Dermatitis Herpetiformis Research Articles

Association between vulvar lichen sclerosus and celiac disease in woman.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition involving mainly the genital area with an undetermined aetiology. Recent studies show that in up to 34% of cases in adult women, VLS coexists with allergies or autoimmune diseases like celiac disease (CD), among others. However, literature data relating strictly to the co-occurrence of celiac disease and Duhring's disease (DH) in patients with VLS are very limited. In our study, we sought to clarify the possible relationship between vulvar lichen sclerosus in adult women and celiac disease in its cutaneous form. The aim of the study was to demonstrate the presence of celiac disease-specific antibodies in women with VLS. The control group consisted of 41 healthy women, and the study group consisted of 50 women aged 24-83 years with diagnosed vulvar lichen sclerosus who were hospitalized in the Department of Gynaecology, Obstetrics and Gynaecologic Oncology of the Bonifraters Medical Centre in Katowice. There were no significant differences in blood serum levels of CD-specific antibodies between both groups. The study conducted did not confirm the association between vulvar lichen sclerosus and celiac disease or Duhring's disease. The main limitation of the research was the small size of the study and control groups. Further studies on a larger group of patients are needed. They could clarify the possible mechanisms behind the co-occurrence of these two conditions. Earlier diagnostic will help prevent the development of severe and irreversible complications.

Inflammatory Myopathies and Autoimmune Gluten-related Disorders: A Scoping Review of Pathophysiological Interconnections and Hypothesis

Introduction: Anecdotal reports describe patients with concurrent idiopathic inflammatory myopathy (IIM) and celiac disease (CeD) in whom the introduction of a gluten-free diet led to dramatic improvement of myositis. We first systematically reviewed all peer-reviewed publications on concomitant IIM and duodenal biopsy-verified CeD. The collected evidence was suggestive of associations between myositis disease activity and gluten exposure in some patients with IIM-CeD. Objective/Methods: To investigate possible explanations for the observations, an exploratory review of basic pathophysiological relationships between IIM and gluten-related disorders was performed using a combined strategy of systematic and non-systematic literature searches and forward and backward citation tracking. Results: The investigations revealed close pathophysiological associations between IIM and the autoimmune gluten-related disorders CeD, dermatitis herpetiformis, and gluten ataxia. Common traits include shared genetic predisposition through HLA-DQ2.5/-DQ8, disease activity-associated autoantibodies, histopathological parallels with inflammatory cell infiltrates, and similarly distributed structural homologous transglutaminases (TGs). HLA-DQ2.5-restricted gluten-specific CD4+ T cells of a rare, uniform phenotype are reported in CeD and connective tissue disease. Expanded T-cell clones with identical phenotypes and CDR3β motifs indicate the presence of a continuous, antigen-driven T-cell response. Conclusion: The investigations revealed that the main components involved in the adaptive immune response in the CeD gut may be present in HLA-DQ2.5+/-DQ8+ IIM muscle. The collected evidence supports the notion that in some genetically predisposed patients with IIM, gluten may act as an exogenous antigen driving myositis. Further Research/Clinical Implications: To test the above hypothesis, clinical trials combined with immunological studies are needed. Meanwhile, the inclusion of HLA-DQ typing may be justified, and subsequent small-intestinal biopsies in HLA-DQ2.5/8+ individuals with IIM.

Not All Antihistamines Are Created Equal: Alimemazine As A Cause Of Drug Induced Parkinsonism

