Inferior vena cava thrombosis as the initial presentation of IgG4-related retroperitoneal fibrosis: Case report and literature review

IgG4-related disease is an immune-mediated deposition of fibro-inflammatory tissue with IgG4-positive plasma cells and dense fibrosis in a single or multiple organs. It often raises concern for malignancy requiring biopsy for diagnosis. Presentation may vary according to organ involved. IgG4-related retroperitoneal fibrosis accounts for two-thirds of the previously considered idiopathic retroperitoneal fibrosis cases. In IgG4-related retroperitoneal fibrosis, computed tomography scan or magnetic resonance imaging shows periaortic soft tissue that extends from below the kidneys to iliac arteries and entrap ureters causing hydronephrosis and renal failure. We present a rare case of IgG4-related retroperitoneal fibrosis presenting with leg swelling and pain due to inferior vena cava compression and thrombosis, and we review current concepts on disease pathogenesis, diagnosis and treatment.

[1]  J. Stone,et al.  Emerging Treatment Models in Rheumatology: IgG4‐Related Disease: Insights Into Human Immunology and Targeted Therapies , 2017, Arthritis & rheumatology.

[2]  P. Lipsky,et al.  Circulating plasmablasts/plasma cells: a potential biomarker for IgG4-related disease , 2017, Arthritis Research & Therapy.

[3]  J. Stone,et al.  IgG 4 -Related Disease , 2017 .

[4]  A. Vaglio,et al.  Erdheim-Chester Disease as a Mimic of IgG4-Related Disease , 2016, Medicine.

[5]  F. Baas,et al.  Immunoglobulin G4+ B‐cell receptor clones distinguish immunoglobulin G 4‐related disease from primary sclerosing cholangitis and biliary/pancreatic malignancies , 2016, Hepatology.

[6]  A. Fernández-Codina,et al.  IgG4-Related Disease: Results From a Multicenter Spanish Registry , 2015, Medicine.

[7]  J. Stone,et al.  International Consensus Guidance Statement on the Management and Treatment of IgG4‐Related Disease , 2015, Arthritis & rheumatology.

[8]  A. Tomelleri,et al.  Erdheim-Chester disease. , 2015, European journal of internal medicine.

[9]  J. Stone,et al.  IgG4-related disease , 2015, The Lancet.

[10]  Jing Yang,et al.  Immunoglobulin G4-related disease mimicking lymphoma in a Chinese patient , 2015, Rheumatology International.

[11]  J. Stone,et al.  Rituximab for IgG4-related disease: a prospective, open-label trial , 2015, Annals of the rheumatic diseases.

[12]  J. Stone,et al.  Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations , 2014, Annals of the rheumatic diseases.

[13]  J. Stone,et al.  The clinical spectrum of IgG4-related disease. , 2014, Autoimmunity reviews.

[14]  J. Stone,et al.  Compléments : De novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG 4 -related disease , 2014 .

[15]  Hua Chen,et al.  Characterizing IgG4-related disease with 18F-FDG PET/CT: a prospective cohort study , 2014, European Journal of Nuclear Medicine and Molecular Imaging.

[16]  Arezou Khosroshahi,et al.  Brief Report: Spuriously Low Serum IgG4 Concentrations Caused by the Prozone Phenomenon in Patients With IgG4‐Related Disease , 2014, Arthritis & rheumatology.

[17]  Vikram Deshpande,et al.  IgG4-Related Disease , 2013, International journal of rheumatology.

[18]  N. Sainani,et al.  Rethinking Ormond’s Disease: “Idiopathic” Retroperitoneal Fibrosis in the Era of IgG4-Related Disease , 2013 .

[19]  K. Momma,et al.  Clinical features of 10 patients with IgG4-related retroperitoneal fibrosis. , 2013, Internal medicine.

[20]  D. Sahani,et al.  Consensus statement on the pathology of IgG4-related disease , 2012, Modern Pathology.

[21]  K. Tsubota,et al.  A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details , 2011, Modern rheumatology.

[22]  Augusto Vaglio,et al.  Retroperitoneal fibrosis. , 2012, Best practice & research. Clinical rheumatology.

[23]  S. Achenbach,et al.  Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. , 2011, Mayo Clinic proceedings.

[24]  A. S. Brandt,et al.  Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry. , 2011, The Journal of urology.

[25]  M. Brodmann,et al.  Thrombosis of the inferior vena cava related to Ormond's disease , 2013, Clinical Rheumatology.

[26]  F. Huang,et al.  Idiopathic retroperitoneal fibrosis (RPF): clinical features of 61 cases and literature review , 2011, Clinical Rheumatology.

[27]  D. Vu,et al.  Where there is blood, there is a way: unusual collateral vessels in superior and inferior vena cava obstruction. , 2010, Radiographics : a review publication of the Radiological Society of North America, Inc.

[28]  T. Hendriksz,et al.  Idiopathic Retroperitoneal Fibrosis Prospective Evaluation of Incidence and Clinicoradiologic Presentation , 2009 .

[29]  P. Scheel,et al.  Retroperitoneal Fibrosis: The Clinical, Laboratory, and Radiographic Presentation , 2009, Medicine.

[30]  P. Parren,et al.  Anti-Inflammatory Activity of Human IgG4 Antibodies by Dynamic Fab Arm Exchange , 2007, Science.

[31]  S. Ferretti,et al.  Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. , 2007, Kidney international.

[32]  Y. Paksoy,et al.  Epidural Venous Plexus Enlargements Presenting With Radiculopathy and Back Pain in Patients With Inferior Vena Cava Obstruction or Occlusion , 2004, Spine.

[33]  H. Nakajima,et al.  Autoimmune pancreatitis is a systemic autoimmune disease , 2003, American Journal of Gastroenterology.

[34]  H. Nakajima,et al.  A new clinicopathological entity of IgG4-related autoimmune disease , 2003, Journal of Gastroenterology.

[35]  A. Stanson,et al.  Iliocaval complications of retroperitoneal fibrosis. , 1994, American journal of surgery.

[36]  P. Tournigand,et al.  [Rare cause of intra-abdominal venous compression: retroperitoneal fibrosis (apropos of a case)]. , 1974, Phlebologie.