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Gazzetta Medica Italiana Archivio per le Scienze Mediche 2017 March;176(3):145-8

DOI: 10.23736/S0393-3660.16.03322-2


language: English

Immunoglobulin-G4-related disease and acute kidney injury: a case report

Filipe M. MARTINS 1, 2, Edite M. PEREIRA 1, Paulo M. BETTENCOURT 1, 2

1 Department of Internal Medicine, Centro Hospitalar São João, Porto, Portugal; 2 Faculty of Medicine, University of Porto, Porto, Portugal


Immunoglobulin-G4-related disease (IgG4-RD) is a recently recognized rare condition characterized by immunomediated fibro-inflammatory lesions with dense lymphoplasmocytic infiltrate rich in IgG4-positive plasma cells. It can affect virtually any organ but in most cases the pancreas, salivary glands and/or retroperitoneum are the preferred sites. The authors report a case of a 43-year-old male admitted to the emergency department because of recent onset of left lumbar pain and vomiting. He had no history of trauma or urinary complaints. Evaluation showed obstructive acute kidney injury and the patient underwent emergent bilateral decompressive nephrostomy. Further assessment revealed periureteral masses suspected of neoplasm so the patient was submitted to right nephroureterectomy and left partial distal ureterectomy with ureteroneocystostomy. Histologic analysis was compatible with Ormond Disease and the patient was referred to the autoimmune diseases clinic where the diagnosis of IgG4-RD was established. He was treated with steroids for one year with clinical and imagiological improvement. IgG4-RD is alone a rare entity and the single retroperitoneal involvement presenting as acute renal failure makes this case unique. Should the diagnosis have been made early and the appropriate treatment started the patient might not have needed surgical treatment. The authors intend to raise awareness to this condition since an early diagnosis and treatment might spare not only disease evolution but also invasive procedures.

KEY WORDS: Immunoglobulin G - Retroperitoneal fibrosis - Acute kidney injury - Autoimmune diseases

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