cargill, Tamsin N, Martin, Harry, Fadipe, Adetokunbo, Leandro, Maria, Bailey, Adam, Collier, Jane, Firmin, Louisa, Chouhan, Manil, Rodriguez-Justo, Manuel, Sadler, Ross, Chapman, Roger W, Bungay, Helen, Fryer, Eve, David, Joel, Luqmani, Raashid, Barnes, Eleanor, Webster, George J, Culver, Emma L
Format Journal Article
LanguageEnglish
Published England Elsevier Ltd 01.05.2020
Royal College of Physicians
Subjects
Cancer
Disease
Histology
Hospitals
Humans
IgG4-related disease
Immunoglobulin G
Immunoglobulin G4-Related Disease
Immunoglobulins
inflammation
London
Medical referrals
Monoclonal antibodies
multidisciplinary team
Original Research
Physicians
Rheumatology
rituximab
Specialization
Targeted cancer therapy
United Kingdom
London
United Kingdom
United Kingdom > UK
IgG4-related disease
multidisciplinary team
inflammation
rituximab
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Summary:Immunoglobulin G4-related disease (IgG4-RD) is a complex multisystem fibro-inflammatory disorder, requiring diagnostic differentiation from malignancy and other immune-mediated conditions, and careful management to minimise glucocorticoid-induced toxicity and prevent progressive organ dysfunction. We describe the experience of the first inter-regional specialist IgG4-RD multidisciplinary team meeting (MDM) incorporating a broad range of generalists and specialists, held 6-weekly via web-link between Oxford University Hospitals NHS Foundation Trust and University College London Hospitals NHS Foundation Trust. Over 3 years, there were 206 discussions on 156 patients. Of these, 97 (62%) were considered to have definite or possible IgG4-RD; 67% had multi-organ involvement and 23% had a normal serum IgG4. The average number of specialist opinions sought prior to MDM was four per patient. Management was changed in the majority of patients (74%) with the treatment escalation recommended in 61 cases, including 19 for rituximab. Challenges arose from delays and misdiagnosis, cross-specialty presentation and the management of sub-clinical disease. Our cross-discipline IgG4-RD MDM enabled important diagnostic and management decisions in this complex multisystem disorder, and can be used as a model for other centres in the UK
ISSN:1470-2118
1473-4893
DOI:10.7861/clinmed.2019-0457