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

Abstract

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.

Case report: Three cases of systemic lupus erythematosus presenting primarily with massive ascites and significantly elevated CA-125 levels and a review of pseudo-pseudo Meigs’ syndrome in literature

“…Notably, of all reported cases, only in the case reported by Horino et al. ( 27 ), the patient experienced lupus activity with abundant ascites for the first time, and the symptoms resolved after treatment. However, after 2 years, the patient’s lupus disease became active again, with the reappearance of massive ascites.…”

Pseudo‐pseudo Meig's syndrome presenting as an acute surgical abdomen: A rare entity and review of the literature

“…The majority of cases were reported by internal medicine physicians (11/17) even though the first case of PPMS reported by a gynecologist-oncologist (Tjalma 2005). Besides in literature, 4 of 17 of the PPMS cases have been evaluated by gynecologists and three of four cases were performed abdominal hysterectomy with the suspicion of cancer [3][4][5] (Table 2). The reason for this might be that at first glance malignancy is the initial diagnosis that comes to mind as in the case of massive ascites and CA-125 elevation.…”