IgG4-related disease presenting with renal injury and obstructive nephropathy discovered incidentally: a case report with literature review

IgG4-related disease presenting with renal injury and obstructive nephropathy discovered incidentally: a case report with literature review

Mouhammad J Alawad¹*, Saffa Elawad¹+, Bara Wazwaz ²**, Abdul Wahab Al Allaf ³¶

*Department of Medical Education, Internal Medicine Residency Program, Hamad Medical Corporation, Doha, Qatar
+Department of Nephrology, Hamad Medical Corporation, Doha, Qatar
** Department of Laboratory Medicine & Pathology, Hamad Medical Corporation, Doha, Qatar
¶ Department of Rheumatology, Hamad Medical Corporation, Doha, Qatar

IgG-4 related disease (IgG4-RD) is a chronic inflammatory condition characterized by tissues infiltration with lymphocytes and IgG4 secreting plasma cells, significant fibrosis and response well to steroids. It is typically a systemic disease that involves various organs. We are presenting a 57-year-old female who was found to have elevated serum creatinine, Proteinuria. An ultrasound scan revealed bilateral hydronephrosis and an abdominal computed tomography (CT) scan illustrated a large retroperitoneal mass. Further evaluation of the mass with whole-body positron emission tomography (PET CT) scan was suggestive of retroperitoneal fibrosis or lymphoma. A CT guided biopsy was done, and results were consistent with fibrosis in the context of IgG-4 related disease. IgG-4 serum levels were elevated. The patient was treated with oral steroids. Azathioprine and Rituximab. Renal function improved significantly and was stable upon 3 months follow up with a decrease in IgG-4 levels and with a significant reduction in the mass lesion.

Keywords: IgG-4 related diseases (IgG-4 RD), Retroperitoneal fibrosis (RPF), Obstructive nephropathy.

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How to cite this article:

Mouhammad J Alawad, Saffa Elawad, Bara Wazwaz, Abdul Wahab Al Allaf.IgG4-related disease presenting with renal injury and obstructive nephropathy discovered incidentally: a case report with literature review. International Journal of Case Reports, 2022, 6:265. DOI: 10.28933/ijcr-2022-01-2405

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