Abstract Background A 67year old female presented to a Movement Disorder Service for assessment of tremor. She had a history of presbycusis, dermatitis herpetiformis, bronchiectasis, osteoporosis and stage A chronic lymphocytic leukaemia. History elucidated hypophonia, somniloquy, REM-sleep behaviour disorder, bruxism, presence hallucinosis, insidious bradykinesia and of titubation of increasing prevalence and amplitude over 20years. Examination demonstrated cognitive impairment (Montreal Cognitive Assessment 12+1/30) in the domains of visuospatial function, executive thinking, naming, attention, language, abstraction and anterograde recall. Luria test was 0/6. Also found was: micrographia, left exotropia, reduced left arm and leg swing, kinetic tremor asymmetry, decrement of amplitude and frequency of rapid repetitive movements asymmetrically, mandibular and lingual tremor, positive Kinnier-Wilson test and orthostatic deterioration in yes-yes titubation. Drg history identified use of Alimemazine 10mg for insomnia for approximately 20years. Methods Modified Simpson Angus Scale score was 6 indicating a clinically significant degree of extrapyramidal side effects. Unified Parkinson’s Disease Rating Scale part III was 17. Results Functional dopamine uptake imaging did not indicate a parkinsonian syndrome. Drg induced parkinsonism was diagnosed. 0.0097% of the Northern Ireland population were prescribed Alimemazine in 2022 for non-dermatological reasons, representing 73.49% of all dispensed preparations. In the first financial quarter of 2024 in Northern Ireland, all dispensed prescriptions cost £218,395. Conclusion Not all antihistamines are created equal. Alimemazine is an older phenothiazine derivative antihistamine with central sedative effects equivalent to Chlorpromazine but without anti-adrenaline action. It is rarely prescribed and easily overlooked as “just another antihistamine”. It is commonly prescribed by dermatologists for urticaria. It has substantial pharmacoeconomic implications given its cost disparity with other antihistamines. Extrapyramidal features of tremor, akinesia and hypertonicity are more common in older people; usually developing within weeks or months of commencement. Stopping the drug is the first-line intervention. Resolution of symptoms is not guaranteed.

Beyond the Gut: Exploring Cardiovascular Implications of Celiac Disease.

Celiac disease (CD) is an autoimmune disorder that presents with gastrointestinal symptoms including diarrhea, weight loss, and abdominal bloating due to the inflammation in the small intestine. It has been associated with various extraintestinal manifestations, including mucocutaneous findings such as dermatitis herpetiformis, anemia, dental enamel defects, osteoporosis, and arthritis. Studies have revealed an increasing association between CD and cardiovascular diseases (CVDs), including atherosclerosis, cardiomyopathy, and arrhythmia. Chronic inflammation, nutritional deficiencies from malabsorption, endothelial dysfunction, thrombophilic autoantibodies, thrombocytosis, and protein C and S deficiency have been proposed as the probable mechanisms for the association between the 2 conditions. This article aims to provide a review of the pathophysiological mechanism of celiac disease causing various CVDs and to compare and contrast the existing studies suggesting both favorable and unfavorable CVD outcomes in patients with CD.

Price, quality, and availability of gluten-free products in Australia and New Zealand – a cross-sectional study

ABSTRACT Adhering to a gluten-free diet (GFD) is essential for individuals with gluten-related disorders such as coeliac disease, dermatitis herpetiformis, gluten ataxia, and wheat allergy. Approximately 1%-2% of the population is affected by these conditions. Access to affordable, high-quality gluten-free products (GFPs) is crucial for maintaining dietary compliance and well-being. This study explores the experiences of those on a GFD, focusing on the availability, quality, and cost of GFPs compared to non-GFPs. The study was conducted in two stages: (1) An online survey was distributed to Australian Coeliac Association and Coeliac New Zealand members to gather data on shopping habits, preferred GFPs, and opinions on product availability, value, and quality. (2) Retail observations involved creating a `shopping basket/list' based on survey results to compare the availability and price of GFPs versus non-GFPs in major supermarkets across urban and rural areas. Results from 1501 Australian and 700 New Zealand respondents and retail observations revealed that GFPs are significantly more expensive and of lower quality than non-GFPs. Commonly purchased items include bread, pasta, crackers, flour, and breakfast cereals. The study highlights the need for improved availability, affordability, and quality of GFPs, especially in bridging the cost disparity between urban and rural areas.

Dapsone Use in Dermatology.

