Case reports

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

JD Cilliers, SC Eindhoven, EH Louw, CFN Koegelenberg, E Irusen, C Bruce-Brand, BW Allwood

Abstract


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.


Authors' affiliations

JD Cilliers, Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

SC Eindhoven, Department of Respiratory Medicine, Franciscus Gasthuis and Vlietland, Rotterdam, The Netherlands

EH Louw, Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

CFN Koegelenberg, Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

E Irusen, Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

C Bruce-Brand, 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

BW Allwood, Division of Pulmonology, Department of Medicine, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

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Cite this article

African Journal of Thoracic and Critical Care Medicine 2021;27(1):29. DOI:10.7196/AJTCCM.2021.v27i1.130

Article History

Date submitted: 2021-03-09
Date published: 2021-03-09

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African Journal of Thoracic and Critical Care Medicine| Online ISSN: 2617-0205

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