Penelope Shirlaw

American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

We propose new classification criteria for Sjögrens syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS.

Differential Expression of Candida albicans Secreted Aspartyl Proteinase and Phospholipase B Genes in Humans Correlates with Active Oral and Vaginal Infections

The in vivo expression of Candida albicans secreted aspartyl proteinase (SAP1-SAP8) and phospholipase B (PLB1 and PLB2) genes was analyzed in 137 human subjects with oral and vaginal candidiasis or carriage. Total RNA was isolated from whole unstimulated saliva or vaginal swabs, and the expression of SAP1-8 and PLB1-2 was evaluated by reverse-transcriptase polymerase chain reaction using specific primer sets. A spectrum of SAP gene expression profiles was obtained from different C. albicans strains during symptomatic disease and asymptomatic carriage. SAP2 and SAP5 were the most common genes expressed during both infection and carriage. SAP1, SAP3, SAP4, SAP7, SAP8, and PLB1 expression was correlated with oral disease, whereas SAP1, SAP3, and SAP6-SAP8 expression was correlated with vaginal disease. Furthermore, SAP1, SAP3, and SAP8 were preferentially expressed in vaginal, rather than oral, infections. This study demonstrates the differential expression of the hydrolytic enzyme genes in humans and correlates the expression of specific Candida species virulence genes with active disease and anatomical location.

Oro-facial granulomatosis: Crohn's disease or a new inflammatory bowel disease?

Background: Oro‐facial granulomatosis (OFG) is a rare chronic inflammatory disorder presenting characteristically with lip swelling but also affecting gingivae, buccal mucosa, floor of mouth, and a number of other sites in the oral cavity. Histologically, OFG resembles Crohns disease (CD), and a number of patients with CD have oral involvement identical to OFG. However, the exact relationship between OFG and CD remains unknown. Methods: Thirty‐five patients with OFG and no gut symptoms were identified from a combined oral medicine/gastroenterology clinic. All underwent a standardized assessment of the oral cavity and oral mucosal biopsy to characterize the number of sites affected and the type of inflammation involved. Hematological and biochemical parameters were also recorded. All 35 patients underwent ileocolonoscopy and biopsy to assess the presence of coexistent intestinal inflammation. Results: Ileal or colonic abnormalities were detected in 19/35 (54%) cases. From gut biopsies, granulomas were present in 13/19 cases (64%). An intestinal abnormality was significantly more likely if the age of OFG onset was less than 30 years (P = 0.01). Those with more severe oral inflammation were also more likely to have intestinal inflammation (P = 0.025), and there was also a correlation between the histologic severity of oral inflammation and the histologic severity of gut inflammation (P = 0.047). No relationship was found between any blood parameter and intestinal involvement. Conclusions: Endoscopic and histologic intestinal abnormalities are common in patients with OFG with no gastrointestinal symptoms. Younger patients with OFG are more likely to have concomitant intestinal involvement. In these patients, granulomas are more frequent in endoscopic biopsies than reported in patients with documented CD. OFG with associated intestinal inflammation may represent a separate entity in which granulomatous inflammation occurs throughout the gastrointestinal tract in response to an unknown antigen or antigens.

Distinguishing orofacial granulomatosis from crohn's disease: Two separate disease entities?

Background: Orofacial granulomatosis (OFG) is a rare chronic inflammatory disease of unknown etiology sharing histological features with Crohns disease (CD). This study aimed to 1) define the clinical presentation of OFG, 2) establish differentiating features for those with CD, 3) examine if onset of OFG is predictive of CD, and 4) establish differentiating features for children. Methods: Data were extracted from medical notes (n = 207) for demographics, clinical features, blood parameters, diagnosis of CD, and treatments for patients with OFG. Results: Ninety‐seven patients (47%) were female. The lips (184/203; 91%) and buccal mucosa (151/203; 74%) were mainly affected. Forty‐six (22%) had intestinal CD. Ulcers (24/46; 46% versus 29/159; 15%, P = <0.001) were more common in patients with CD as was a raised C‐reactive protein (24/33; 73% versus 60/122; 49%, P = 0.016) and abnormal full blood count (19/41; 46% versus 35/150; 23%). The buccal‐sulcus (12/44; 27% versus 20/158; 13%, P = 0.019) was more often affected in those with CD. Half the patients with CD were diagnosed prior to onset of OFG. The remainder were diagnosed after. The incidence of CD is similar for children (16/69; 23%) and adults (29/132; 22%), although oral onset in childhood is more likely to occur prior to diagnosis of CD. Conclusions: OFG mainly presents in young adults with lip and buccal involvement. Abnormalities in inflammatory markers, hematology and oral features of ulceration, and buccal‐sulcal involvement are factors more commonly associated with CD. Initial presentation of OFG does not necessarily predict development of CD, although this is more likely in childhood. (Inflamm Bowel Dis 2011;)