Dapsone, initially synthesized for textile dyeing, gained recognition in the 1930s for its antibacterial properties, leading to its utilization in dermatology for leprosy and dermatitis herpetiformis. Despite US Food and Drug Administration (FDA) approval for these conditions, dapsone's off-label uses have expanded, making it a valuable option in various dermatologic conditions. This review seeks to highlight the common uses of dapsone in its FDA indications and off-label indications. Diseases in which dapsone is considered first-line therapy or adjunctive therapy are reviewed, with highlights from the resources included. An overview of dapsone's pharmacokinetics, pharmacodynamics, indications, dosages, and safety profile are also reviewed. Dapsone's versatility and safety profile make it a cost-effective treatment option in dermatology, particularly for patients with limited access to specialized medications. Ongoing clinical trials are also described exploring dapsone's efficacy in novel dermatologic uses. Dapsone has been a valuable adjunctive therapy across various dermatologic conditions for years and evidence for its use continues to expand.

Celiac Disease: A Forty-Year Analysis in an Italian Referral Center.

Celiac disease (CD) is an autoimmune disorder triggered by gluten ingestion. Herein, we assessed clinical, serological and histopathological findings of a single-center, large cohort of CD patients diagnosed and followed-up over forty years. From January 1980 to December 2020, 1547 CD patients (1170 females; age range: 8-81 years; F:M ratio = 3.1:1) were diagnosed in an Italian tertiary referral center. Comorbidities and complications were recorded at diagnosis and during follow-up. CD diagnoses quadrupled after 2000. The most frequent phenotype was the non-classical CD (63.3%), and the most prevalent histotype was Marsh 3C (44.7%). Gastrointestinal manifestations, detectable in 51% of patients, were diarrhea (24.3%), bloating (28%) and aphthous stomatitis (19.7%). The most common CD-associated disorder was osteopenia (59.9%), predominant in females (64.3%); extraintestinal manifestations included anemia (35.8% iron-deficiency; 87% folic acid malabsorption), cryptogenic hypertransaminasemia (27.9%), and recurrent miscarriages (11.5%). Thyroiditis (26.9%), type 1 diabetes mellitus (2.9%), and dermatitis herpetiformis (1.4%) were the most common CD-related autoimmune disorders. Six patients had inflammatory bowel disease. Complications and mortality rate occurred in 1.8% and 1.9%, respectively. This single-center, large cohort analysis confirmed that CD presentation changed over the years, with an increase of non-classical and subclinical clinical phenotypes.

Impact of moderate wine consumption on immune-mediated diseases

This review critically assesses scientific literature in order to determine whether alcoholic beverages can positively or negatively affect the incidence and severity of immune-mediated disorders, including autoimmune diseases. Few scientific studies explore the association between alcoholic beverages and the immune system, in contrast to the extensive literature dedicated to the cardiovascular system. Because wine is the most investigated product in this review, it does not take into account studies that evaluate the role of alcohol itself without specifying the beverages included (e.g., wine, spirits and beer).The analysis of the data regarding the diseases reviewed here (allergies, celiac disease, the common cold and COVID-19, chronic inflammation bowel diseases, type 1 diabetes, dermatitis herpetiformis, multiple sclerosis, psoriasis, rheumatoid arthritis, systemic lupus erythematosus and thyroid disorders) revealed that, in principle (although with differences between the various pathologies), moderate consumption of wine does not negatively affect the risk of development or the progression of immune-mediated diseases. In some cases, the effect is mainly attributable to the alcohol itself, in others the wine is responsible for a more favourable trend compared to other alcoholic beverages.This review was limited by the scarcity of available papers, so that new prospective studies on the association between wine consumption and immune-mediated diseases should also be specifically designed to draw more solid conclusions.

