Beyond the symptoms: Personalizing giant cell arteritis care through multidimensional patient reported outcome measure.

BACKGROUND: Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50. Published research highlighted the lack of a disease-specific patient reported outcomes (PROMs) for GCA. OBJECTIVES: To assess the validity, reliability and responsiveness to change of a devised disease specific patient self-reported outcome measures questionnaire for Giant Cell Arteritis (GCA). METHODS: The GCA-PROMs was conceptualized based on frameworks outlined in the OMERACT developed core set of Outcome Measures for Large-Vessel Vasculitis and the guiding principles of the FDA guidance. Initially, cognitive interviews were conducted to identify item pool of questions. Item selection and reduction was achieved based on patients as well as an interdisciplinary group of specialists. Rasch and internal consistency reliability analyses were implemented. RESULTS: A total of 54 GCA patients completed the questionnaire. The GCA-PROMs questionnaire was reliable as demonstrated by a high standardized alpha (0.878-0.983). Content construct assessment of the GCA-PROMs functional disability and QoL revealed significant correlation (p< 0.01) with both HAQ and EQ-5D. Changes in functional disability, QoL showed significant (p< 0.01) variation with diseases activity status in response to therapy. CONCLUSIONS: The developed GCA-PROMs questionnaire is a reliable and valid instrument for assessment of GCA patients. A stratified treatment regimen depending on the individual patient's risk factors as well as preferences and associated comorbidities is the best approach to tailored patient management.

Toward electronic health recording: evaluation of electronic patient reported outcome measures (e-PROMs) system for remote monitoring of early systemic lupus patients.

The study aimed to assess the value of evaluation of electronic patient reported outcome measures (e-PROMs) in the assessment and management of SLE disease activity flares, its association with adherence to therapy as well as organ damage. A randomized, controlled crossover study was carried out over a 24-month duration. One hundred forty-seven SLE patients meeting the revised American College of Rheumatology (ACR) criteria were enrolled. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was used to assess disease activity, whereas organ damage was scored using the Systemic Lupus International Collaborating Clinics (SLICC)/ACR Damage Index. In the first 12 months, the patients were assessed every 3 months. At 12 months, the patients were randomized into a cohort of 73 patients who continued their care in the same style and 74 patients who completed an online e-PROMs questionnaire on monthly basis for another 12-month period. The data captured were then retrospectively analyzed at the end of the 24-month study period. At the end of the first year of the study, the mean SLEDAI and SDI scores were 8.72 (6.1) and 1.9 (2.2). At the end of the second year, the mean SLEDAI and SDI scores in the e-PROMs cohort were 3.1 (2.6) and 1.2 (1.3), whereas in the control group, the scores were 7.63 (6.7) and 1.8 (2.3), respectively (p < 0.01). Adjusting for possible confounding variables, the number of flares, regardless of their severity, was associated with damage accrual (OR 2.03, 95% CI 1.34 to 2.83, p < 0.001). Adherence to therapy was significantly (p < 0.1) higher in the e-PROMs group. e-PROMs was equivalent to PROMs paper format and has a potential disease-modifying effect as it facilitated close monitoring of disease activity with an option of management escalation whenever indicated.

Diagnostic value of FASH ultrasound and chest X-ray in HIV-co-infected patients with abdominal tuberculosis.

In human immunodeficiency virus (HIV) co-infected tuberculosis (TB) patients with negative acid-fast bacilli smears, chest radiography (CXR) is usually the first imaging step in the diagnostic work-up. Ultrasound, also in the form of focused assessment with sonography for TB-HIV (FASH), is an additional imaging modality used to diagnose extra-pulmonary TB (EPTB). Findings from 82 patients with abdominal TB diagnosed by ultrasound were analysed and compared with CXR results. Enlarged abdominal lymph nodes were seen in 75.6% of the patients, spleen abscesses in 41.2% and liver lesions in 30.6%. CXR showed a miliary pattern in 21.9% of the patients; 26.8% of the CXR had no radiological changes suggestive of pulmonary TB. This patient group would benefit from ultrasound in diagnostic algorithms for HIV-associated EPTB.

Systemic lupus erythematosus in Saudi Arabia: morbidity and mortality in a multiethnic population.

