Chronic Lupus Peritonitis Is Characterized by the Ascites with a Large Content of Interleukin-6

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease and can cause multi-organ damage. Peritoneal involvement, also called lupus peritonitis, is a rare but sometimes fatal manifestation. Deposition of immune complexes consisting of immunoglobulin G and complement is considered to be involved in the pathogenesis of lupus peritonitis; however, it remains unknown whether inflammatory cytokines contribute to the pathology of this manifestation. Here we present two patients with treatment-resistant lupus peritonitis: a 37-year-old woman with a 26-year history of SLE who had been treated with prednisolone and cyclophosphamide followed by azathioprine and a 65-year-old woman with a 33-year history of SLE who had been treated with prednisolone alone. Both patients were admitted to our department because of abdominal distention. Computed tomography scans showed massive ascites. Ascitic fluid examinations of both patients showed leukocytosis with no evidence of malignancy or infection. After eliminating other causes for ascites, they were diagnosed with lupus peritonitis. Despite the intensified immunosuppressive therapy, they died of uncontrolled peritonitis several months after admission. Examinations of the ascites at admission also revealed a large content of interleukin (IL)-6, compared with other inflammatory cytokines, IL-1β and tumor necrosis factor-α. In fact, the ascitic IL-6 levels of these two patients were 12,389 pg/mL and 5,486 pg/mL, much higher than their serum IL-6 levels of 36 pg/mL and 140 pg/mL, respectively. We therefore suggest that IL-6 may contribute to the pathogenesis of lupus peritonitis and that the inhibition of IL-6 signaling may provide a novel therapeutic strategy for lupus peritonitis.

Pseudo-pseudo Meigs’ syndrome: a rare presentation of systemic lupus erythematosus

“…Raised Interleukin-1b, IFN-gamma and VEGF have been described with this condition. The finding of raised serum ferritin and raised IL-6 levels in the ascitic fluid also contribute to the inflammatory theory of pathogenesis of PPMS (12,13). Serum ferritin level in the present case was also slightly elevated although the patient had iron deficiency anemia.…”

Encapsulating Peritoneal Sclerosis in Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Systemic Sclerosis

“…During hospitalization of our patient, the cause of refractory ascites was not fully understood. In general, lupus peritonitis is less common (16,17) and is reported to occur in approximately 8%-11% (18). In our case, the presence of ANA: antinuclear antibody, anti-dsDNA antibody: anti-double stranded DNA antibody, CART: cell-free and concentrated ascites reinfusion therapy, EPS: encapsulating peritoneal sclerosis, MP pulse therapy: methylprednisolone pulse therapy, SLE: systemic lupus erythematosus chronic lupus peritonitis was suspected because of increased exudative ascites with serological activity, such as elevated anti-double stranded DNA IgG antibody titer and hypocomplementemia.…”

Protein‐losing enteropathy as the first presentation of systemic lupus erythematosus: A case report from Sudan

“…Immune complex deposition in the peritoneum with activation of cytokines, IL‐1β, IL6, TNF‐α, INF‐ɣ. Vasculitis of peritoneal vessels may contribute to the pathogenesis of lupus peritonitis 11 …”

“…Vasculitis of peritoneal vessels may contribute to the pathogenesis of lupus peritonitis. 11 Although an association between SLE and proteinlosing enteropathy has been recognized, there have been less than 200 cases documented in world literature 2,12 there are no documented cases of such an association in Sudan literature. SLE presenting as protein-losing enteropathy has been reported even less commonly.…”