Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature.

OBJECTIVE: To review current literature on the gastrointestinal tract (GIT) manifestations of systemic sclerosis (SSc) and to report on 5 patients with severe gastrointestinal SSc. MATERIALS AND METHODS: The clinical course and histopathology of 5 patients are described. A review of the medical literature registered in MedLine and PreMedLine databases from 1996 through mid-2004 was performed using the keywords systemic sclerosis and scleroderma and combining them with text words such as gastric, gastrointestinal, anorectal, colonic, and hepatic. RESULTS: All 5 patients had severe GIT involvement: 4 with diffuse cutaneous SSc (dcSSc) and 1 with limited cutaneous SSc (lcSSc). Autopsy results of 2 patients who died from severe malnutrition and aspiration pneumonia are presented. Literature review includes involvement from oral cavity to anus with varying degrees of severity. Most GIT manifestations result from dysmotility secondary to infiltration of the gastrointestinal wall with fibrous tissue and can cause life-threatening malabsorption and malnutrition. Diagnostic tests, pathology, and treatments of GIT SSc are reviewed. CONCLUSIONS: GIT involvement in SSc can be severely debilitating and even life-threatening. Although morbidity is inevitable, appropriate supportive treatment can prolong survival. RELEVANCE: GI complications of SSc cause significant morbidity and mortality.

Refractory acute kidney transplant rejection with CD20 graft infiltrates and successful therapy with rituximab.

Acute rejection is an expected event after transplantation and has been associated with poor long-term kidney transplant outcome. The presence of B cells in the kidney graft with acute rejection is thought to be an omnious sign, as it has been associated with poor graft outcome. There is no definitive treatment for acute rejection with B cells in the graft. Rituximab, a humanized monoclonal antibody against CD20, has been used in the treatment of B cell lymphoma. We present the case of a 49-yr-old Caucasian male with early acute kidney allograft rejection that was refractory to high doses of steroids and rabbit anti-thymocyte globulin (thymoglobulin). Repeat renal biopsy revealed T cell and B cells in the kidney graft and responded to the combination of rituximab and muromonab (a mouse monoclonal antibody to CD3 receptor). Over 9 months post-transplant, the patient remains rejection free with a serum creatinine of 1.7 mg/dL.

Intraoperative cytology increases the diagnostic accuracy of frozen sections for the confirmation of various tissues in the parathyroid region.

The identification of parathyroid gland tissue and its distinction from adjacent structures such as thyroid gland, lymphoid, fibroadipose, and, rarely, thymic tissues on frozen section (FS) may be challenging owing to freezing artifact. Intraoperative cytology (IC) provides valuable complementary morphologic details. We evaluated 72 specimens with IC alone (group 1), followed by interpretation with FS to reach a final interpretation using IC and FS together (group 2). An additional 105 specimens were evaluated by FS alone (group 3). Permanent section diagnosis was used as the "gold standard." Sensitivity and specificity were 100% for group 2, compared with lower values for group 1 (98% and 100%, respectively) and group 3 (94% and 94%, respectively). IC is a valuable adjunct to FS during intraoperative consultation for evaluation of tissue in a parathyroid location.

Unsedated transnasal endoscopy accurately detects Barrett's metaplasia and dysplasia.

BACKGROUND: Unsedated transnasal upper endoscopy has a diagnostic yield comparable with that of sedated conventional upper endoscopy. The ability of transnasal upper endoscopy to detect Barrett's metaplasia or dysplastic change has not been systematically evaluated. The aim of this study was to assess the feasibility of transnasal upper endoscopy for surveillance of patients with Barrett's esophagus and to evaluate its histopathologic yield for Barrett's metaplasia and dysplasia. METHODS: Thirty-two patients with Barrett's esophagus were evaluated with conventional upper endoscopy and transnasal upper endoscopy. An effort was made to recruit patients known to have dysplasia. Quadrantic biopsy specimens were taken with standard (conventional upper endoscopy) and pediatric (transnasal upper endoscopy) biopsy forceps at procedures performed at least 1 week apart. Two blinded pathologists evaluated the specimens. RESULTS: Transnasal upper endoscopy detected Barrett's metaplasia histopathologically in 31 of 32 patients. Level of agreement for presence of dysplasia in biopsy specimens obtained between conventional upper endoscopy and transnasal upper endoscopy was excellent (k = 0.79). Interobserver agreement for dysplasia in specimens obtained by conventional upper endoscopy (k = 0.61) and by transnasal upper endoscopy (k = 0.61) were similar. Intraobserver agreement between conventional upper endoscopy and transnasal upper endoscopy biopsy specimens for dysplasia by pathologist 1 (k = 0.73) and pathologist 2 (k = 0.75) were also similar. No significant adverse effects were noted. CONCLUSIONS: Transnasal upper endoscopy is feasible and accurate for identification and histopathologic confirmation of Barrett's metaplasia with a histopathologic yield for dysplasia comparable with conventional upper endoscopy.