IgG4-Related Disease Manifesting as Hypocomplementemic Tubulointerstitial Nephritis: A Rare Case Report and Literature Review

Abstract

Immunoglobulin G4–related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. The different phenotypes of IgG4-RD can lead to delayed or incorrect diagnosis. We report the case of a 66-year-old male with coal worker’s pneumoconiosis who presented with progressive kidney disease and was diagnosed with tubulointerstitial nephritis due to IgG4-RD. The patient was noted to have progressive kidney disease, skin involvement, worsening interstitial lung disease, complete vision loss in the left eye, and retroperitoneal fibrosis. Serologic workup revealed elevated inflammatory markers, IgG4 and IgG1 levels, and hypocomplementemia. A tissue biopsy helped us establish a definitive diagnosis of IgG4-RD and initiate treatment with glucocorticoids to prevent further progression of kidney disease and other end-organ damage.

A fatal multiple-lesion, inflammatory, pseudotumor in the lung: a rare case report

“…We are of the view that the patient’s final poor prognosis was related to the lack of effective systemic treatment. For example, glucocorticoid therapy can help relieve symptoms of inflammatory pseudotumor according to published literature reports, and in some cases, the lesions may disappear ( 7 - 9 ). However, in this case, hormone therapy only slightly relieved the symptoms, and lesion size and anemia did not significantly improve.…”

Übersetzung: Spektrum der IgG4-assoziierten Erkrankungen: Eine Literaturübersicht

“…Es können jedoch auch Beteiligungen an anderen Stellen, wie in TABELLE 1 aufgeführt, festgestellt werden, da IgG4-RD praktisch jedes Organ betreffen kann und Erkrankungen, die früher als Teil anderer Krankheiten angesehen wurden, heute dem IgG4-RD-Spektrum zugeordnet werden (Al-Mujaini et al, 2018;Obiorah;Velasquez;Özdemirli, 2018). (Obiorah;Velasquez;Özdemirli, 2018;Au et al, 2020).…”

A rare case: IgG4‐related chronic inflammatory disease with kidney involvement

“…Tissue biopsy is important to support the diagnosis. 1,5 While the disease is first described as associated with autoimmune pancreatitis, it can affect organs such as the thyroid, salivary gland, biliary tract, kidney etc. 6,7 The kidney is involved in approximately 20% of patients with IgG4-RD.…”

“…IgG4‐RD can lead to fibrosis and can cause to organ failure. Tissue biopsy is important to support the diagnosis 1,5 . While the disease is first described as associated with autoimmune pancreatitis, it can affect organs such as the thyroid, salivary gland, biliary tract, kidney etc 6,7 …”