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Systemic lupus erythematous (SLE) is a chronic inflammatory disease of unknown etiology. It can affect nearly any organ. Gastrointestinal (GI) involvement in SLE is frequent but is mostly related to medication side effects and concomitant infections. Chylous ascites is a rare form of ascites that is milky appearing due to the high concentration of...
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... 6 Among these cases, only 2 were from Western populations, one was Latin-American, and only 2 had (non-massive) chylothorax as the initial manifestation of SLE. 7,8 The mechanisms by which SLE causes fluid accumulation have yet to be entirely understood. Inflammation of the lymphatic vessels may increase intraluminal lymphatic pressure, leading to fluid drainage into the serous cavities. ...
This case report describes a 23-year-old male patient who presented with right chylothorax as the initial manifestation of a severe flare of systemic lupus erythematosus (SLE) and secondary Evans syndrome. Chylothorax and chylous ascites are rare features of SLE that can occur due to the accumulation of triglyceride-rich fluid in serous cavities. However, they have never been reported as the initial manifestation of a lupus flare. Evans syndrome is a rare disease characterized by autoimmune hemolytic anemia and immune thrombocytopenia, which can be secondary to SLE. The concomitant occurrence of both chylothorax and Evans syndrome in the setting of systemic lupus erythematosus has never been described, and the exact causative mechanisms of both entities are yet to be fully understood. In this report, we discuss our approach to this challenging case to broaden the understanding of the clinical manifestations of systemic lupus erythematosus. Our findings emphasize the importance of considering rare features of systemic lupus erythematosus and secondary diseases when evaluating patients with the disease.
... [7,8] Patients with SLE may present or develop chylothorax and/or chylous ascites concomitant with the primary disease. [6,[9][10][11][12][13][14][15] To the best of our knowledge, fewer than 15 cases of chylothorax and/or chylous ascites secondary to SLE have been reported in English-language literature, and clinical data have been limited to case reports or small cohorts. [6,[9][10][11][12][13][14][15] Because the clinical characteristics of SLE-related chylothorax and/or chylous ascites remain largely unknown, additional studies are required to improve our understanding of this rare disorder. ...
... [6,[9][10][11][12][13][14][15] To the best of our knowledge, fewer than 15 cases of chylothorax and/or chylous ascites secondary to SLE have been reported in English-language literature, and clinical data have been limited to case reports or small cohorts. [6,[9][10][11][12][13][14][15] Because the clinical characteristics of SLE-related chylothorax and/or chylous ascites remain largely unknown, additional studies are required to improve our understanding of this rare disorder. ...
... Lee et al [10] 47/F Lin et al [12] 43/F Chen et al [13] 93/F Hasan et al [14] 52/F African-American in SLE patients was 35.7 ± 3.7 years. The disease duration of chylothorax and/or chylous ascites was 13.7 ± 3.4 months. ...
This analysis of clinical data from systemic lupus erythematosus (SLE) patients with chylothorax and/or chylous ascites was conducted to guide further clinical work.
From June 2008 to June 2019, 15 SLE patients (14 females and 1 male) with chylothorax and/or chylous ascites were hospitalized at the Beijing Shijitan Hospital. Sixty SLE patients without chylothorax and chylous ascites were randomly selected as controls. Patients’, clinical data was investigated.
The mean age of onset of chylothorax and/or chylous ascites in patients with SLE was 35.7 ± 3.7 years (range, 15–69 years). The mean disease duration of chylothorax and/or chylous ascites in patients with SLE was 13.7 ± 3.4 months (range, 1–48 months). Patients with chylothorax and/or chylous ascites were always diagnosed at later stages of SLE compared with the controls. Among cases, glomerulonephritis and hematologic system involvement were the most common complications. Anti-Sjogren's syndrome antigen A antibody was positive in 7 cases (46.7%). Among cases, direct lymphangiography was performed in 13 patients, indicating thoracic duct outlet obstruction or a poor backflow at the terminal of the thoracic duct. Subsequently, 13 patients were treated with corticosteroids, combined with immunosuppressants in 11 patients and thoracic duct surgery in 6 patients. Eleven patients were followed up for 0.5 to 7.0 years. One patient died of infection. Eight patients (53.3%) achieved remission.
Chylothorax and/or chylous ascites are rare complications of SLE. An early diagnosis and timely initiation of glucocorticoids, immunosuppressants, and surgery are critical to relieve symptoms and to improve prognosis.
... 2 Lupus as a cause of chylous ascites has been described only in adults and elderly people. 1,[3][4][5][6][7] We have reviewed all reported cases of chylous ascites with SLE and were able to find only seven cases in literature (Table 2). However, to the best of our knowledge pediatric lupus presenting as chylous ascites has never been described to date. ...
Systemic lupus erythematosus (SLE) is an autoimmune disorder that can affect virtually any organ. Chylous ascites as a presenting manifestation of SLE has been described in a handful of cases in adults. However, to the best of our knowledge this presentation has never been reported in the pediatric age group. Podocytopathy in SLE was initially considered to be a chance association. However, more recently it has been suggested that minimal change disease is not only a chance association; it is part of the lupus nephritis spectrum.
