Pseudo-pseudo Meigs’ syndrome: a rare presentation of systemic lupus erythematosus

Tansir, Ghazal; Kumar, Pravin; Pius, Abish; Sandhu, Sunny; Soneja, Manish

doi:10.4081/reumatismo.2019.1140

Cited by 15 publications

(6 citation statements)

References 7 publications

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“…Pseudo-pseudo Meigs' syndrome presents with clinical features similar to Meigs' syndrome and pseudo-Meigs' syndrome, including pleural effusion, ascites, and a high CA 125 level, and is easily misdiagnosed. While Meigs' syndrome and pseudo-Meigs' syndrome are secondary to benign ovarian fibromas or other abdominal or pelvic malignancies and resolve after tumour removal, pseudo-pseudo Meigs' syndrome occurs in SLE patients without abdominal or pelvic tumours [9]. The pathophysiology of underlying ascites in pseudo-pseudo Meigs' syndrome is debated.…”

Section: Discussionmentioning

confidence: 99%

“…CA 125 is a commonly used tumour marker, but lacks sensitivity and specificity for ovarian malignancies; its levels can be elevated in other conditions (e.g. SLE) [9]. In inflammatory conditions, CA 125 secretion is increased from the mesovarium and omentum [11].…”

Section: Discussionmentioning

confidence: 99%

“…Corticosteroids are effective for LUPLE and pseudo-pseudo Meigs' syndrome, and a good prognosis has been shown with a combined steroid and immunosuppressive therapy [2,8,9]. In treating pseudo-pseudo Meigs' syndrome, administering moderate-dose corticosteroids in case of an underlying tumour, Meigs' syndrome, and pseudo-Meigs' syndrome and then adding immunosuppressive agents are recommended [12].…”

Section: Discussionmentioning

confidence: 99%

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Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs’ syndrome and successfully treated with hydroxychloroquine

Horino

Ogasawara

Kashio

et al. 2022

Romanian Journal of Internal Medicine

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We herein report the first case of lupus-related protein-losing enteropathy associated with pseudo–pseudo Meigs’ syndrome. Lupus-related protein-losing enteropathy and pseudo–pseudo Meigs’ syndrome are extremely rare complications in patients with systemic lupus erythematosus, Both have a similar clinical course characterized by producing marked ascites, and respond to steroids in typical cases. However, in our case, steroid monotherapy was inadequate and the addition of hydroxychloroquine was effective for their treatment. Furthermore, no reports have previously confirmed elevated CA 125 levels with lupus-related protein-losing enteropathy or increased 99mTc-HSA activity with pseudo–pseudo Meigs’ syndrome. In addition, we are the first to report an evaluation of the histopathology of lupus-related protein-losing enteropathy. Previously reported cases have been described as being caused by either pseudo-Meigs’s syndrome or lupus-related protein-losing enteropathy as the cause of the rare pathology that causes marked pleural effusion and ascites in patients with systemic lupus erythematosus, but It has not been evaluated whether the other is co-occurring. Our case highlights that there is a potential case of overlapping lupus-related protein-losing enteropathy and pseudo-Pseudo-Meigs’s syndrome. Furthermore, it is possible that patients with marked ascites with elevated CA 125 levels were mistakenly diagnosed with Meigs’s syndrome or pseudo-Meigs’s syndrome associated with malignant or benign ovarian tumors and underwent surgery. Clinicians should not forget SLE with pseudo-Pseudo-Meigs’s syndrome as one of the differential diagnoses for marked ascites with elevated CA 125 levels.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs’ syndrome and successfully treated with hydroxychloroquine

Horino

Ogasawara

Kashio

et al. 2022

Romanian Journal of Internal Medicine

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“…SLE patients with serositis had greater MUC16 levels and disease duration compared with SLE patients without serositis ( 343 ). Pseudo–pseudo Meigs’ syndrome is a rare manifestation in SLE patients presenting with ascites, pleural effusion, and elevated serum MUC16 levels unrelated to malignancy ( 344 – 346 ). MUC20 transcript levels were increased with the progression of lupus in the kidneys of MRL/lpr lupus-prone mice ( 347 ).…”

