Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report 3 cases of a hitherto undescribed phenomenon in women aged 46 to 49 in which there was replacement of the myometrium or cervical stroma by an accumulation of hypocellular myxoid stromal material containing bland spindle-shaped cells and small blood vessels. In all cases, this change was conspicuous, and in 2 it was multifocal and widespread, resulting in consideration of an infiltrative myxoid mesenchymal neoplasm. Immunohistochemistry revealed a characteristic immunophenotype with diffuse strong positivity with CD34 and CD10 but no immunoreactivity with the smooth muscle markers desmin, alpha-smooth muscle actin, and h-caldesmon. In comparison, 3 cases of uterine myxoid leiomyoma were positive with smooth muscle markers and negative with CD34 and CD10. We believe the lesion we describe to be an unusual pseudoneoplastic, possibly degenerative, phenomenon. The patients past medical histories were unremarkable, with no history of connective tissue disease (myometrial myxoidosis has rarely been described in association with lupus erytematosus) or Carney's syndrome. Two patients had been prescribed local or systemic progestogens, raising the possibility of an association with these compounds. Pathologists should be aware of this unusual pseudoneoplastic phenomenon to avoid a misdiagnosis of a neoplastic lesion.
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PMID:Myxoid change of the myometrium and cervical stroma: description of a hitherto unreported non-neoplastic phenomenon with discussion of myxoid uterine lesions. 2056 49

Germinal centers (GC) give rise to high-affinity and long-lived Abs and are critical in immunity and autoimmunity. IL-27 supports GCs by promoting survival and function of T follicular helper cells. We demonstrate that IL-27 also directly enhances GC B cell function. Exposure of naive human B cells to rIL-27 during in vitro activation enhanced their differentiation into CD20+CD38+CD27lowCD95+CD10+ cells, consistent with the surface marker phenotype of GC B cells. This effect was inhibited by loss-of-function mutations in STAT1 but not STAT3 To extend these findings, we studied the in vivo effects of IL-27 signals to B cells in the GC-driven Roquinsan/san lupus mouse model. Il27ra-/-Roquinsan/san mice exhibited significantly reduced GCs, IgG2a(c)+ autoantibodies, and nephritis. Mixed bone marrow chimeras confirmed that IL-27 acts through B cell- and CD4+ T cell-intrinsic mechanisms to support GCs and alter the production of pathogenic Ig isotypes. To our knowledge, our data provide the first evidence that IL-27 signals directly to B cells promote GCs and support the role of IL-27 in lupus.
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PMID:IL-27 Directly Enhances Germinal Center B Cell Activity and Potentiates Lupus in Sanroque Mice. 2761 97

The disease entity of acquired cystic disease-associated renal cell carcinoma (ACD-RCC) has been recently incorporated into the international renal tumor classification. We performed a clinicopathologic study of a patient with bilateral and multifocal ACD-RCCs. The patient received long-term hemodialysis in the end-stage renal disease caused by systemic lupus erythematous. First left-sided nephrectomy and after 6 months right-sided nephrectomy was performed. None of the preoperative radiologic examinations revealed lesions suspected of malignancy. All of the 6 tumors were incidentally found on grossing radical nephrectomy specimens. Histologically, tumors consisted of a variety of growth patterns (including papillary and cribriform) of neoplastic cells with granular eosinophilic cytoplasm and intratumoral oxalate crystals. Neoplastic cells were positive for AMACR, CK AE1/AE3, and CD10; focally positive for CK7; and negative for PAX8. Seven months after the first nephrectomy, the patient still receives dialysis. There was no evidence of lymph node or distant metastases.
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PMID:Bilateral and Multifocal Acquired Cystic Disease-Associated Renal Cell Carcinomas in Patient With End-Stage Renal Disease Caused by Systemic Lupus Erythematosus. 3251 33