Extra-digestive manifestations of celiac disease.

Introduction: Celiac disease (CD) is a chronic autoimmune disorder triggered by gluten ingestion in genetically predisposed individuals, presenting with a diverse range of symptoms that extend beyond the gastrointestinal tract. The condition's systemic nature is evidenced by its extra-digestive manifestations, which can affect various organs including the skin, joints, liver, and nervous system. Methods: This descriptive, retrospective study was conducted at a tertiary care center, focusing on adult patients diagnosed with CD who exhibited extra-digestive symptoms. Data were extracted from medical records of patients admitted between January 1, 2010 and June 30, 2024. Variables included demographic information, primary diagnosis, and associated extra-digestive manifestations. Descriptive statistical methods were employed for data analysis. Results: The sample included 108 patients with CD, the mean age was 43.21 years, with a predominance of females (76.85%). Iron deficiency anemia was the most common extra-digestive manifestation, affecting 20.37% of patients, followed by hypoproteinemia (18.52%) and Hashimoto's thyroiditis (14.81%). Co-occurrence analysis revealed frequent combinations of conditions, such as anemia with cardiovascular diseases and depressive disorders. Notable associations with neurological conditions like gluten ataxia and peripheral neuropathy were also observed. Conclusion: This study highlights the extensive extra-digestive manifestations of celiac disease, underscoring its systemic impact. The high prevalence of autoimmune conditions such as Hashimoto's thyroiditis and rheumatoid polyarthritis among CD patients reflects the need for holistic management strategies. Discrepancies between our findings and existing literature, particularly regarding skin and neurological conditions, emphasize the need for further research to better understand these associations and the long-term effects of a gluten-free diet.

Incidence and prevalence of autoimmune diseases in China: A systematic review and meta-analysis of epidemiological studies.

Background: Autoimmune diseases account for a substantial burden of disease in high-income countries, including Europe and North America. However, their epidemiology remains under-researched in other regions. We examined the incidence and prevalence of eight autoimmune diseases in the adult Chinese population through a systematic review of epidemiological studies. Methods: We searched OvidSP MEDLINE and Google Scholar from 1995 to 2023 (inclusive) for articles on the incidence or prevalence of autoimmune thyroiditis (AT), Graves' disease (GD), type 1 diabetes mellitus (T1D), multiple sclerosis (MS), Crohn's disease (CD), ulcerative colitis (UC), rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). We included studies from mainland China, Taiwan, Hong Kong or Macau. The study is registered with PROSPERO (CRD42021225842). Findings: We retrieved 2278 records, of which 62 studies (161 estimates) were included in the systematic review, and 42 studies (101 estimates) were included in the meta-analysis. Pooled fixed-effects estimates for incidence of CD, UC, MS, T1D and SLE were 0.22 (95% CI 0.21-0.23), 1.13 (1.10-1.17), 0.28 (0.26-0.30), 2.20 (1.70-2.84) and 4.87 (4.21-5.64) per 100,000 persons, respectively. For RA, one study estimate was included, with an incidence of 15.8 per 100,000 persons. Fixed-effects estimates for the prevalence of CD, UC, MS, SLE, RA, GD and AT were 3.73 (95% CI 3.68-3.78), 16.11 (15.93-16.29), 4.08 (3.95-4.21), 93.44 (92.27-94.63), 104 (103-106), 450 (422-481) and 2322 (2057-2620), respectively, per 100,000 persons. Across all conditions, women were almost twice as likely as men to be diagnosed with an autoimmune disease. Interpretation: There is marked variation in the frequency of autoimmune diseases among Chinese adults. We estimate that 2.7-3.0% (>31 million people) of the adult Chinese population have one or more autoimmune diseases, comparable to Western populations, with the majority of the burden from autoimmune thyroid diseases and rheumatoid arthritis.

A Case of Primary Biliary Cholangitis in a Patient With Multiple Sclerosis.

