IgG4-related disease: an atypical presentation of steroid-responsive renal mass.
BMJ Case Rep
; 14(5)2021 May 24.
Article
in English
| MEDLINE | ID: covidwho-1242196
ABSTRACT
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are typically elevated but half of the patients had normal serum IgG4 levels. IgG4-RD represents a spectrum of diseases that involve various organs such as the pancreas, liver, kidneys, and salivary glands often manifesting as diffuse organ enlargement or a mass-like lesion mimicking cancer. An increased incidence of malignancy among patients with IgG4-RD has been reported. Thus, differentiating malignancy from IgG4-RD manifestation is important as the treatment differs. Glucocorticoids are considered first-line therapy and should be started early to prevent fibrosis. Patients usually have an excellent clinical response to steroids, and poor steroid response is indicative of an alternative diagnoses such as malignancy. This case report describes a case of IgG4-RD with renal mass in a young man that resolved with glucocorticoid therapy alone.
Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Phlebitis
/
Autoimmune Diseases
Type of study:
Case report
/
Diagnostic study
/
Observational study
/
Prognostic study
Limits:
Humans
/
Male
Language:
English
Year:
2021
Document Type:
Article
Affiliation country:
Bcr-2020-240611
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