Unique Challenges in Diagnosing IgG4-Related Tubulointerstitial Nephritis with Arteritis

Kansas Journal of Medicine

article

Grace Falk¹  Lynn Cornell²  Mona Brake³  Ryan Ford⁴  Kaleb Todd⁴  Christopher Fox³ 

[1] University of Kansas School of Medicine - Wichita;Mayo Clinic;Robert J. Dole Veterans Affairs Medical Center;University of Kansas School of Medicine-Wichita

关键词: IgG4-related disease;    tubulointerstitial nephritis;    crescentic glomerulonephritis;    arteritis;    pseudocrescent;   

DOI  :  10.17161/kjm.vol15.18510

学科分类：医学（综合）

来源: The University of Kansas Medical Center

【 摘 要 】

Tubulointerstitial nephritis (TIN) can be part of the systemic diseaseimmunoglobulin G4 related disease (IgG4-RD).1,2 IgG4-RD can affectan isolated organ, or it can involve multiple organs, synchronously ormetachronously.3,4 IgG4-RD diagnosis is challenging due to the presentations of nonspecific findings that mimic many diseases, includingmalignancy.5Regarding IgG4-TIN, common laboratory abnormalities, outsideof elevated creatinine (Cr) and blood urea nitrogen (BUN) includedelevated serum total IgG or IgG4 (79% of patients), hypocomplementemia (56%), eosinophilia (33%), and positive ANA (31%).6 Proteinuria(30%) and hematuria (22%) occurred less often.3,6 On imaging, renalcortical nodules, round or wedge shaped lesions, or diffuse patchyinvolvement can be seen.6 Outside of the kidneys, inflammatory massescan be seen in any organ in patients with IgG4-TIN, with autoimmunepancreatitis, sclerosing cholangitis, retroperitoneal fibrosis, sialadenitis, and aortic aneurysms constituting the more commonly reportedextra-renal comorbidities.

【 授权许可】

Unknown