Multiple sclerosis (MS) is a chronic inflammatory disease that causes demyelination in the brain and spinal cord, leading to significant neurological disability in young adults. Patients with MS are predisposed to other autoimmune disorders, though the co-occurrence of MS and primary biliary cholangitis (PBC) is rare. PBC is an autoimmune liver disease that affects bile ducts, leading to cholestasis and liver cirrhosis, predominantly in women aged over 40 years. We report the case of an 81-year-old woman with a history of MS and hypertension, bedridden for 10 years, who was admitted with a severe sacral ulcer and bacteremia. During hospitalization, she developed persistent itching, and elevated liver enzymes were detected. Imaging ruled out cholecystitis but revealed a large gallstone and hepatomegaly. Elevated M2 antimitochondrial antibodies confirmed PBC. The patient was treated with ursodeoxycholic acid, leading to symptom improvement. This case highlights the necessity for a thorough evaluation of autoimmune comorbidities in patients with MS and suggests a potential genetic and environmental link between MS and PBC. Further research is needed to explore this association and improve treatment strategies.

Neonatal lupus is a novel cause of positive newborn screening for X-linked adrenoleukodystrophy.

We report three unrelated individuals, each exposed to maternal autoantibodies during gestation and found to have elevated very long-chain fatty acids (VLCFAs) in the newborn period after screening positive by California newborn screening (NBS) for X-linked adrenoleukodystrophy (ALD). Two probands presented with clinical and laboratory features of neonatal lupus erythematosus (NLE); the third had features suggestive of NLE and a known maternal history of Sjogren's syndrome and rheumatoid arthritis. In all three individuals, subsequent biochemical and molecular evaluation for primary and secondary peroxisomal disorders was nondiagnostic with normalization of VLCFAs by 15 months of age. These cases add to the expanding differential diagnosis to consider in newborns who screen positive for ALD via elevated C26:0-lysophosphatidylcholine. Though the pathophysiology of how transplacental maternal anti-Ro antibodies damage fetal tissue is not well-understood, we postulate that the VLCFA elevations reflect a systemic inflammatory response and secondary peroxisomal dysfunction that improves once maternal autoantibodies wane after birth. Additional evaluation of this phenomenon is warranted to better understand the intricate biochemical, clinical, and possible therapeutic overlap between autoimmunity, inflammation, peroxisomal dysfunction, and human disease.

Autoimmune conditions and pancreatic cancer risk in older American adults.

Pancreatic cancer (PC) is highly fatal, and its incidence is increasing in the United States. Population-based registry studies suggest associations between a few autoimmune conditions and PC risk, albeit based on a relatively small number of cases. We conducted a population-based, nested case-control study to examine the associations between autoimmune conditions and PC risk within the Surveillance, Epidemiology, and End Results Program (SEER)-Medicare population. Incident primary malignant PC cases (n = 80 074) were adults ≥66 years and diagnosed between 1992 and 2015. Controls (n = 320 296) were alive at the time cases were diagnosed and frequency-matched to cases (4:1 ratio) by age, sex, and year of diagnosis. We used multivariable-adjusted, unconditional logistic regression to calculate odds ratios (ORs) and 95% confidence intervals (CIs) for 45 autoimmune conditions identified from Medicare claims. Eight autoimmune conditions including ankylosing spondylitis (OR = 1.45; 95% CI: 1.14-1.84), Graves' disease (OR = 1.18; 95% CI: 1.03-1.34), localized scleroderma (OR = 1.27; 95% CI: 1.06-1.52), pernicious anemia (OR = 1.08; 95% CI: 1.02-1.14), primary sclerosing cholangitis (OR = 1.37; 95% CI: 1.18-1.59), pure red cell aplasia (OR = 1.31; 95% CI: 1.16-1.47), type 1 diabetes (OR = 1.11; 95% CI: 1.07-1.15), and ulcerative colitis (OR = 1.18; 95% CI: 1.07-1.31) were associated with increased PC risk (false discovery rate-adjusted P values <.10). In subtype analyses, these conditions were associated with pancreatic ductal adenocarcinoma, whereas only ulcerative colitis was associated with pancreatic neuroendocrine tumors. Our results support the hypothesis that autoimmune conditions may play a role in PC development.