Probiotic and prebiotic use in patients with inflammatory bowel disease: A case‐control study

Background: The use of complementary and alternative medicine in inflammatory bowel disease (IBD) has been extensively studied. However, the use of probiotics and prebiotics is poorly documented, despite evidence of efficacy of particular probiotic strains in specific forms of IBD. Methods: A case‐control study comprising interviewer‐administered questionnaires was conducted in IBD patients and healthy controls. Data regarding use and knowledge of probiotics and prebiotics, demographic, and clinical information were collected. Results: In total, 334 participants (234 IBD, 100 controls) were interviewed. Significantly more IBD patients than controls had ever used probiotics to manage their health (Crohns disease [CD] 43%, ulcerative colitis [UC] 51%, controls 21%, P < 0.001). Prebiotic use was negligible. On logistic regression analysis, having UC (odds ratio [OR] 4.30, 95% confidence interval [CI] 2.27–8.12) or CD (OR 3.05, 95% CI 1.66–5.60) were the strongest predictors of probiotic use. Within IBD patients the strongest predictor of probiotic use was current steroid use (OR 2.4, 95% CI 1.11–5.18). IBD patients had greater probiotic knowledge scores than controls (P = 0.003), although 20% of IBD probiotic users could not provide a definition of a probiotic. Less than half of IBD probiotic users discussed probiotic use with healthcare professionals, with commercial advertising being the primary source of information. Conclusions: Patients with IBD use probiotics to manage their health but frequently choose strains without evidence of efficacy in IBD. Patients rely on nonclinical sources of information and often do not disclose probiotic use to healthcare professionals. Conventional healthcare providers should inquire about probiotic use among their patients and offer evidence‐based advice. (Inflamm Bowel Dis 2010)

The transition of pemphigus vulgaris into pemphigus foliaceus: a reflection of changing desmoglein 1 and 3 autoantibody levels in pemphigus vulgaris.

Summary The transition of pemphigus vulgaris (PV) into pemphigus foliaceus (PF) is rare and the immunological changes underlying this event are not well understood. We report a 44‐year‐old woman who presented with oral and cutaneous erosions typical of PV. Over a 9‐year period, the clinical features evolved into those of PF. To examine whether quantitative changes in desmoglein (Dsg) antibodies were associated with this transition, Dsg1 and Dsg3 antibody levels were measured by enzyme‐linked immunosorbent assay in 82 sequential serum samples collected over this period. At presentation, when the phenotype was PV with oral and cutaneous erosions, antibodies to both Dsg1 and Dsg3 were detected. The disappearance of oral involvement was associated with a decline in Dsg3 antibodies, which are now undetectable, while the development of more severe skin involvement was associated with rising Dsg1 antibody levels. These data strongly suggest that the change in clinical features is a reflection of qualitative and quantitative changes in antibody profile. It is not known whether the transition to PF is permanent or whether disease relapses in the future may be associated with the re‐emergence of Dsg3 antibodies, oral ulceration and a PV phenotype.

