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

2019 ◽  
Vol 10 (12) ◽  
pp. 348-353
Author(s):  
Manaf Aljishi ◽  
Zaw Thet ◽  
Thin Han ◽  
Krishan Madhan
2019 ◽  
Vol 9 (2) ◽  
pp. 85-91
Author(s):  
Magdalini Velegraki ◽  
Kostas G. Stylianou ◽  
Dimitrios Xydakis ◽  
Hariklia Gakiopoulou ◽  
Evangelos Voudoukis ◽  
...  

Membranous nephropathy (MN) with tubulointerstitial nephritis (TIN) is an established manifestation of immunoglobulin G4 (IgG4)-related disease (IgG4-RD). A pathological feature aiming to distinguish between primary and secondary MN is the presence or absence of glomerular staining for phospholipase A2 receptor (PLA2R), respectively. Isolated MN without TIN has been rarely reported in the context of IgG4-RD. This case report describes a patient with a history of MN successfully treated with steroids and cyclophosphamide, who, 3 years later, presented with unexplained exacerbation of diabetes mellitus due to IgG4-related autoimmune pancreatitis. Pancreatitis was treated, and diabetes improved after treatment with steroids. Based on the presence of isolated IgG4 glomerular capillary deposits along with negative staining for PLA2R and the metachronous appearance of autoimmune pancreatitis, MN was retrospectively classified as secondary to IgG4-RD. Isolated IgG4-positive/PLA2R-negative MN without TIN can be a prodrome of IgG4-RD, reminiscent of MN secondary to neoplasms.


2021 ◽  
Vol 15 (2) ◽  
pp. 44-51
Author(s):  
Saika Farook ◽  
Abdullah Ahmed Solaiman ◽  
Md Shariful Alam Jilani

Immunoglobulin G4 related disease (IgG4-RD) is a recently perceived fibroinflammatory condition, identified as a systemic illness for the first time in the early 2000. It can involve virtually every organ of the body, commonly presenting as lymphadenopathy, retroperitoneal fibrosis, autoimmune pancreatitis, tubulointerstitial nephritis, parotid or lacrimal gland enlargement. The diagnosis is confirmed by histopathological analysis and is often, but not always accompanied by an increased level of serum IgG4 concentration. In fact, the name addressing this autoimmune fibroinflammatory condition may be considered a misnomer, as the role of the non-inflammatory immunoglobulin IgG4 in the immune mechanism of IgG4-RD remains to be elucidated. Ibrahim Med. Coll. J. 2021; 15(2): 44-51


2020 ◽  
Vol 8 ◽  
pp. 232470962095221
Author(s):  
Pradnya Brijmohan Bhattad ◽  
David L. Joseph ◽  
Eric Peterson

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.


2019 ◽  
Vol 7 (16) ◽  
pp. 2309-2315
Author(s):  
Eray Eroglu ◽  
Murat Hayri Sipahioglu ◽  
Soner Senel ◽  
Sule Ketenci Ertas ◽  
Seyma Savas ◽  
...  

2016 ◽  
Vol 38 (3) ◽  
Author(s):  
Rafael Coimbra Ferreira Beltrame ◽  
Maurício Friderichs ◽  
Bárbara Rayanne Fior ◽  
Pedro Guilherme Schaefer ◽  
Gustavo Gomes Thomé ◽  
...  

Author(s):  
Satoshi Kurahashi ◽  
Naohiro Toda ◽  
Masaaki Fujita ◽  
Katsuya Tanigaki ◽  
Jun Takeoka ◽  
...  

2020 ◽  
Vol 103 (11) ◽  
pp. 1230-1235

Immunoglobulin G4-related disease (IgG4-RD) has recently been recognized as an autoimmune disorder involving multiple organs. The kidney is a represented organ with a wide range of renal manifestations. The authors report a case of an 83-year-old Thai male with combined IgG4 tubulointerstitial nephritis and membranous nephropathy coexisting with cholangiocarcinoma. The patient presented with proteinuria, acute renal failure, eosinophilia, hypocomplementemia, and high serum IgG4 concentration. The diagnosis was IgG 4-related tubulointerstitial nephritis and membranous nephropathy on renal biopsy, with negative immunohistochemistry for anti-phospholipase A2 receptor antibodies. Magnetic resonance imaging (MRI) abdomen showed two wedge shaped arterial enhancing lesions of liver. Liver biopsy revealed adenocarcinoma, compatible with cholangiocarcinoma. Proteinuria and renal failure were resolved with initial steroid treatment. Meanwhile, IgG4-related membranous nephropathy should be considered in the differential diagnosis for patients with proteinuria. Potentially, IgG4-RD may be rarely associated with carcinoma development. However, further studies are recommended to ratify and confirm the association between IgG4-RD and incidence of malignancies. Keywords: IgG4-related disease, Membranous nephropathy, Secondary membranous nephropathy, Tubulointerstitial nephritis, Cholangiocarcinoma


2019 ◽  
Vol 26 (5) ◽  
pp. 290-294 ◽  
Author(s):  
S. Clavé ◽  
C. Rousset-Rouvière ◽  
L. Daniel ◽  
M. Tsimaratos

2020 ◽  
Vol 45 (4) ◽  
pp. e195-e197
Author(s):  
Vankadari Kousik ◽  
Rajender Kumar ◽  
Bhagwant Rai Mittal ◽  
Divya Aggarwal ◽  
Ashutosh Nath Aggarwal

2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.


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