4. SLE diagnosis and manifestations

LP-056 Pseudo-pseudo meigs syndrome or tjalma syndrome: an initial presentation in a young female with Systemic lupus erythematosus

Abstract

Description Pseudo-pseudo Meigs Syndrome (PPMS) or Tjalma Syndrome is characterized by ascites, pleural effusion, increased CA-125 marker, and a diagnosis of Systemic Lupus Erythematosus (SLE) with no evidence of any gynecologic tumors. It is a rare diagnosis of exclusion from Meigs syndrome and Pseudo-Meigs syndrome which are associated with benign and malignant tumors respectively. We are presenting an interesting case of PPMS which is an initial manifestation in a patient with lupus. Literature search through Pubmed, Google scholar, Scopus, Medline, and HERDIN (Health Research and Information Development Network), revealed only 18 reported cases worldwide. Since it was first described in 2005, this is the first reported case in the Philippines.

A 26-year-old gravida 1 para 1 (1001) Filipino female was admitted due to abdominal distention associated with undocumented intermittent fever, nausea, epigastric pain, easy fatigability, dizziness and arthritis. Physical findings revealed pallor, malar rash, decreased breath sounds on the right lower lung field, distended abdomen and grade two bipedal edema. Immunologic markers were positive (ANA, DsDNA, Coombs test, low C3) thus fulfilling a diagnosis of SLE based on SLICC criteria. CA- 125 was also elevated. Transvaginal ultrasound revealed normal sized anteverted uterus and no evidence of pelvic mass. Whole abdomen computed tomography scan revealed loculated exudative massive ascites. Colonoscopy revealed non-specific colitis and mixed hemorrhoids. Paracentesis and peritoneal fluid biopsy revealed negative for malignancy and tuberculosis. Treatment with glucocorticoids and hydroxychloroquine resulted in disease remission. At one month‘s follow up, there is normal CA 125 level with resolution of ascites and pleural effusion.

Conclusions PPMS associated with SLE is a rare manifestation which needs thorough investigation and prompt management. This rare clinical entity is still new in the medical literature thus a physician must have a high index of clinical suspicion when ruling out other disease entities.

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