Terri Getzug

Reliability and Validity of the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument

OBJECTIVE To refine the previously developed scleroderma (systemic sclerosis [SSc]) gastrointestinal tract (GIT) instrument (SSC-GIT 1.0). METHODS We administered the SSC-GIT 1.0 and the Short Form 36 to 152 patients with SSc; 1 item was added to the SSC-GIT 1.0 to assess rectal incontinence. In addition, subjects completed a rating of the severity of their GIT involvement (from very mild to very severe). Evaluation of psychometric properties included internal consistency reliability, test-retest reliability (mean time interval 1.1 weeks), and multitrait scaling analysis. RESULTS Study participants were mostly women (84%) and white (81%); 55% had diffuse SSc. Self-rated severity of GIT involvement ranged from no symptoms to very mild (39%), mild (21%), moderate (31%), and severe/very severe (9%). Of an initial 53 items in the SSC-GIT 1.0, 19 items were excluded, leaving a 34-item revised instrument (the University of California, Los Angeles Scleroderma Clinical Trial Consortium GIT 2.0 [UCLA SCTC GIT 2.0]). Analyses supported 7 multi-item scales: reflux, distention/bloating, diarrhea, fecal soilage, constipation, emotional well-being, and social functioning. Test-retest reliability estimates were >/=0.68 and coefficient alphas were >/=0.67. Participants who rated their GIT disease as mild had lower scores on a 0-3 scale on all 7 scales. Symptom scales were also able to discriminate subjects with corresponding clinical GIT diagnoses. The Total GIT Score, developed by averaging 6 of 7 scales (excluding constipation), was reliable and provided greater discrimination between mild, moderate, and severe self-rated GIT involvement than individual scales. CONCLUSION This study provides support for the reliability and validity of the UCLA SCTC GIT 2.0, an improvement over the SSC-GIT 1.0, and supports a Total GIT Score in SSc patients with GIT.

Minimally important differences of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument.

Objective. To provide minimally important difference (MID) estimates for the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0) in a longitudinal observational cohort. Methods. We administered the UCLA SCTC GIT 2.0 to 115 patients with systemic sclerosis (SSc) at 2 timepoints 6 months apart. The UCLA SCTC GIT 2.0 has 7 multi-item scales: Reflux, Distension/Bloating, Diarrhea, Fecal Soilage, Constipation, Emotional Well-being, and Social Functioning and a total GIT score. All scales are scored from 0 [better health-related quality of life (HRQOL)] to 3 (worse HRQOL) except the diarrhea and constipation scales (ranges 0–2 and 0–2.5, respectively). Patients also rated their overall and upper and lower GIT involvement during the second visit using a response scale with options “much better; somewhat better; almost the same; somewhat worse; or much worse.” The minimally changed group was defined by those reporting they were somewhat better or somewhat worse compared to first visit. Results. Study participants were 84% female and 81% white with a mean disease duration of 6.9 years. The MID estimates for improvement ranged from 0.07 for the Social Functioning scale to 0.36 for the Emotional Well-being scale. For worsening, the MID estimates ranged from 0.06 for the Fecal Soilage scale to 0.21 for the Social Functioning scale. Conclusion. We provide MID estimates for the UCLA SCTC GIT 2.0 scales. This information can aid in interpreting scale scores in future randomized controlled trials and observational studies.

Association of Systemic Sclerosis With a Unique Colonic Microbial Consortium.

To compare colonic microbial composition in systemic sclerosis (SSc) patients and healthy controls and to determine whether certain microbial genera are associated with gastrointestinal (GI) tract symptoms in patients with SSc.

Reversal of clonidine toxicity by naloxone

Clonidine is a centrally acting antihypertensive agent used in the management of essential hypertension. Oral clonidine loading is now used frequently in the management of hypertensive urgencies (ie, increases in arterial pressure not associated with acute, life-threatening end-organ injury). We report the case of a patient with an acute inferior myocardial infarction associated with blunt chest trauma who developed an abrupt and unexplained increase in arterial pressure 24 hours after admission and who was treated with oral clonidine (0.5 mg in divided doses over two hours). Drug therapy was followed by prolonged (four hours) systemic arterial hypotension (mean arterial pressure less than 70 mm Hg). Four milligrams of naloxone in two divided doses was given. Each naloxone bolus was followed by a 15-mm-Hg increase in mean arterial pressure and a return to values that were normal for this patient. Naloxone may be of value in reversing clonidine toxicity when clonidine is given to treat an acute rise in arterial pressure.

Systemic sclerosis is associated with a unique colonic microbial consortium

To compare colonic microbial composition in systemic sclerosis (SSc) patients and healthy controls and to determine whether certain microbial genera are associated with gastrointestinal (GI) tract symptoms in patients with SSc.

Management of gastrointestinal involvement in scleroderma

Opinion statementGastrointestinal tract (GIT) is commonly involved in patients with systemic sclerosis (SSc). The GI involvement is quite heterogeneous varying from asymptomatic disease to significant dysmotility causing complications like malabsorption, weight loss, and severe malnutrition. This review focuses on the management of GI involvement in SSc and has been categorized based on the segment of GIT involved. A brief discussion on the role of patient-reported outcome measures in SSc-GI involvement has also been incorporated.

Small and Large Intestinal Involvement

Small intestinal involvement occurs in 40–70% of SSc patients while large intestinal and anorectal involvement occur in 20–50% and 50–70%, respectively. These intestinal involvements impact SSc patients in several adverse ways: high morbidity, a poor quality of life, and, in a few instances, shortened life spans. In fact, GI involvement is a major contributor to poor quality of life as exemplified by different cohort studies.