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Biomedical subjects

Arthur Bookman

Publications and source records attributed to Arthur Bookman.

4 recordsLinked to original sources

Office capillaroscopy in systemic sclerosis.

The aims of this study are to assess the reliability of two office techniques, the ophthalmoscope and the Dermlite dermatoscope, and to detect nailfold capillaroscopy abnormalities in systemic sclerosis (SSc). Two separate studies were performed. In the first, the nailfolds of two fingers on one hand of 13 SSc patients and two normals were examined by four rheumatologists using an ophthalmoscope. In the second, the nailfolds of the two fingers of each hand of six SSc patients and two normals were examined by six rheumatologists with a Dermlite dermatoscope. Widefield capillary microscopy was performed by one observer in the ophthalmoscope study to assess validity. The examiners determined the presence or absence of dilated loops, giant capillary loops, and/or avascular areas on each digit. The kappa coefficient was calculated to demonstrate agreement. With the ophtalmoscope, the inter-observer kappa coefficients were 0.43, 0.54, and 0.19; the average intra-observer agreements were 0.61, 0.56, and 0.31; and the ophthalmoscope-microscope agreement were 0.63, 0.52, and <0.1 for dilated capillaries, giant capillaries, and avascular areas, respectively. With the dermatoscope, the kappa values for inter-observer reliability were 0.63, 0.40, and 0.20; and intra-observer reliability was 0.71, 0.55, and 0.40 for dilated capillaries, giant capillaries, and avascular areas, respectively. The ophthalmoscope and the dermatoscope provide moderate to substantial reliability to detect the presence of giant and dilated capillaries but poor inter-observer agreement for avascular areas. The ophthalmoscope is valid when compared to the microscope for detecting giant or dilated capillaries. We conclude that these techniques are useful office tools to detect capillary abnormalities in SSc.

Dermoscopy↗

Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis.

OBJECTIVE: To estimate the prevalence of elevated pulmonary arterial pressures (PAP) as a correlate for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) in rheumatology centers in Canada. METHODS: During the one-year study period (June 2002-May 2003), charts of patients with SSc were reviewed to determine demographics, SSc characteristics, percentage of patients with a PAH diagnosis, and the site criteria for such diagnosis. Subjects with no PAH that had symptoms of dyspnea and/or fatigue were invited to undergo Doppler echocardiography to estimate their systolic PAP (sPAP). RESULTS: A total of 539 patients with SSc (age 56 yrs +/- 13 SD, 84% female, 41% with diffuse SSc, 58% limited SSc, SSc disease duration 9 yrs +/- 7 SD) at 8 rheumatology centers were reviewed. Twenty-three percent of patients were diagnosed with elevated sPAP based on the site diagnosis criteria (i.e., > 30 mm Hg or > 35 mm Hg). From the non-PAH, not recently screened patients that had symptoms of dyspnea or fatigue, a total of 89 patients underwent a Doppler echocardiograph; 40% had sPAP > 35 mm Hg. CONCLUSION: Elevated PAP are common in both limited and diffuse SSc disease, occurring in 21% of limited and 26% of diffuse SSc patients. During the screening most patients had mild PAP elevations that would require further assessments such as right heart catheterization to diagnose PAH where appropriate. A high index of suspicion is important and routine echocardiography in symptomatic patients may allow earlier diagnosis of PAH and intervention. The proportion of SSc patients with mild to moderate elevations of PAP who will develop significant PAH is unknown and longterm studies are needed to address the natural progression.

Adolescent↗

Correlation between corneal sensitivity, subjective dry eye symptoms and corneal staining in Sjögren's syndrome.

BACKGROUND: Previous studies have shown a poor correlation between dry eye symptoms and objective clinical signs in patients with Sjögren's syndrome. We examined the hypothesis that reduced corneal sensitivity is associated with increased ocular surface disease and reduced symptoms in patients with Sjögren's syndrome. METHODS: Eighteen subjects with a diagnosis of Sjögren's syndrome attending a Sjögren's clinic participated in the study. All participants completed the Ocular Surface Disease Index (OSDI) and the Symptom Severity of Discomfort (SSD) scale and answered a question regarding overall severity of dry eye symptoms. The subjects underwent measurement of best-corrected Snellen visual acuity, corneal sensitivity testing with the Cochet-Bonnet esthesiometer, fluorescein and lissamine green staining of the cornea, Schirmer's test I and determination of the tear film break-up time. The results were analysed using Pearson correlational analysis. RESULTS: Both fluorescein and lissamine green staining of the cornea correlated negatively with central corneal sensation (r = -0.3542, p = 0.034, and r = -0.3748, p = 0.029 respectively), indicating that corneal sensation was reduced with increased ocular surface disease. The overall symptom severity correlated negatively with lissamine green staining of the cornea (r = -0.4310, p = 0.011), suggesting reduced symptoms with increased corneal disease. INTERPRETATION: Reduced corneal sensation correlated with increased ocular surface disease. Ocular surface disease similarly demonstrated a reciprocal relation with patients' dry eye symptoms. Consequently, we found that patients with Sjogren's syndrome with advanced corneal staining tended to have fewer dry eye symptoms than patients with less corneal staining.

Adult↗

Primary Sjögren's syndrome and deficiency of ICA69.

BACKGROUND: Sjögren's syndrome is a common (about 1% of the population) autoimmune disease of salivary and lacrimal glands. Its cause and pathogenesis are poorly understood, and treatments are mostly for symptoms of the disease. ICA69 is a self-antigen expressed in brain, pancreas, salivary, and lacrimal glands. NOD-strain mice are an animal model of spontaneous Sjögren's syndrome. We aimed to assess the role of ICA69 in autoimmunity against Sjögren's syndrome. METHODS: We inactivated the genomic ICA69 locus, generated NOD congenic mice that were deficient in ICA69, and assessed development of Sjögren's syndrome. ICA69 autoimmunity was investigated in controls and in patients with primary Sjögren's syndrome or systemic lupus erythematosus, and in various NOD mice, some of which were given an ICA69-directed prototype peptide vaccine. FINDINGS: Disruption of the ICA69 locus prevented lacrimal gland disease and greatly reduced salivary gland disease in NOD mice. In healthy NOD mice, ICA69-specific T cells accumulated in lymph nodes that drain salivary tissue. T-cell and B-cell autoreactivity against ICA69 was much the same in patients with primary Sjögren's syndrome, but not in those with systemic lupus erythematosus or in healthy controls. Immunotherapy with a high-affinity mimicry peptide targeting ICA69-specific T-cells reduced established Sjögren's syndrome in wild-type NOD mice in the long term. INTERPRETATION: ICA69 is a new autoantigen in primary Sjögren's syndrome that has an important role in progression of disease and could be of diagnostic value. Immunotherapy of primary Sjögren's syndrome is promising, since autoimmunity in NOD mice with Sjögren's syndrome seems to be uniquely susceptible to such treatment even late in disease.

Adult↗