Incident autoimmune conditions among males receiving quadrivalent human papillomavirus vaccine in the United States.

BACKGROUND: The potential for vaccines to induce autoimmunity has been the subject of considerable investigation and autoimmune induction remains a common focus for vaccine safety research. This study assessed the risk of new onset autoimmune conditions among males receiving the 4-valent human papillomavirus (HPV) vaccine (4vHPV). METHODS: Within a US health insurance claims database, we formed a cohort of male 4vHPV vaccine recipients between 2009 and 2016, along with a propensity score matched cohort of males who did not receive the 4vHPV vaccine. The study outcome was new onset autoimmune conditions (20 separate conditions) within four categories (rheumatologic/hematologic, gastroenterologic, endocrinologic and neurologic/ophthalmalogic). Outcomes identified using diagnosis codes were adjudicated through medical record review. Incidence rates (per 1,000 person-years) were estimated for the vaccinated and unvaccinated groups along with rate ratios (RRs). RESULTS: There were 65,606 males receiving at least one dose of 4vHPV vaccine, and 55,670 were matched to a comparator. The matched 4vHPV vaccine cohort provided 35 confirmed cases among 39,735 person-years, for an incidence rate of 0.88 (95% CI: 0.61-1.23), while the comparator cohort provided 47 confirmed cases among 58,215 person-years, an incidence rate of 0.81 (0.59-1.07), a RR of 1.09 (0.70-1.69). The RR within categories was 0.49 (0.10-2.42) for rheumatologic/hematologic, 1.26 (0.58-2.71) for gastroenterologic, 1.11 (0.61-2.02) for endocrinologic and 1.46 (0.21-10.40) for neurologic. CONCLUSIONS: The incidence of autoimmune conditions among males receiving the 4vHPV vaccine was similar to that among unvaccinated males. These results are consistent with other studies that have assessed autoimmunity with the 4vHPV vaccine.

Severe COVID-19 in patients with immune-mediated rheumatic diseases: a stratified analysis from the SORCOM multicenter registry.

OBJECTIVES: To evaluate risk factors for severe Coronavirus Disease 2019 (COVID-19) in patients with immune-mediated rheumatic diseases, stratified by systemic autoimmune conditions and chronic inflammatory arthritis. METHODS: An observational, cross-sectional multicenter study was performed. Patients from 10 Rheumatology departments in Madrid who presented with SARS-CoV-2 infection between Feb 2020 and May 2021 were included. The main outcome was COVID-19 severity (hospital admission or mortality). Risk factors for severity were estimated, adjusting for covariates (sociodemographic, clinical and treatments), using logistic regression analyses. RESULTS: 523 patients with COVID-19 were included, among whom 192 (35.6%) patients required hospital admission and 38 (7.3%) died. Male gender, older age and comorbidities such as diabetes mellitus, hypertension and obesity were associated with severe COVID-19. Corticosteroid doses over 10 mg/day, rituximab, sulfasalazine and mycophenolate use, were independently associated with worse outcomes. COVID-19 severity decreased over the different pandemic waves. Mortality was higher in the systemic autoimmune conditions (univariate analysis, p<0.001), although there were no differences in overall severity in the multivariate analysis. CONCLUSIONS: This study confirms and provides new insights regarding the harmful effects of corticosteroids, rituximab and other therapies (mycophenolate and sulfasalazine) in COVID-19. Methotrexate and anti-TNF therapy were not associated with worse outcomes.

Autoimmune conditions in the World Trade Center general responder cohort: A nested case-control and standardized incidence ratio analysis.