Targeted serum proteome profiling reveals nicotinamide adenine dinucleotide phosphate (NADPH)-related biomarkers to discriminate linear IgA bullous disorder from dermatitis herpetiformis

Linear IgA bullous dermatosis (LABD) and dermatitis herpetiformis (DH) represent the major subtypes of IgA mediated autoimmune bullous disorders. We sought to understand the disease etiology by using serum proteomics. We assessed 92 organ damage biomarkers in LABD, DH, and healthy controls using the Olink high-throughput proteomics. The positive proteomic serum biomarkers were used to correlate with clinical features and HLA type. Targeted proteomic analysis of IgA deposition bullous disorders vs. controls showed elevated biomarkers. Further clustering and enrichment analyses identified distinct clusters between LABD and DH, highlighting the involvement of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. Comparative analysis revealed biomarkers with distinction between LABD and DH and validated in the skin lesion. Finally, qualitative correlation analysis with DEPs suggested six biomarkers (NBN, NCF2, CAPG, FES, BID, and PXN) have better prognosis in DH patients. These findings provide potential biomarkers to differentiate the disease subtype of IgA deposition bullous disease.

State-of-the-art diagnosis of autoimmune blistering diseases.

Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis herpetiformis. The exact diagnosis of AIBDs is critical for both prognosis and treatment and is based on the clinical appearance combined with the detection of tissue-bound and circulating autoantibodies. While blisters and erosions on the skin and/or inspectable mucosal surfaces are typical, lesions may be highly variable with erythematous, urticarial, prurigo-like, or eczematous manifestations. While direct immunofluorescence microscopy (IFM) of a perilesional biopsy is still the diagnostic gold standard, the molecular identification of the major target antigens opened novel therapeutic avenues. At present, most AIBDs can be diagnosed by the detection of autoantigen-specific serum antibodies by enzyme-linked immunosorbent assay (ELISA) or indirect IFM when the clinical picture is known. This is achieved by easily available and highly specific and sensitive assays employing recombinant immunodominant fragments of the major target antigens, i.e., desmoglein 1 (for pemphigus foliaceus), desmoglein 3 (for pemphigus vulgaris), envoplakin (for paraneoplastic pemphigus), BP180/type XVII collagen (for bullous pemphigoid, pemphigoid gestationis, and mucous membrane pemphigoid), laminin 332 (for mucous membrane pemphigoid), laminin β4 (for anti-p200 pemphigoid), type VII collagen (for epidermolysis bullosa acquisita and mucous membrane pemphigoid), and transglutaminase 3 (for dermatitis herpetiformis). Indirect IFM on tissue substrates and in-house ELISA and immunoblot tests are required to detect autoantibodies in some AIBD patients including those with linear IgA disease. Here, a straightforward modern approach to diagnosing AIBDs is presented including diagnostic criteria according to national and international guidelines supplemented by long-term in-house expertise.

PEMPHIGUS HERPETIFORMIS TREATED EFFECTIVELY WITH DERMOCORTICOIDS ALONE

Introduction: Pemphigus herpetiformis is a rare form of pemphigus. Case Report: A patient presented with a well-limited erythematous-squamous and crusty dermatosis with a ring-like arrangement, with vesiculo-bullous lesions with a herpetiform grouping of the trunk and 4 limbs. Histological and direct immunofluorescence findings were consistent with pemphigus. The diagnosis of pemphigus herpetiformis (PH) was made. The patient was treated with dermocorticoids with lasting remission. Discussion: Pemphigus herpetiformis is a particular form of pemphigus characterised by a clinical picture resembling that of dermatitis herpetiformis and an immunopathological appearance similar to that of pemphigus. It generally has a good prognosis. Treatment is based on dapsone and/or general corticosteroid therapy.

Granular C3 dermatosis-A report of two cases and a mini-review of literature.

