Primary Biliary Cholangitis

This study was approved by the Institutional Review Board of the University of Texas Southwestern Medical Center at Dallas. The conditions under which a new measure is studied should closely resemble the conditions of eventual use.6 Thus, five different patient groups representing the most common causes of itching were studied. Subjects were recruited from university-affiliated clinics for dermatology, burn, HIV, liver disease and emergent dialysis. Eligibility criteria for the study included: (i) pruritus secondary to one of the following conditions: primary dermatological disease, burn wounds, HIV, hepatobilary disease or chronic kidney disease; (ii) between the ages of 8 and 90 years; (iii) able to complete the questionnaires in English.
After providing written informed consent, subjects were asked about their medical diagnosis, stage of disease, comorbid medical conditions and current medication. All subjects then completed a questionnaire packet which included the 5-D, the VAS, and the itch domain of the PBC-40, a quality of life assessment in patients with the chronic itchy liver disease, primary biliary cirrhosis (PBC). Six weeks later, subjects were asked to repeat the questionnaires and report any changes in stage of disease, medication or skin care regimens. Follow-ups were completed (i) with patients in their regularly scheduled clinic visits whenever possible, and (ii) by contacting subjects by telephone and recording responses to the questionnaires at the time of telephone contact. A subset of patients (n = 50) not treated for their itching also completed the questionnaires 2–3 days after enrolment to assess test-retest reliability. These patients were either (i) approached in person during an inpatient stay or regularly scheduled clinic visit or (ii) contacted by telephone and asked to complete the questionnaires at that time.

The participating subjects were evaluated in the Sjögren's Research Clinic at Oklahoma Medical Research Foundation or at a similar clinic at the University of Minnesota. Participants were self or physician-referred. Each potential clinic participant was interviewed by phone by trained personnel who assessed the presence of ocular and oral symptoms by asking the six standardized and validated [17 (link)] questions in the subjective criteria of the revised AECG Classification Criteria (Table 1).[19 (link)] In order to be eligible for an appointment at the clinic, at least one ocular and one oral question had to be answered affirmatively. The exclusion criteria for evaluation at the clinic were also based on the recommendations of the AECG (Table 1).[19 (link)] In addition, we excluded individuals that presented with known current pregnancy or inability to provide informed consent.
With very few exceptions, participants were evaluated in a single morning clinic visit using standardized protocols. Patients underwent an oral exam consisting of measurement of stimulated and timed whole unstimulated salivary flow (WUSF), a lip biopsy and collection and storage of saliva. Participant evaluation did not include sialography or scintigraphy. The ocular specialist performed ocular surface staining with lissamine green and fluorescein, a unanesthesized Schirmer's I test, and collection and storage of tears. The ocular vital dye score was determined using the quantitative dot-counting method [23 ] rather than by descriptive features [24 (link)] and the score for each section was recorded independently before generating a final score for each eye. Blood samples were collected for general laboratory tests and extraction of DNA, RNA, and serum. A physician completed a detailed history and physical examination, including general medical, rheumatological and neurological evaluations. If patients gave a history of a past diagnosis of rheumatoid arthritis, mixed connective tissue disease, systemic sclerosis, myositis, primary biliary cirrhosis, multiple sclerosis, or systemic lupus erythematosus, classification criteria for these illnesses were specifically ascertained by history, medical record review and testing for the corresponding autoantibodies.
All procedures were approved by the Oklahoma Medical Research Foundation and University of Minnesota Institutional Review Boards. Each participant provided written informed consent prior to entering the study.