BACKGROUND: The World Trade Center (WTC) general responder cohort (GRC) was exposed to environmental toxins possibly associated with increased risk of developing autoimmune conditions. OBJECTIVES: Two study designs were used to assess incidence and risks of autoimmune conditions in the GRC. METHODS: Three clinically trained professionals established the status of possible GRC cases of autoimmune disorders adhering to diagnostic criteria, supplemented, as needed, by specialists' review of consenting responders' medical records. Nested case-control analyses using conditional logistic regression estimated the risk associated with high WTC exposure (being in the 9/11/2001 dust cloud or ≥median days' response worked) compared with low WTC exposure (all other GRC members'). Four controls were matched to each case on age at case diagnosis (±2 years), sex, race/ethnicity, and year of program enrollment. Sex-specific and sensitivity analyses were performed. GRC age- and sex-adjusted standardized incidence ratios (SIRs) were compared with the Rochester Epidemiology Project (REP). Complete REP inpatient and outpatient medical records were reviewed by specialists. Conditions meeting standardized criteria on ≥2 visits were classified as REP confirmed cases. RESULTS: Six hundred and twenty-eight responders were diagnosed with autoimmune conditions between 2002 and 2017. In the nested case-control analyses, high WTC exposure was not associated with autoimmune domains and conditions (rheumatologic domain odds ratio [OR] = 1.03, 95% confidence interval [CI] = 0.77, 1.37; rheumatoid arthritis OR = 1.12, 95% CI = 0.70, 1.77). GRC members had lower SIR than REP. Women's risks were generally greater than men's. CONCLUSIONS: The study found no statistically significant increased risk of autoimmune conditions with WTC exposures.

Dermatology for the internist: optimal diagnosis and management of atopic dermatitis.

Internists are front-line health care providers that commonly provide the first encounter to patients for dermatological conditions, especially atopic dermatitis (AD). Internists need to be comfortable with managing mild-moderate AD in their practices. Criteria and guidelines established in dermatology literature are available to help the general practitioner diagnose and treat AD. AD is a systemic disease associated with multiple cutaneous and extra-cutaneous comorbidities that warrant screening by internists, especially mental health conditions. Environmental factors may play a role in the development or worsening of AD; however, there is currently no strong evidence to guide specific population- or clinic-based interventions for their avoidance. While food allergies are common in AD patients, the role of food allergens as an exacerbating factor for AD is controversial. Before starting any dietary modifications, careful evaluation should be performed by an allergist. If the patient is not well-controlled despite adequate topical therapies or is experiencing severe/worsening disease, early referral to dermatology is warranted to rule out confounding diagnoses and/or escalation to systemic therapies. Finally, it is important to recognise the racial disparities present in AD and address these when formulating treatment plans.Key messages:Confounding dermatoses, either instead of or in addition to AD, should be considered in treatment-refractory AD, and the appropriate workup may be initiated while awaiting dermatology referral.AD patients have multiple cutaneous and extra-cutaneous comorbidities that warrant screening by internists, especially mental health conditions.

Fears for the future: A qualitative exploration of the experiences of individuals living with multiple sclerosis, and its impact upon the family from the perspective of the person with MS.

OBJECTIVES: Notorious for its unpredictable nature, Multiple sclerosis (MS) can have a profound impact on all areas of a person's life, as well as the lives of their family. The aim of this qualitative study was to develop an in-depth understanding of the experiences of individuals living with MS and its impact upon the family system from the perspective of the person with M. METHODS: Qualitative data were collected through semi-structured interviews conducted with 14 people living with MS (pwMS). Interviews lasted for approx. 1 hr, were digitally recorded and transcribed verbatim. Anonymized data were then analysed using thematic analysis (Braun & Clarke, 2006, Qualitative Research in Psychology, 3, 77). RESULTS: The themes identified within participant narratives reflected the turbulent emotions experienced by pwMS after diagnosis and the impact upon their family. Three key themes were identified with six subthemes. The central themes were feeling let down by health care, fears for the future, and finding a way forward. Fears about loss of autonomy and independence characterized the narratives that notably focussed upon impact on the self, despite the study emphasis. Discussions of suicide also featured. CONCLUSIONS: The data demonstrate the psychosocial impact of living with MS as life-changing, encompassing traumatic losses and adaptation that was ongoing. The findings highlight the important role of health professionals and the dynamic impact of illness identity and engulfment. Opportunities to improve patient care by acknowledging the emotional impact of MS are discussed. Targeted and timely interventions to enhance psychological well-being, and areas for future research are considered.

