A 26-Year-Old Woman With Pleural Effusion, Hydronephrosis, and Ascites

Kaleem Ullah Toori, MB BS; Sumaira Nabi, MB BS; Sadaf Khattak, MB BS; Herbert S Diamond, MD

Disclosures

August 16, 2018

SLE is an inflammatory disorder that can involve virtually any organ system. The prevalence of SLE varies with ethnicity and location, reflecting the interaction of genetic and environmental influences. The best estimates in white individuals is approximately 30 cases/100,000 population, whereas the rate in black individuals is closer to 100 cases/100,000 population.[1,2,3] The disease targets females 9 times as frequently as males and mainly affects women of reproductive age, although it can occur at any age.[4]

SLE is characterized by immune system deregulation, leading to the production of antinuclear and other autoantibodies. These can form circulating immune complexes or bind directly to tissues.[4] Many immune disturbances are observed, including genetic associations with HLA type, immunoregulatory genes, complement genes, T-cell function, and clearance of apoptotic debris. This disease can involve any part of the body, with cutaneous, renal, neurologic, cardiac, pulmonary, and/or hematologic manifestations. Joint inflammation causing arthralgia and arthritis and dermatologic manifestations are the most common presentations.

Gastrointestinal (GI) symptoms occur in as many as 50% of cases.[5] In 1895, Sir William Osler was the first researcher to emphasize the myriad abdominal complaints seen in SLE, and the ability of SLE to mimic other diseases.[6] The most common symptoms include anorexia, nausea, vomiting, diarrhea and abdominal pain. Complications such as lupus vasculitis that involves the vasculature of the GI tract, chronic intestinal pseudo-obstruction, and sterile peritonitis due to serositis can manifest as chronic abdominal pain and distension and, rarely, as an acute abdomen.[7]

GI vasculitis in SLE can involve any part of the gut from the esophagus to the colon, with a variable prevalence of 0.2%-53%.[8] However, most cases of GI vasculitis involve the superior mesenteric artery, (ie, the small intestine).[9] Symptoms are often nonspecific and may include bloating, anorexia, nausea, vomiting, diarrhea, or rarely as an acute abdomen. It is almost always accompanied by clinical and biochemical evidence of active disease.[7,8,9,10]

Some patients present with chronic intestinal pseudo-obstruction due to intestinal hypomotility (as was seen in this patient).[6] It is a rare clinical syndrome of unknown pathophysiology and can be caused by the involvement of the visceral smooth muscle, the enteric nerves, or the visceral autonomic nervous system.[10,11] Similar complications may be seen in systemic sclerosis and other related disorders.[12]

Genitourinary manifestations are rare and are often associated with GI disorders. The pathogenesis of lupus cystitis is proposed to be an immune-complex mediated vasculitis. The resulting chronic inflammation and urologic dysmotility leads to bladder wall thickening, obstructive uropathy and hydronephrosis.[13] The patient in this case had bilateral hydronephrosis with a thickened bladder wall on ultrasonography and CT scanning, which was later confirmed on cystoscopy. These are the most common urologic manifestations of SLE and the vasculitic phenomenon.[13,14]

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