The objective of this study is to summarize the features of patients with Lupus erythematosus in Saudi Arabia. Racial differences of patients and predictors of mortality are assessed. Ninety-three patients treated for SLE at the University Hospital in Jeddah were reviewed. Frequencies of clinical manifestations, causes of admission and causes of death were analysed. Variables predicting mortality were assessed by logistic regression and survival probabilities were estimated by the Kaplan-Meier method. The most frequent presenting symptoms were arthritis (68%) and fever (58%). Renal involvement was seen in 61% of patients. The majority of patients (61%) showed ANA titers higher than 1:1280. C4 levels were significantly lower in patients who died during the observation period than in survivors. The overall five-year survival rate was 92%. Variables predicting early death (<2 years after diagnosis) were young age at diagnosis, male sex and skin involvement. Death after more than two years correlated with older age at diagnosis and renal involvement. Patients of African descent had higher rates of neurological involvement and renal failure. The mortality in this group was highest, though this was not statistically significant. The overall survival in our cohort compares with mortality rates reported from western countries. However, renal disease tends to be common and has a severe prognosis, and thus merits additional attention.

Utilisation and costs of investigations, and accuracy of diagnosis of polymyalgia rheumatica by family physicians.

To determine the utilisation and costs of investigations, and the accuracy of polymyalgia rheumatica (PMR) by family physicians, a retrospective chart review of 123 patients referred to a tertiary care rheumatology clinic was undertaken. The accuracy of diagnosis of PMR in the cohort was 24%. A variable number of investigations and costs occurred prior to referral, ranging from $110 in those with an unspecified locomotor diagnosis, through $74 in those with a correct diagnosis of PMR, to $24 where no diagnosis was entertained. In general, these costs were considered higher than necessary to make the diagnosis compared to those advocated by rheumatologists. We conclude that there is a low accuracy of diagnosis of PMR and an overutilisation of investigations, with resultant increased costs, by family physicians referring patients with PMR. Appropriate educational interventions are required to address both diagnostic and investigational needs.

Clinical outcome of 149 patients with polymyalgia rheumatica and giant cell arteritis.

OBJECTIVE: To assess the clinical outcome of patients with polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). METHODS: All charts of consecutive patients with a diagnosis of PMR and/or GCA attending a tertiary referral center from June 1989 to February 1996 were reviewed following a predetermined protocol. Subsequently, the majority of patients (90%) were assessed clinically or by telephone interview. Registered variables included demographic data, disease characteristics, prednisone dosage and duration, comorbidities, and clinical outcomes. RESULTS: There were 149 patients (133 with PMR alone, 7 with GCA alone, 9 with both); 94 (63%) were females; the mean age was 68 +/- 9 years, and the mean disease duration from the first symptom to the rheumatology consultation was 13 +/- 12 weeks (1-99). Typical clinical features of PMR were present in patients with PMR. Synovitis was observed in 26 patients. The presenting symptoms for GCA were typical features in 13 patients and blindness in 3 (2%) patients. Mean followup was 3.7 +/- 2 years. Comorbid conditions were present in 71 patients: 12 patients had hypertension, 13 had fractures, 8 diabetes, 29 cataract, 8 major infection, and 37 had other complications. Cancer was diagnosed in 4 patients and 6 patients had died. Prednisone was prescribed in 148 patients (mean dose 23 +/- 14 mg) for a mean time of 28 +/- 29 mo. Nonsteroidal antiinflammatory drugs were prescribed in 51 (34%) patients and methotrexate in 2. Disease remission was achieved in 81 (54%) patients (72 remissions, 9 presumed remissions) in whom steroid therapy had been stopped. Another 54 (36%) patients were still taking prednisone at the time of the interview, all were in clinical remission. Seventeen patients developed rheumatoid arthritis subsequent to the diagnosis of PMR. CONCLUSION: PMR and GCA should not necessarily be considered diseases with favorable outcome. In many of our patients, steroids were required for a prolonged period. Some patients developed significant complications attributable to steroid therapy. A significant number of patients progressed to rheumatoid arthritis.

Rheumatic features of gastrointestinal tract, hepatic, and pancreatic diseases.

Rheumatic manifestations of diseases primarily of the gastrointestinal tract, liver, or pancreas are frequent and vary from soft tissue rheumatism and carpal tunnel syndrome to Raynaud's phenomenon, osteoporosis, arthritis, and vasculitis. Some patients with celiac disease develop osteoporosis and arthritis responsive to a gluten-free diet. It seems that osteoporosis is a frequent feature in patients with inflammatory bowel disease, and steroid use is an important risk factor. The use of steroids in patients with Crohn's disease and ulcerative colitis has generated medicolegal questions. This year a wealth of information has been published regarding hepatitis C virus infection. Arthritis, myopathy, and antibody production are some of the reported manifestations of chronic hepatitis C virus infection. There is evidence that may link chronic hepatitis C virus infection with the antiphospholipid syndrome.