Evidence Of IgG4-related Disease Research Articles

IgG4 related disease involving the renal pelvis: a case report and the literature review

Objective To summarize the characteristics of IgG4 related disease(IgG4-RD), and to avoid unnecessary surgical procedures due to misdiagnosis. Methods Retrospective analysis of the clinical data of one case of IgG4 related disease involving the renal pelvis in our hospital, and the clinical features of IgG4 related disease involving the renal pelvis were reviewed and discussed. A 56-year-old man presented with microscopic hematuria, CT showed right renal pelvis and ureteral wall thickening, local soft tissue density, post contrast CT showed low-density renal pelvic mass and the wall of the ureter-pelvic was irregularly thicken, indicating renal pelvic cancer and lymph node metastasis. PET-CT(18F-FDG) findings indicated that the renal pelvic mass was a malignant tumor, because the glucose metabolism was very high. The preoperative diagnosis was retroperitoneal lesion, suspecting renal pelvic carcinoma or lymphoma. The patient underwent 3D laparoscopic nephroureterectomy with out preoperative biopsy. During operation, we found extensive enlargement of the right renal portal and retroperitoneal lymph nodes, including that the renal portal and the renal pelvis mass had a serious adhesion to the vena cava and renal arterial vein. Results Postoperative pathology results revealed lymphatic tissue hyperplasia and germinal center formation. Immunohistochemical staining showed that most plasma cells positive expression of IgG4 (IgG4>40%, IgG4>100 /HPF), reactive hyperplasia of lymph node, considering the IgG4 related diseases. Serum IgG4 level was 15.2 g/L(0.03-2.01 g/L)after operation. Combined with serological examination, IgG4 related disease was confirmed. There was no evidence of IgG4-related disease at any other site during the follow up period, thereby obviating the need for any additional therapy. Conclusions IgG4 related disease is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells, which leads to the enlargement of the organ or nodular/proliferative lesions. The disease can be revealed as a soft tissue tumor of the renal pelvis and be misdiagnosed as malignant tumor. Surgical intervention could be avoided according to the correct diagnosis. Key words: IgG4 related disease; Renal pelvic carcinoma; Surgery; Diagnosis and therapy

Polyclonality in Sclerosing Angiomatoid Nodular Transformation of the Spleen.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a morphologically distinctive lesion. Although the clinical course of SANT is benign, its reactive or neoplastic nature remains to be clarified. Furthermore, some investigators have suggested that SANT is related to IgG4 sclerosing lesion or inflammatory pseudotumor with stromal cells positive for Epstein-Barr virus (EBV). In this study, we assessed 22 cases of SANT derived from adult women. Clinical data and follow-up information were obtained by chart review. Immunohistochemical studies for IgG4, IgG, and CD21 stains and in situ hybridization to detect EBV-encoded small RNAs were performed. We also assessed genomic DNA extracted from paraffin-embedded tissue for human androgen-receptor α gene analysis using conventional and methylation-specific polymerase chain reaction methods. The median patient age was 41.5 years (range, 25 to 82 y). Most (77%) patients presented with a single mass that was detected incidentally (59%). The mean size of the lesions was 3.8 cm (range, 1.0 to 9.0 cm). Clinical symptoms correlated with multiple lesions (P=0.043) but not lesional size (P=0.637) or location in the spleen (hilum vs. periphery, P=0.696). None of the cases had evidence of IgG4-related disease or recurred after splenectomy. The mean number of IgG4 cells was 27.7 (range, 4 to 125), and the mean IgG4/IgG ratio was 16.4% (range, 1.6% to 55.7%) with only 2 cases being >40%. Cases with higher IgG4 cells did not correlate with inflammatory pseudotumor-like morphology. No lesions were positive for EBV-encoded small RNAs, and almost all cases with informative results (n=19) showed a polyclonal pattern. We conclude that SANT is a polyclonal, reactive lesion rather than a neoplasm.

IgG4-related inflammatory pseudotumor of the kidney mimicking renal cell carcinoma: A case report.

IgG4-related disease is a recently recognized clinical entity. It is characterized by diffuse organ swelling or mass formation, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis and typically an increased serum IgG4 concentration, which may affect various organs. An 80-year-old woman with an otherwise unremarkable previous medical history was revealed to have a renal mass that was indicative of renal malignant carcinoma, for which a radical nephrectomy was performed. The mass was diagnosed as an IgG4-related inflammatory pseudotumor, which was histopathologically confirmed. The patient is currently well without evidence of IgG4-related disease at 3 months post-surgery, and did not require any additional therapy.