Granular C3 dermatosis (GCD) is characterized by bullous, erythematous, and eczematous skin lesions similar to dermatitis herpetiformis, and granular deposition of complement C3 and C5b-9 along the epidermal basement membrane zone (BMZ) by direct immunofluorescence (IF). Here, we present two cases of GCD with different clinical features. Case 1, a 49-year-old man, showed pruritic blisters and erythema of the extremities. Case 2, a 53-year-old woman, showed severely pruritic papules, erythema, and erosions on the entire body with scattered blisters, mainly on the lower extremities. Both patients showed mild eosinophilia on blood tests, subepidermal blisters and prominent eosinophilic infiltration in the upper dermis on histopathological examination, and granular BMZ deposition of C3, but not of immunoglobulins or other complement components, on direct IF. No circulating autoantibodies were detected on enzyme-linked immunosorbent assays, chemiluminescent enzyme immunoassays, indirect IF using 1 mol/L NaCl-split normal human skin, or immunoblotting. Diagnosis of GCD was made in both cases. Case 1 was successfully treated with topical steroids, oral minocycline, and nicotinamide without any recurrence of symptoms. Case 2 was treated with oral steroids and showed remarkable improvement, although mild pruritic papules remained. We reviewed 30 reported GCD cases, including the two cases presented here, since Hashimoto etal. first described GCD in 2016. GCD should be more widely recognized, and further accumulation and validation of cases are required.

Are gluten-free products healthy for non-celiac consumers? How the perception of well-being moderates gluten-free addiction

Gluten-free products are showing an acceleration in assortment and sales growth. Nevertheless, these data should be read considering that a minimal share of the population is allergic or intolerant to gluten compared to the market share of gluten-free products. This study aims to understand why non-gluten-sensitive consumers purchase and consume gluten-free products. Through a survey, we seek to explore how the perception of well-being can contribute to the desire for gluten-free products among these consumers, potentially leading to an addiction to such products. A sample of 182 Italian consumers with no disease, such as Celiac Disease, Gluten Sensitivity, or Dermatitis Herpetiformis, was surveyed online. The empirical results of the Structural Equation Model highlight that the purchase of gluten-free products is based solely on hedonistic-emotional psychological motivations. Furthermore, care for well-being positively moderates the intention-addiction relationship leading healthy consumers to be addicted to gluten-free products. Medical studies have highlighted problems with the consumption of gluten-free products by healthy individuals. However, results evidence a growing trend among the population to consume gluten-free products for psychological and well-being reasons. These findings sparked a debate on the topic and raised important considerations for operators and policymakers.

Diagnostic Utility of Direct Immunofluorescence on Paraffin-Embedded Skin Biopsy Samples for the Diagnosis of Autoimmune Vesiculobullous Lesions.

Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating autoantibodies against antigens in the epidermis or the dermo-epidermal junction. Direct immunofluorescence (DIF) for immunoglobulin (Ig)G, IgC3, and IgA on fresh-frozen tissue is the gold standard diagnostic test for AIBDs. However, DIF in the absence of frozen tissue is challenging for the diagnosis of AIBDs. This study aimed to analyze the practical utility of DIF using paraffin-embedded skin biopsy rather than fresh frozen tissue for the diagnosis of AIBDs. Methodology This cross-sectional comparative study included 30 cases of AIBDs. DIF for IgG and IgA was performed on paraffin-embedded tissue (PE-DIF) after proteinase digestion on histopathologically confirmed 15 pemphigus vulgaris (PV), three pemphigus foliaceous (PF), four bullous pemphigoid (BP), three dermatitis herpetiformis (DH), three subcorneal pustular dermatosis (SCPD), and one case each of linear IgA disease and pemphigoid gestationis (PG). PE-DIF staining pattern was compared with the DIF on fresh frozen tissue (FF-DIF). Results All cases of PV and PF showed an intercellular IgG chicken wire staining pattern similar to FF-DIF. However, background staining was more intense in PV cases while less intense in PF cases. Three BP cases showed linear IgG staining in PE-DIF. DH, SCPD, linear IgA disease, and PG cases did not show IgG positivity. Out of three DH cases, two cases showed granular IgA positivity while linear IgA positivity along the basement membrane was seen in a single case of linear IgA disease. Negative IgG staining was observed in SCPD. Immunofluorescence in PE-DIF was rapidly deteriorating than in FF-DIF. Conclusions DIF done on paraffin-embedded tissue can be used as a supplement and salvage technique with histopathology for the diagnosis of AIBDs,particularly when a cryostat facility for frozen tissue is not available and the patient is unable to undergo a second biopsy procedure.

