Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 10, Number 12, December 2019, pages 348-353

Acute Tubulointerstitial Nephritis as a Sole Manifestation of Immunoglobulin G4-Related Disease


Figure 1.
Figure 1. kidney biopsy findings. (a, b) Interstitial inflammation on hematoxylin and eosin (H&E) stain. (c) Lymphocytes, plasma cells and eosinophils. (d) Plasma (arrowhead) and Mott cells (arrow). (e) Plasma cell (arrow). (f) Marked tubular atrophy on silver stain. (g, h) Immunohistochemistry positive straining for IgG4 in plasma cells. IgG4: immunoglobulin G4.
Figure 2.
Figure 2. Creatinine trend from presentation in October 2017 to April 2019. Prednisone was commenced in November 2017 which coincided with the improvement in renal function seen then.
Figure 3.
Figure 3. IgG4 levels from presentation in October 2017 to January 2019. IgG4: immunoglobulin G4.


Table 1. Conditions Once Regarded as Individual Disorders That Merged Into IgG4-Related Disease
Autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis)
Eosinophilic angiocentric fibrosis (affecting the orbits and upper respiratory tract)
Fibrosing mediastinitis
Hypertrophic pachymeningitis
Idiopathic hypocomplementemic tubulointerstitial nephritis with extensive tubulointerstitial deposits
Inflammatory pseudotumor (affecting the orbits, lungs, kidneys, and other organs)
Kuttner’s tumor (affecting the submandibular glands)
Mikulicz’s disease (affecting the salivary and lacrimal glands)
Multifocal fibrosclerosis (commonly affecting the orbits, thyroid gland, retroperitoneum, mediastinum, and other tissues and organs)
Periaortitis and periarteritis
Inflammatory aortic aneurysm
Retroperitoneal fibrosis (Ormond’s disease)
Riedel’s thyroiditis
Sclerosing mesenteritis