Excision of a Lower Eyelid Inflammatory Pseudotumor Combined with Lower Blepharoplasty.

Excision of a Lower Eyelid Inflammatory Pseudotumor Combined with Lower Blepharoplasty.

IgG4-Related Disease Presented as a Mural Mass in the Stomach

Isolated gastric IgG4-related disease (IgG4-RD) is a very rare tumefactive inflammatory condition, with only a few cases reported to date. A 48-year-old woman was incidentally found to have a subepithelial tumor in the stomach. Given a presumptive diagnosis of gastrointestinal stromal tumor or neuroendocrine tumor, she underwent wedge resection. The lesion was vaguely nodular and mainly involved the submucosa and proper muscle layer. Microscopically, all classical features of type I autoimmune pancreatitis including lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and numerous IgG4-positive plasma cells were seen. She had no evidence of IgG4-RD in other organs. Although very rare, IgG4-RD should be considered one of the differential diagnoses in the setting of gastric wall thickening or subepithelial mass-like lesion. Deep biopsy with awareness of this entity might avoid unnecessary surgical intervention.

IgG4 plasma cell myeloma: new insights into the pathogenesis of IgG4-related disease

IgG4-related disease is a newly described systemic fibroinflammatory process, characterized by increase in IgG4-positive plasma cells. Its pathogenesis, including the role of IgG4, remains poorly understood. Plasma cell myeloma is typically associated with a large monoclonal serum spike, which is frequently of IgG isotype. We sought to identify and characterize a subset of IgG4-secreting myeloma, as it may provide a biological model of disease with high serum levels of IgG4. Six out of 158 bone marrow biopsies (4%) from patients with IgG myeloma expressed IgG4. Four patients were men and two were women, with a mean age of 64 (range 53–87) years. Imaging showed fullness of pancreatic head (1), small non-metabolic lymphadenopathy (1), and bone lytic lesions (6). Two patients developed necrotizing fasciitis. All had elevated serum M-protein (mean 2.4, range 0.5–4.2 g/dl), and none had definite signs or symptoms of IgG4-related disease. Four myelomas had plasmablastic morphology. Four had kappa and two had lambda light chain expression. Three cases expressed CD56. Two patients had a complex karyotype. In conclusion, the frequency of IgG4 myeloma correlates with the normal distribution of IgG4 isoform. The patients with IgG4 myeloma appear to have a high rate of plasmablastic morphology and could be predisposed to necrotizing fasciitis. Despite high serum levels of IgG4, none had evidence of IgG4-related disease. These findings suggest that the increased number of IgG4-positive plasma cells is not the primary etiologic agent in IgG4-related disease. Elevated serum levels of IgG4 is not sufficient to produce the typical disease presentation and should not be considered diagnostic of IgG4-related disease.

IgG4-Related Autoimmune Prostatitis: Is It an Unusual or Underdiagnosed Manifestation of IgG4-Related Disease?

IgG4-related disease (IgG4-RD) encompasses a wide range of extrapancreatic manifestations. Albeit some are relatively well known, others such as autoimmune prostatitis remain poorly described. We present a 61-year-old Latin-American male with autoimmune pancreatitis (AIP) who presented with lower urinary tract symptoms (LUTS), normal prostate specific antigen (PSA) test, and prostate enlargement attributed to benign prostatic hyperplasia (BPH). He underwent a transurethral resection of the prostate (TURP) after which symptoms were resolved. On histopathology, prostatic stroma had a dense inflammatory infiltrate rich in plasma cells and lymphocytes; immunohistochemical morphometric assessment showed >10 IgG4-positive plasma cells/high power field (HPF). The diagnosis of IgG4-related prostatitis was postoperatively. We compared the patient characteristics with those of previous reports on Asian patients. Shared findings included prostate enlargement, LUTS (symptoms that can be confused with BPH), and PSA within normal limits or mild elevations. IgG4-related prostatitis is rarely considered as a preprocedural diagnosis, even in patients with evidence of IgG4-RD. Involved prostate zones include mainly central and transitional zones and less frequently the peripheral. Currently, there is insufficient data about the natural history and outcome. Whether steroids, transurethral resection, or both are the treatment of choice needs to be elucidated.