Navigating Food Allergies: Advances in Diagnosis and Treatment Strategies.

Food allergy is a major health concern worldwide, encompassing both immunologic and non-immunologic reactions. This review thoroughly examines the pathophysiology, clinical manifestations, and treatment options for various types of food allergies. Immunologic food allergies, including IgE-mediated reactions such as oral allergy syndrome and systemic anaphylaxis, pose various diagnostic and management challenges. Non-IgE-mediated reactions such as food protein-induced enterocolitis syndrome, dermatitis herpetiformis, and proctocolitis necessitate individualized patient care.In addition, mixed reactions such as eosinophilic esophagitis and atopic dermatitis complicate the clinical picture. Skin prick tests, serum-specific IgE tests, and oral food challenges are all necessary for accurate food allergy diagnosis. The primary therapeutic options are allergen avoidance, epinephrine-based emergency management, and emerging treatments like immunotherapy. Our review emphasizes the importance of multidisciplinary collaboration and ongoing research in improving our understanding and managing food allergies, promising a brighter future for those affected.

The Need of Differential Diagnosis Between Vulvar Lichen Sclerosus and Autoimmune Dermatoses in Adolescent Girls.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which can manifest in prepubertal or adolescent patients. The prevailing theories point to autoimmune and genetic factors. The primary symptoms of VLS typically include vulvar itching, discomfort, dysuria, and constipation. Physical examination often reveals a characteristic figure8 pattern, involving the labia minora, clitoral hood, and perianal region. However, these symptoms and the age of onset are nonspecific and require differentiation from autoimmune dermatoses such as bullous diseases, pemphigus diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. We performed this study to distinguish VLS from autoimmune dermatoses, and in doing so, uncover the underlying causes of chronic vulvar changes. This knowledge will enable healthcare providers to offer appropriate medical care to affected patients. The study was conducted between July 2020 and February 2021, with a sample of 55 girls aged 2-18years who did not have any systemic diseases. The study group was composed of 20 girls previously diagnosed with vulvar lichen sclerosus, while the control group included 35 girls without VLS. Questionnaires regarding the medical history of the children were completed by their legal guardians. Blood samples were collected and analyzed biochemically to assess human immunoglobulin A (IgA), IgG, and IgM antibodies against various substrates, including the desmosome of stratum spinosum, basement membrane zone, desmoglein 1 (DSG1), desmoglein 3 (DSG3), BP180-NC16A-4X, BP230gC, pemphigoid antigen, collagen type VII NC1, transitional epithelium, gliadin (GAF-3X), endomysium (EMA), and cellular nucleus (ANA). The analysis of the study group revealed that the most commonly observed signs and symptoms included: itching, soreness, burning sensations, and excoriation, as well as erythema or/and pallor of the skin and perineal mucosa. Among the assessed antibodies, only anti-GAF3x antibodies and ANA antibodies were detected. However, the results did not reach statistical significance (p > 0.5).

