Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

Abstract

Intestinal pseudo-obstruction (IPO) is not uncommon in systemic lupus erythematosus (SLE), and IPO in SLE has an apparent association with ureterohydronephrosis. However, hepatobiliary dilatation without mechanical obstruction presenting together with IPO and ureterohydronephrosis is much more scarce in SLE. Here, we named this rare triad of IPO, ureterohydronephrosis, and biliary tract dilatation as visceral muscle dysmotility syndrome (VMDS). It always imitates an acute abdomen and is even life-threatening if treated incorrectly. To diagnose a VMDS, infections and mechanical obstructions should be ruled out carefully. Here, we report a 24-year-old Chinese woman with SLE who presented of VMDS that associated with corticoids tapering induced SLE flare. In this case, early vigorous immunosuppressive treatment conquered the triad timely and thus yielded a good outcome.

Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus

“…Corticosteroids (methylprednisolone and prednisone) were used as the initial and first-line immunosuppressive therapy in 27 patients (68%), 4 – 9 , 11 , 12 , 14 , 15 , 17 – 21 , 22 – 25 18 of them were given pulses of high dose corticosteroids (500 mg or 1000 mg/day), 5 , 6 , 9 , 11 – 14 , 18 – 21 , 22 , 23 , 25 and 9 patients were discharged on 1 to 2 mg/kg/day methylprednisolone or prednisolone. 4 , 7 , 8 , 15 , 17 , 21 , 24 Twenty-two (81%) of them had a good response to corticosteroids.…”

Intestinal Pseudo-Obstruction as an Initial Manifestation of Systemic Lupus Erythematosus

“… 1 In addition, Chen et al observed the presence of a distended common bile duct without any evidence of mechanical obstruction with IPO in patients with SLE. 16 However, no histopathologic evidence of vasculitis was found in some cases of IPO in SLE. 1 17 18 Another pathophysiological mechanism of IPO is an intrinsic muscle dysmotility.…”

“…Although the pathogenic mechanisms have not been clarified, high-dose steroids are helpful in improving the symptoms of IPO in SLE. 2 7 16 Intravenous methylprednisolone initiated at 1 mg/kg/day for 3 days, followed by oral prednisone 1 mg/kg/day resulted in a good clinical response in most cases. 2 8 In this case, the therapeutic effect of oral prednisone could not be evaluated due to poor compliance induced by side effects.…”

“… 1 2 Neostigmine or pyridostigmine are parasympathomimetic and reversible cholinesterase inhibitors. 16 Long-term clinical outcomes vary among patients with SLE who presented with IPO. Narváez et al analyzed 22 cases of IPO in SLE, reporting that 4 patients died within 6 months after presentation with IPO.…”

A case with hydroureteronephrosis accompanied by Lupus Mesenteric Vasculitis

“…Pathophysiological mechanisms underlying the development of IPO in SLE have not been clearly elucidated, although proposed mechanisms include development of visceral smooth muscle injury and dysmotility subsequent to immune-complex mediated vasculitis. The smooth muscle dysfunction may be diffuse and may involve urinary and biliary systems as well [8][9][10]. Mesenteric ischemia due to LMV may be associated with a number of findings in abdominal CT including focal or diffuse intestinal wall thickening, enlarged intestinal segments, abnormal contrast enhancement in the intestines (target or double halo sign), mesenteric edema, engorgement of the vascular structures penetrating the intestinal wall, and ascites.…”