Section: Neus and Mucs In Autoimmune Diseasesmentioning

confidence: 99%

Mammalian Neuraminidases in Immune-Mediated Diseases: Mucins and Beyond

2022

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Mammalian neuraminidases (NEUs), also known as sialidases, are enzymes that cleave off the terminal neuraminic, or sialic, acid resides from the carbohydrate moieties of glycolipids and glycoproteins. A rapidly growing body of literature indicates that in addition to their metabolic functions, NEUs also regulate the activity of their glycoprotein targets. The simple post-translational modification of NEU protein targets—removal of the highly electronegative sialic acid—affects protein folding, alters protein interactions with their ligands, and exposes or covers proteolytic sites. Through such effects, NEUs regulate the downstream processes in which their glycoprotein targets participate. A major target of desialylation by NEUs are mucins (MUCs), and such post-translational modification contributes to regulation of disease processes. In this review, we focus on the regulatory roles of NEU-modified MUCs as coordinators of disease pathogenesis in fibrotic, inflammatory, infectious, and autoimmune diseases. Special attention is placed on the most abundant and best studied NEU1, and its recently discovered important target, mucin-1 (MUC1). The role of the NEU1 - MUC1 axis in disease pathogenesis is discussed, along with regulatory contributions from other MUCs and other pathophysiologically important NEU targets.

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“…This is evident in patients with adenoma, where it was found that the CA125 concentration may reach 100 times its characteristic value [ 102 ]. Among the cases in which CA125 values are increased are lung diseases, including active tuberculosis, with values up to four times, the discriminatory value, which also increases to twice the discriminatory value in pneumonia, chronic obstructive pulmonary disease (COPD), lupus erythematosus, and interstitial lung disease [ 103 , 104 , 105 ].…”

Section: Muc16 (Ca 125) and Non-malignant Proliferationmentioning

confidence: 99%

The Potential Role of MUC16 (CA125) Biomarker in Lung Cancer: A Magic Biomarker but with Adversity

et al. 2022

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Lung cancer is the second most commonly diagnosed cancer in the world. In terms of the diagnosis of lung cancer, combination carcinoembryonic antigen (CEA) and cancer antigen 125 (CA125) detection had higher sensitivity, specificity, and diagnostic odds ratios than CEA detection alone. Most individuals with elevated serum CA125 levels had lung cancer that was either in stage 3 or stage 4. Serum CA125 levels were similarly elevated in lung cancer patients who also had pleural effusions or ascites. Furthermore, there is strong evidence that human lung cancer produces CA125 in vitro, which suggests that other clinical illnesses outside of ovarian cancer could also be responsible for the rise of CA125. MUC16 (CA125) is a natural killer cell inhibitor. As a screening test for lung and ovarian cancer diagnosis and prognosis in the early stages, CA125 has been widely used as a marker in three different clinical settings. MUC16 mRNA levels in lung cancer are increased regardless of gender. As well, increased expression of mutated MUC16 enhances lung cancer cells proliferation and growth. Additionally, the CA125 serum level is thought to be a key indicator for lung cancer metastasis to the liver. Further, CA125 could be a useful biomarker in other cancer types diagnoses like ovarian, breast, and pancreatic cancers. One of the important limitations of CA125 as a first step in such a screening technique is that up to 20% of ovarian tumors lack antigen expression. Each of the 10 possible serum markers was expressed in 29–100% of ovarian tumors with minimal or no CA125 expression. Therefore, there is a controversy regarding CA125 in the diagnosis and prognosis of lung cancer and other cancer types. In this state, preclinical and clinical studies are warranted to elucidate the clinical benefit of CA125 in the diagnosis and prognosis of lung cancer.

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Pseudo-pseudo Meigs’ syndrome: a rare presentation of systemic lupus erythematosus

Cited by 15 publications

References 7 publications

Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs’ syndrome and successfully treated with hydroxychloroquine

Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs’ syndrome and successfully treated with hydroxychloroquine

Mammalian Neuraminidases in Immune-Mediated Diseases: Mucins and Beyond

The Potential Role of MUC16 (CA125) Biomarker in Lung Cancer: A Magic Biomarker but with Adversity

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