Esophageal investigations in connective tissue disease - Which tests are most appropriate?

Our aim was to review the use of esophageal investigations in patients with suspected connective tissue disease (CTD). Forty-seven patients (39 women and 8 men) with suspected CTD were referred for esophageal manometry at the gastrointestinal physiology unit in the Royal Victoria Hospital, Belfast, U.K., over a 10-year period (1987-1997). The mean age was 51.7 years (range = 21-79 years). Chart review was conducted 1 to 10 years after manometry t o confirm the final diagnoses: scleroderma was found in 11; CREST (calcinos is, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiect asia), 8; mixed connective tissue disease, 14; Raynaud's alone, 5; and othe r CTDs, 9. All 47 successfully underwent esophageal manometry. In addition to manometry, 24 underwent gastroscopy; 27, barium meal; and 3, esophageal pH studies. Clinically significant esophageal abnormalities were noted in 8 (33%) on gastroscopy, in 15 (56%) on barium meal, and in 31 (66%) on manom etry. Gastroscopy had a significantly lower positivity rate than the others (p < 0.05). Only three patients had pH testing, yet all three pH tests wer e abnormal. During manometry, abnormal findings were significantly more com mon in scleroderma-CREST when compared with other diagnoses (89% vs. 50%; p < 0.02). Thirty-three patients reported dysphagia. Abnormal manometry was more likely in these cases (82% vs. 33%; p < 0.02). A high percentage of pa tients with CTD have significant esophageal motility disorders. Investigati ons were more likely to be positive with scleroderma-CREST than other CTDs, even if dysphagia was present. Barium meal and manometry are more useful t han OGD. pH studies were under-used. There is need for a standardized appro ach to esophageal investigations in patients with CTDs.