A175 IMPACT OF THE COVID-19 PANDEMIC ON CANADIANS WITH CELIAC DISEASE

Abstract Background The COVID-19 pandemic affected various healthcare services, including primary care and management of certain health conditions. There is limited data on how the pandemic impacted those with celiac disease (CD). Aims Explore the implications of the COVID-19 pandemic on CD diagnosis and management. Methods The State of Celiac Disease in Canada Survey was made available online by Celiac Canada in December 2022. Over 75 questions were included, covering diagnostic pathways, COVID-19 factors such as infection and vaccination, perceptions around COVID-19, and gluten-free food access during the pandemic. Analysis was restricted to those who responded to having CD, dermatitis herpetiformis, or gluten ataxia. Results A total of 6052 participants were included. A total of 2320 (38.3%) reported having tested positive for COVID-19 at some time during the pandemic. Among those with an infection at some point, 93.8% tested positive after already being diagnosed with CD. Just under 2% of those testing positive for COVID-19 required hospitalization, of which 10% were admitted to the intensive care unit. Vaccination against COVID-19 was reported by 4695 (77.6%) individuals, although 1157 (19.1%) did not provide a response to this specific question. This did not differ significantly by gender (p = 0.901), although it did across age groups (p ampersand:003C 0.001), with a mean age of 54.2 years who were vaccinated compared to a mean age of 45.7 years who were not vaccinated. 718 (11.9%) of individuals felt they were at higher risk of COVID-19 complications due to having CD; however, 1290 did not respond. Reported delays from symptoms to diagnosis did not increase among those diagnosed during the pandemic compared to those diagnosed before. Most respondents did not feel the pandemic made it more challenging to follow the gluten-free diet, and just over half (52.8%) did not feel it was harder to obtain or access gluten-free food during this time. However, almost three-quarters (73.4%) noted an increase in the cost of gluten-free food during the pandemic – this meant adjusting finances in other areas to afford gluten-free food (28.5%), changing the type of food purchased (19.3%), or accessing a food bank (0.7%). Conclusions A significant number of patients with CD reported COVID-19 infections, but less than 2% had serious outcomes. This survey identified gaps in our understanding of COVID-19 vaccination in patients with CD and highlighted some adverse impacts of the pandemic on the costs of gluten-free foods. Funding Agencies Celiac Canada (FKA Canadian Celiac Association)

A173 WHAT HAS CHANGED IN THE DIAGNOSIS OF CELIAC DISEASE IN CANADIANS OVER THE PAST 25 YEARS? RESULTS FROM THE STATE OF CELIAC IN CANADA SURVEY 2022

Abstract Background Prior surveys of Canadians with celiac disease (CD) have demonstrated several challenges on the path to diagnosis. It has been over a decade since findings from the most recent survey were published. Aims To compare the experiences of Canadian individuals prior to CD diagnosis across time. Methods The State of Celiac Disease in Canada Survey is an online, nationwide survey distributed by Celiac Canada in December 2022. Over 75 questions were included, covering diagnostic pathways, presenting symptoms, and involvement of health care professionals (HCPs). Analysis was restricted to those who responded to having CD, dermatitis herpetiformis, or gluten ataxia, as well as provided the time since their diagnosis. Results A total of 6052 participants were included: 1210 (20.0%) were diagnosed in the last two years, 1113 (18.4%) two to five years ago, 1136 (18.8%) five to 10 years ago, 1040 (17.2%) 10 to 15 years ago, 960 (15.9%) 15 to 25 years ago, and 593 (9.8%) more than 25 years ago. Diarrhea was reported in over 70% of respondents diagnosed over 25 years ago, compared to approximately 50% diagnosed in the last five years (Figure 1A). Conversely, 31% of individuals diagnosed over 25 years ago experienced brain fog compared to 57% of those diagnosed in the last two years. Approximately 25% of respondents diagnosed at least 15 years ago experienced symptoms for more than 20 years prior, compared to 15% of those diagnosed in the last two years (Table 1). There were no clear differences in the proportion of respondents who saw a family physician before diagnosis, but there was a drop in those consulting a gastroenterologist (60% diagnosed more than 25 years ago versus 43% diagnosed in the last five years, Figure 1B). Conclusions Over time, there has been a shift in the clinical presentation of celiac disease, which may reflect improved detection of cases without gastrointestinal symptoms. Delays in diagnosis remain a concern but have also improved over time. The phenomenon of fewer patients consulting with gastroenterologists requires further investigation to understand the cause and impact on accurate diagnosis. Delays in diagnosis across time since diagnosis Note: 496 respondents did not report time from symptoms to diagnosis Symptoms experienced (A) and healthcare professionals consulted (B) prior to diagnosis Funding Agencies Celiac Canada (FKA Canadian Celiac Association)

