Progression of esophageal dysmotility in systemic sclerosis.

OBJECTIVE: To longitudinally evaluate esophageal dysmotility (ED) in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). METHODS: We performed a retrospective review of all adult patients with SSc seen between 1995 and 2008. Patients were included if they had undergone 2 or more esophageal transit scintigraphy (ETS) studies at least 1 year apart. Data from 382 ETS studies of 102 patients with SSc were analyzed. Eighty patients had lcSSc and 22 patients had dcSSc. A grading system was used to quantify the degree of esophageal dysfunction, ranging from grade 0 (normal) to grade 3 (severe hypomotility). Change in esophageal motility over time was evaluated and compared between the limited and diffuse subtypes. RESULTS: Sixty-eight patients (66.7%) had an abnormal ETS study at any time. Of patients with dcSSc, 95.4% had an abnormal ETS study, compared to 58.5% of patients with lcSSc. dcSSc and regurgitation were independent risk factors for ED. There was no association between the presence of anticentromere antibodies or antitopoisomerase (anti-Scl-70) antibodies and an abnormal ETS study. Esophageal motility in patients with dcSSc worsened in 96% of cases compared with only 58.8% in those with lcSSc. CONCLUSION: ED is more frequent in patients with dcSSc than in those with lcSSc, and is more likely to deteriorate over time. Given the potential associated risks of erosive esophagitis, Barrett's esophagus, and esophageal cancer in patients with SSc, routine screening and monitoring for ED is advised.

Benign spontaneous pneumoperitoneum in systemic sclerosis.

The gastrointestinal tract is commonly involved in patients with systemic sclerosis (SSc) (Am J Gastroenterol. 1972;58:30-44). Intestinal manifestations of SSc include impaired motility, malabsorption, bacterial overgrowth, jejunal and wide-mouthed colonic diverticuli, localized dilatation, pseudo-obstruction, and, less commonly, pneumatosis cystoides intestinalis (PCI) (Rheumatology. 2009;48:iii36-iii39; J Clin Gastroenterol. 2008;42:5-12). Benign spontaneous pneumoperitoneum is a rare complication of SSc and is often associated with PCI (Mo Med. 1967;64:117-118). We report a case of spontaneous pneumoperitoneum without evidence of peritoneal irritation in a malnourished patient with long-standing SSc. Computed tomography scan of the abdomen and pelvis on admission revealed pneumoperitoneum with PCI without visceral perforation. Subsequent imaging revealed the persistence of the pneumoperitoneum with resolution of the PCI. It is important for the clinician to recognize benign spontaneous pneumoperitoneum as a possible complication in patients with SSc and to be able to differentiate this from a ruptured viscus with peritonitis based on clinical presentation. The management of benign spontaneous pneumoperitoneum is supportive, thereby avoiding unnecessary surgery.

Orbital Wegener's granulomatosis: a case report and review of the literature.

Wegener's granulomatosis (WG) is a multisystem granulomatous, necrotizing vasculitis of presumed autoimmune origin that affects small- to medium-sized blood vessels. The respiratory tract and kidneys are typically involved (Gross and Reinhold-Keller, "Clinical features of primary ANCA-associated vasculitis" in Oxford textbook of rheumatology, third edition, 2004). The limited form usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease (Cassan et al., Am. J. Med. 49:366-379, 1970). Ocular involvement, which may be the initial manifestation, is often encountered and can result in significant morbidity and possibly blindness (Pakrou et al., Semin. Arthritis Rheum. 35:284-292, 2006). We report an unusual case of WG presenting as an orbital mass. The diagnostic triad of granulomatous inflammation with multinucleated giant cells, vasculitis, and necrosis was discovered on histopathology (McDonald and Edwards, JAMA 173:1205-1209, 1960).