Clinical and Pathological Characteristics of IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis Diagnosed Based on Experts’ Diagnosis

Abstract

IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men, 15/84; mean age 67.3±9.5 years), 33 were diagnosed based on the histopathological findings of perivascular/retroperitoneal lesions, 50 were diagnosed based on the characteristic imaging findings of perivascular/ retroperitoneal lesions and the presence of definitive IgG4related disease in other organ(s), and the remaining 16 patients were diagnosed by experts based on the characteristic imaging findings of perivascular/retroperitoneal legions, serological findings, response to glucocorticoid treatment, and/or the presence of suspected IgG4-related disease in other organ(s). According to the new organ-specific criteria proposed by experts, 73 (73.7%) diagnoses were categorized to be definitive, and 6 (6.1%) and 17 (17.2%) diagnoses were categorized to be probable and possible, respectively. Further analyses are needed to clarify the optimal diagnostic and therapeutic strategy of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. (This is a translation of

Tertiary lymphoid tissue in early‐stage IgG4-related tubulointerstitial nephritis incidentally detected with a tumor lesion of the ureteropelvic junction: a case report

“…Although ectopic GC frequently form in head and neck lesions [ 9 , 25 ], very few cases with IgG4-TIN accompanied by GC formation in kidney lesions have been reported [ 12 – 14 ]. Similarly, the frequency of ectopic GCs is rare and their number is small in the pancreas and retroperitoneal lesions [ 26 ]. TLT formation reflects the immune response against locally displayed antigens [ 21 ].…”

Renal Involvement in Retroperitoneal Fibrosis: Prevalence, Impact and Management Challenges

“…A Japanese multicenter study investigating 99 patients with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis showed the prevalence of kidney lesion to be 16.2%. 31 A Chinese retrospective study analyzing 58 patients with IgG4-related RPF among 407 with IgG4-RD reported 25.9% of cases with kidney lesion in the 58 patients. 9 On the other hand, non-IgG4-related IRPF rarely has extra-retroperitoneal lesions including renal ones.…”

Granulocyte colony stimulating factor-associated aortitis evaluated via multiple imaging modalities including vascular echography: a case report

“…These lesions tend to be detected in men in their sixties. 11 Other differential diagnoses include infection (bacterial, syphilis, human immunodeficiency virus, and tuberculosis), drug-induced disease, 12 malignancy, 13 Behçet disease, Cogan syndrome, systemic lupus erythematosus, and anti-neutrophil cytoplasmic antibody-related vasculitis ( Supplementary material online, Figure S2 ). 14 The differential diagnosis needs to be performed based on the characteristics of the diseases in addition to the imaging evaluation.…”