A170 A RARE CASE OF CELIAC DISEASE WITH PULMONARY HEMOSIDEROSIS AND DILATED CARDIOMYOPATHY

Abstract Background Celiac disease (CD) is a primary disease of the intestine but patients also present with extra intestinal manifestations like iron deficiency anemia, dermatitis herpetiformis, selective IgA deficiency, thyroid disorders, diabetes mellitus and various connective tissue disorders but rarely with pulmonary hemosiderosis and dilated cardiomyopathy. Aims We present a rare case of CD associated with pulmonary hemosiderosis and dilated cardiomyopathy in a 10 year old child. Methods Patient interview and chart review was conducted. Literature reveiw was performed using pub med data base. Results A 10 year old presented with a 1 day of cough and hemoptysis. His past medical history includes Constipation and low percentile for weight and height. His first cousin is recently diagnosed with celiac disease, otherwise his family history is unremarkable. He is pale on exam and has a BMI of 13.64 which is ampersand:003C1% for age. He had fine crackles on bilateral lung fields but no signs of respiratory distress. His pericardium was active but no murmur or gallop. CXR showed cardiomegaly and Echo was suggestive of dilated cardiomyopathy. ECG was significant for LBBB. Chest CT showed bilateral confluent symmetric ground glass densities with possibility of pulmonary hemosiderosis. His blood work was significant for iron deficiency anemia, low selenium of 0.94umol/L and zinc 8.4umol/L. His antibody screen was positive for ANA, a tTGA of ampersand:003E4,965.5 and positive EMA. The cardiomyopathy and pulmonary hemosiderosis in this case are considered to be secondary findings as both have been reported in the context of celiac disease and patient was initiated on gluten free diet (GFD). Patient was blood transfused and kept on oral iron and multivitamins. He was also started on aldactazide 1 tab daily, 20mg twice daily enteresto and carvedilol 3.125mg twice daily as part of his heart failure management. At one month follow up, patient was having abdominal discomfort due to his constipation and follow up labs showed improvement of his anemia. Conclusions Patients with pulmonary hemosiderosis occuring with CD demonstrate improvement of their symptoms with GFD highlighting the importance of screening all pulmonary hemosiderosis patients for CD. Cardiomyopathy associated with celiac disease is also another very rare occurrence and only few studies have been reported so far. In conclusion, although a rare occurrence, the association of CD with pulmonary hemosiderosis and cardiomyopathy has huge therapeutic implications emphasizing the need to have high index of suspicion and routine screening for CD among patients with pulmonary hemosiderosis and dilated cardiomyopathy. Funding Agencies The hospital for sick children/sick kids hospital

Retrospective Evaluation of Risk Factors for Ischemic Heart Disease in Patients with Dermatitis Herpetiformis

Background and Purpose: Some have proposed that the cardiovascular risk among individuals with DH differs from that of the general population. This study aimed to analyze cardiovascular risk factors in DH patients and compare them to a matched control group without DH or celiac disease.
 
 Methods: This was a retrospective hospital-based study involving patients diagnosed with DH, both clinically and histopathologically, along with age- and sex-matched control subjects without the disease. The presence of ischemic heart disease, and the risk factors including laboratory values, treatment and comorbidity histories were evaluated comparatively. (SPSS version 28.0). 
 
 Results: Thirty-five patients with DH and 49 controls were included. No significant difference was found between the DH patients and controls regarding the prevalence of ischemic heart disease (22.9% vs. 14.3%, p:0.312). Diabetes mellitus was significantly higher and HDL cholesterol levels were significantly lower in the DH group (respectively, p: 0.044 and p: 0.018). Celiac autoantibodies, the laboratory values, or treatments did not show any significant correlation with heart disease in DH.
 
 Conclusion: This study revealed that HDL cholesterol were significantly lower in DH patients compared to general population. More research is needed to optimize the cardiovascular health of DH patients.