Pulmonary manifestations of IgG4‑related disease in a South African patient.

Autor:	Cilliers JD; Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa., Eindhoven SC; Department of Respiratory Medicine, Franciscus Gasthuis and Vlietland, Rotterdam, The Netherlands., Louw E; Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa., Koegelenberg CFN; Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa., Irusen E; Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa., Bruce-Brand C; Division of Anatomical Pathology, Department of Pathology, Faculty of Medicine and Health Sciences, Stellenbosch University, Cape Town, South Africa.; National Health Laboratory Service, Tygerberg Hospital, Cape Town, South Africa., Allwood BW; Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa.
Jazyk:	angličtina
Zdroj:	African journal of thoracic and critical care medicine [Afr J Thorac Crit Care Med] 2021 Mar 09; Vol. 27 (1). Date of Electronic Publication: 2021 Mar 09 (Print Publication: 2021).
DOI:	10.7196/AJTCCM.2021.v27i1.130
Abstrakt:	Immunoglobin 4-related disease (IgG4-RD) is an auto-immune, multisystem inflammatory disorder characterised by storiform fibrosis, lymphoplasmacytic infiltration and obliterative phlebitis on histology. Its pathophysiology is not well understood, but is thought to occur due to complex interactions between T helper 2 cells, their cytokines, chemokines, and B lymphocytes that become dysregulated and produce dysfunctional immunoglobulins. Here, we present a case report of a 54-year-old man who was initially suspected of having lung cancer on imaging, but was ultimately diagnosed with IgG4-RD on histological analysis of a pneumonectomy specimen. Treatment with glucocorticoids can establish disease remission, with a small proportion of patients relapsing, if the diagnosis is made before significant fibrosis occurs. Competing Interests: Conflicts of interest: None.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu