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![Figure 2. Causes of ascites in SLE [15,21].](profile/M-K-Garg/publication/351461227/figure/fig2/AS:1031539966099458@1622949701673/Causes-of-ascites-in-SLE-15-21_Q320.jpg)
Gastrointestinal involvement in systemic lupus erythematosus (SLE) usually occurs in the form of mesenteric vasculitis, protein-losing enteropathy, intestinal pseudo-obstruction, and pancreatitis. We describe a 23-year-old female, a known case of SLE presented with significant ascites and pleural effusion. Further evaluation showed elevated CA-125...
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Context 1
... significant ascites is a rare presentation in SLE patient, which can be secondary to nephrotic syndrome, protein-losing enteropathy, chronic liver disease due to autoimmune hepatitis and lupus peritonitis (Figure 2). A diagnostic algorithm has been suggested to find out the cause of ascites in a SLE patient (Figure 3). Kawashiri et al. classified lupus peritonitis into two groups, acute and chronic [5]. ...
Citations
... In the context of most PPMS patients, the administration of immunosuppressants and corticosteroids is the standard approach (4,5). The attainment of remission has been realized through the strategic utilization of MMF, azathioprine, cyclophosphamide, and rituximab (9). ...
Elevated CA-125 levels, polyserous effusions (such as pleural effusion, ascites, etc.) in young women with systemic lupus erythematosus (SLE) may signal pseudo-pseudo Meigs’ syndrome (PPMS), after excluding other causes. We describe a 32-year-old SLE patient with recurrent bilateral pleural effusions and unexplained hypercalcemia for 10 months. Extensive evaluations revealed no infections or tumors. Cytokine analysis showed elevated interleukin (IL) levels, especially IL-6 in pleural effusion. Treatment with immunosuppressive therapy resulted in reduced cancer antigen (CA) 125 levels and decreased effusion volume, demonstrating a positive response to intervention in this case of PPMS.
... 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)(345)(346). MUC20 transcript levels were increased with the progression of lupus in the kidneys of MRL/lpr lupus-prone mice (347). ...
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.
... 23-year-old female Yes Corticosteroids, hydroxychloroquine, and azathioprine Meena, et al. [11] 33-year-old female No Corticosteroids, hydroxychloroquine, and mycophenolate mofetil Schmitt, et al. [12] 38-year-old female No Corticosteroids, hydroxychloroquine Ural, et al. [13] 47-year-old female No Corticosteroids Bes, et al. [14] 29-year-old female No Corticosteroids Lee, et al. [2] 54-year-old female Yes Corticosteroids, cyclophosphamide 42-year-old female No Corticosteroids Bes, et al. [15] 40-year-old female No Corticosteroids ...
Tjalma syndrome, also known as pseudo-pseudo Meigs' syndrome, is a rare manifestation of conditions in patients with systemic lupus erythematosus (SLE). The syndrome is characterized by the presence of ascites, pleural effusion, and an elevated cancer antigen-125 (CA-125) level. We present the case of a 27-year-old female patient admitted in 2021 without any comorbidities, who presented with unintentional weight loss, gastrointestinal upset, and ascites. Further evaluation showed elevated CA-125 levels and pleural effusions, with no atypical cells. The patient was initially treated with antiemetics, intravenous fluids, antibiotics, and total parental nutrition with no improvement in her symptoms or laboratory parameters. The results of an autoimmune workup met the criteria for the classification of SLE. After extensive investigation, she was diagnosed with Tjalma syndrome. She was subsequently treated with corticosteroids and hydroxychloroquine, resulting in the rapid resolution of the patient's nausea and emesis, and discharge from the hospital. Her ascites resolved over 4 weeks. Prednisolone was tapered down and azathioprine was added as a steroid-sparing agent. The patient eventually had complete remission of her symptoms, as well as remarkable improvements in her laboratory results. However, 8 months after her initial diagnosis, the patient showed signs of increasing SLE activity with lupus nephritis, anemia, and leukopenia, despite being compliant with her treatment regimen. The patient was initiated on oral prednisolone (1 mg/kg) and azathioprine was replaced with mycophenolate mofetil, which resulted in significant improvement of clinical and laboratory parameters within 3 weeks. Cases of Tjalma syndrome, and specifically, this presentation, are rarely reported in the literature. We present this condition to raise awareness about both the presenting symptoms and therapeutic options for Tjalma syndrome.
The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs’ syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.
The pleural and peritoneal cavity share many related features due to their common celomic origin. Normally these two spaces are completely separated with the development of the diaphragm. Defects in diaphragm morphogenesis may result in congenital diaphragmatic hernias, which is the most known form of communication between the pleural and peritoneal cavity. However, in several cases, findings of pleuroperitoneal communication (PPC) have been described in adults through an apparently intact diaphragm. In this comprehensive review we systematically evaluate clinical scenarios of this form of “unexpected” PPC as reported in the literature and focus on the possible mechanisms involved.
Systemic lupus erythematosus (SLE) has the potential to affect virtually every organ; however, gastrointestinal system manifestations are relatively rare compared to other autoimmune diseases such as systemic sclerosis and inflammatory bowel disease. A 29-year-old female patient attended to the emergency room with abdominal distention, acute onset abdominal pain and constipation. She had watery chronic diarrhea (4-5 times/d) and weight loss (6 kg, 12%) for 4 months. While there was increased intestinal wall thickness, air-liquid levels were shown on abdomen computed tomography scan. The patient underwent abdominal surgery due to diagnosis of ileus. Ileocecal resection was performed and pathologic evaluation revealed intestinal lymphangiectasia. Autoimmune serology was performed with the following resulats: anti-nuclear antibody 1/3200 with homogenous pattern, anti-DNA antibody and anti-Sm/ribonucleoprotein antibodies were positive in addition to low complement levels (C3: 0.28 [0.9-1.8 g/L], C4: 0.06 [0.1-0.4 g/L]) indicating diagnosis of SLE. Development of intestinal involvement in SLE (lupus enteritis) is mainly grouped into 3 headings such as mesenteric vasculitis, pseudo-obstruction, and protein-losing enteropathy. Although the pathogenesis of intestinal lymphangiectasia remains unknown, it has been reported that immune complex-mediated visceral vasculitis may result in bowel wall and mucosal edema. To our knowledge this is the first case report accompanying hyperinflammatory response in addition to intestinal lymphangiectasia in SLE. On the other hand, clinicians should be alert for other reasons for hyperinflammatory syndromes rather than COVID-19, even during the pandemic
Systemic lupus erythematosus is a complex disease and can present with almost any organ involvement, including serosal inflammation. Our patient is not known to have any medical problems, presented for the first time with pseudo-pseudo Meigs' syndrome (PPMS), and after extensive workup to rule out other possibilities like infection and malignancy, she was found to have systemic lupus erythematosus. Several other cases have been reported in the literature; our patient had to have a pleural biopsy for completion of workup. She responded to prednisone and Immune suppression therapy (including mycophenolate mofetil (MMF) and Plaquenil).
Background:
Ascites, pleural effusion and raised CA-125 in the absence of malignancy in systemic lupus erythematosus is known as Tjalma syndrome.
Case summary:
We report a special case of a systemic lupus erythematosus patient presenting with Tjalma syndrome. She presented with ascites and elevated CA-125 in the absence of benign or malignant ovarian tumor and no pleural effusions, which is an unusual presentation for this rare condition.
Conclusion:
Tjalma syndrome can present with massive ascites alone without pleural or pericardial effusions.
Pseudo–pseudo Meigs' syndrome (PPMS) exhibits patients with ascites, pleural effusion, elevated CA-125, and diagnosed of systemic lupus erythematosus (SLE) eventually without evidence of ovarian or pelvic tumor. It's a rare diagnosis but it has a good response to treatment. We here present an 82-year-old female, who was found to have ascites, pleural effusion, and elevated CA-125. CT of abdomen revealed absence of pelvic tumor. However, a rapid decline in renal function and progressive proteinuria were also observed. We performed an autoimmune-associated investigation. A diagnosis of late-onset SLE was made due to meeting the criteria of serositis, hemolytic anemia, thrombocytopenia, renal disease, and positive anti-smith antibody. We gave this patient a regimen with steroids and hydroxychloroquine. Both ascites and pleural effusion resolved in one month. PPMS is an important differential diagnosis in female patients with ascites, pleural effusion, and elevated CA-125. A survey of the pelvic tumor should be done first to exclude Meigs' syndrome or pseudo-Meigs’ syndrome. SLE flare-up should be kept in mind even in the elderly.
Aim:
Pseudo-pseudo Meigs' syndrome is a rare entity of systemic lupus erythematosus, which is defined with the combination of pleural effusion, elevated serum CA-125 levels, and ascites. It has similar clinical aspects with gynecological malignancies which may lead gynecologists to perform unnecessary surgeries and lab workouts. This review seeks to point out the importance of diagnosing pseudo-pseudo Meig's syndrome (PPMS) and endeavors to inform gynecologists about the differential diagnoses.
Methods:
This article includes a review of the literature on different cases of PPMS. We searched the PubMed database using the search terms in various combinations "Pseudo-pseudo Meig's syndrome," "Tjalma syndrome," and "SLE."
Results:
According to the current literature, a majority of internal medicine specialists recognized this clinical condition in the past few years and discussed PPMS as a new onset of systemic lupus erythematosus. PPMS is one of the disregarding entities where gynecologists do not consider it as a differential diagnosis in case of less awareness. When a gynecologist evaluates ascites manifesting as acute abdomen; ectopic pregnancy, ovarian hyperstimulation syndrome, cyst rupture, and malignancy come to mind first. Four-thirds of those patients evaluated by gynecologists were performed hysterectomy leading to unnecessary interventions and economic burden on the health system.
Conclusion:
Although the mechanism is unrevealed and remains unclear, PPMS has been noticed in the literature for the past few years. In order to prevent unnecessary interventions, this syndrome should be considered as a differential diagnosis.
