Clinical characteristics of systemic lupus erythematosus with chylothorax and/or chylous ascites

Abstract

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.

Massive Right Chylothorax Secondary to a Severe Systemic Lupus Erythematosus Flare With Secondary Evans Syndrome: A Case Report and Literature Review

“…Interestingly, in the Zhang et al cohort, the decrease in SLE disease activity did not directly correlate with the resolution of CPE. 6 The observed refractoriness could be linked to lymphoscintigraphy findings of residual adhesions or thoracic duct obstructions that persist after SLE systemic inflammation’s resolution. 10 Surgical procedures may be needed in these cases and have shown better responses than medical therapy alone.…”

“…Although pleural effusions are joint in SLE, chylothorax and chylous ascites are extremely rare, with only a few cases reported in English literature. 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.…”

“…In the study by Zhang et al, chylous effusions were found in patients with low disease activity, which contrasts with our case where chylothorax occurred with a high SLEDAI-2K score (6.4 ± 2.1vs 18 points). 6 The time elapsed after diagnosis has also been recognized as an influencing factor in the presentation of chylous effusions. Two studies reported the occurrence of chylothorax at 120 and 20 to 120 months, respectively, after the diagnosis.…”

“…It is worth noting that one-third of patients with chylous effusions secondary to SLE had concurrent hematological issues, with leukopenia being the most common. 6 , 7 , 14 - 16 While a possible link exists between the simultaneous involvement of the hematological and lymphatic systems, further research is needed to clarify this association.…”

A 28-Year-Old Woman Presenting with a Clinical Flare of Systematic Lupus Erythematosus and Abdominal Pain Due to Rectus Sheath Hematoma

“…Ascites is rare in SLE, even rarer in the chylous form. The mechanism of chylous as-cites in SLE remains unknown, but one may speculate about lymphatic stenosis or an increase in endoluminal pressure and permeability of vascular walls [ 15 ]. Other potential causes of chylous ascites, including tuberculosis, filariasis, liver cirrhosis, neoplastic and traumatic factors, were excluded [ 16 ].…”