Immunology of Diseases of the Oral Cavity

Abstract The oral cavity is a complex immunological site. Both mucosal immunity and systemic immunity merge and play significant roles in protection against infections and damage, and also in the pathogenesis of many oral mucosal diseases. Some protective factors in saliva are shared with other mucosal secretions, and stimulation of GALT may lead to similar responses in oral and vaginal sites. The presence of teeth is unique among mucosae, with associated unique epithelia and the main oral-specific diseases of periodontal diseases and dental caries. Crevicular fluid gives access to the oral cavity of systemic serological immunity. The range of epithelial phenotypes and their diseases reflects gastrointestinal (e.g., Crohn’s), dermatological (e.g., pemphigus), and mucosal diseases (e.g., recurrent aphthous stomatitis and Behcet’s syndrome), as well as autoimmune diseases such as Sjogren’s syndrome or immunodeficiency as in HIV. Viral (e.g., herpes simplex), bacterial (e.g., caries), or fungal (e.g., candidiasis) infections and the associated immunological responses can be specific to the oral cavity or be similar to those of other mucosae.

Age, gender and disease-related platelet and neutrophil activation ex vivo in whole blood samples from patients with Behçet’s disease

OBJECTIVES Behçets disease (BD) is more severe among young males and disease severity decreases with age. Therefore, the effect of disease activity, gender and age on platelet and neutrophil activation in whole blood taken from patients with BD was investigated. METHODS Using an anti-coagulant Tripotassium ethylenediaminetetra acetic acid (K3EDTA) plus citrate-theophylline-adenosine-dipyridamole (CTAD) (K3EDTA/CTAD) that preserves the degree of platelet activation that exists in vivo, we assessed neutrophil and platelet activation, microparticles, and monocyte and neutrophil-platelet aggregate formation in 43 BD patients using flow cytometry. This is the first description of platelet activation and microparticles in BD patients using this methodology. RESULTS Inactive [2.78 (0.56)%, P = 0.0009; 3.11 (0.78)%, P < 0.0001] and active [2.28 (0.84)%, P < 0.0001; 3.071 (0.67)%, P = 0.0031] BD patients had significantly higher percentages of CD62P-expressing platelets and CD62P+ platelet microparticles as compared with healthy controls (HCs) [0.84 (0.1)% and 1.23 (0.14)%], respectively. The percentages of CD62P+ platelets and CD62P+ platelet microparticles in female and male BD patients were also significantly higher than those expressed by female and male HCs. The percentages of CD62P+ microparticles were significantly increased in the 20-30-(P = 0.0301) and 31-50-(P < 0.0162) year age ranges, but not in the >50-year age group of BD patients. CONCLUSION BD is a rare, chronic multi-systemic vasculitis and interaction of activated platelets with leucocytes has been linked to pathological disorders associated with vascular inflammation. Importantly, this study demonstrates that platelet microparticle activation is increased in BD. Also, this is the first report in which changes in platelet activation in BD are concordant with the observations that BD disease activity diminishes with age.

An international survey of oral medicine practice: proceedings from the 5th World Workshop in Oral Medicine.

OBJECTIVES (i) To define the current state of oral medicine clinical practice internationally, and (ii) to make recommendations for future modeling of the practice of oral medicine. MATERIALS AND METHODS A survey was designed by an international panel of oral medicine experts to assess the current state of oral medicine practice internationally. The survey was sent to oral medicine experts across the world, and responses were electronically stored and analyzed using descriptive statistics. RESULTS Two hundred respondents completed the survey representing 40 countries from six continents. The two most common settings for an oral medicine practice were in a hospital and a dental school. More than 88% of respondents considered management of oral mucosal disease, salivary dysfunction, oral manifestations of systemic diseases, and facial pain in the definition of oral medicine. CONCLUSIONS (i) Oral medicine clinicians diagnose and manage a wide variety of orofacial conditions; (ii) There are significant differences in the definition of oral medicine clinical practice from country to country; (iii) India has the largest expansion of oral medicine services as defined by escalating numbers of clinicians within the specialty as compared with other countries; (iv) oral medicine practitioners have a wide range of professional responsibilities.

Congenital heart block associated with Sjögren syndrome: case report

Background Congenital heart block is a rare complication of pregnancy associated with Sjögren Syndrome that may result in the death of the foetus or infant, or the need for pacing in the newborn or at a later stage. Case report The case is presented of a 64-year-old patient with primary Sjögren Syndrome and a history of having given birth to two sons with congenital heart block, both of whom required pacing several years later. Conclusion The literature relating to this association is discussed including the suggested mechanism, long-term outcome of mothers of children with congenital heart block and preventive treatment strategies.