Autoimmune conditions and primary central nervous system lymphoma risk among older adults.

Primary central nervous system lymphoma (PCNSL) risk is highly increased in immunosuppressed individuals, such as those with human immunodeficiency virus infection and solid organ transplant recipients, but rates are increasing among immunocompetent older adults (age ≥65 years). We utilized data from a large, nationally-representative cohort of older adults in the United States and found that PCNSL is significantly associated with systemic lupus erythematosus, polyarteritis nodusa, autoimmune hepatitis, myasthenia gravis and uveitis. Immunosuppressive drugs given to treat these conditions may increase PCNSL risk, but these associations cannot explain the observed temporal increase in PCNSL rates, given the low prevalence of these conditions.

PBC and related extrahepatic diseases.

Patients with PBC have at least 60% of probability to have an autoimmune extrahepatic condition. The pathogenesis of these conditions includes a common mechanism involving both innate and adaptive immune responses targeting cholangiocytes and different extrahepatic tissues. The recent EASL guidelines recommend the management of these conditions, although detailed practical treatments have not been indicated. Autoimmune extrahepatic conditions may include: rheumatologic, endocrine, pulmonary, gastrointestinal, dermatologic diseases. This review aims to focus the most important extrahepatic autoimmune conditions associated to PBC with practical recommendation regarding diagnostic approach and management.

Gender-Based Differences in a Population-Based Cohort with Celiac Disease: More Alike than Unalike.

BACKGROUND: There is a gap in research focused on gender-based differences in non-referral populations with celiac disease. AIMS: The aim of this study was to estimate those differences in a unique population-based cohort of patients with celiac disease with respect to (1) presenting symptoms, (2) associated autoimmune disorders, and (3) survival. METHODS: Clinical data were systematically abstracted from the electronic medical record of a population-based incident cohort of patients with celiac disease. Logistic regression was used to assess the strength of the association of presenting symptoms and gender. Survival differences between genders were evaluated with Cox regression. RESULTS: We included 282 patients (females 65%, median age 39 years) diagnosed between 1990 and 2015. The female to male ratio was 1.85:1. Men and women presented similarly. Women were more likely to present with constipation (OR 2.33; 95% CI 1.06-5.12; p = 0.035). Anemia and abdominal distention or bloating were more frequently seen in women, but not on a statistically significant level. Overall autoimmune diseases were equally prevalent (31.6%) in males (30.2%) and females (32.2%) (p = 0.74). Hypothyroidism predominated in women. Age-adjusted survival was lower among men than women (HR 3.00; 95% CI 1.26-7.21, p = 0.014), but not more so than in the general population. Cancer was the most common cause of death, and there were two possible celiac disease-related deaths. CONCLUSIONS: This study showed that men and women are more alike than unalike when it comes to celiac disease presentation and prevalence of concurrent autoimmune disease.

Severe Sequelae to Mold-Related Illness as Demonstrated in Two Finnish Cohorts.

The presence of toxic indoor molds with accompanying bacterial growth is clearly detrimental to human health. The pathophysiological and toxicological effects of toxins and structural components of molds and bacteria have been clarified in experiments conducted in tissue culture and animals, and there is convincing epidemiologic evidence; nonetheless their implications for human health are either ignored or denied, at least in Finland. In this communication, we describe two cohorts suffering severe sequelae to mold-related illness. One cohort is a nine-member family with pets that moved into a new house, which soon proved to be infested with pathogenic molds. The other cohort consists of 30 teachers and 50 students from a mold-infested school building. The first cohort experienced a plethora of mucosal irritation, neurological, skin, allergic, and other symptoms, with all family members ultimately developing a multiple chemical syndrome. In the second cohort, we detected a greatly elevated prevalence of autoimmune conditions and malignancies. We claim that mold-related illness exists in multiple facets; if not simply a transient mucosal irritation or even an increased risk of asthma onset or its exacerbation. We propose a scheme to explain the natural course of the mold-related illness. We recommend that future studies should combine data from, e.g., cancer, autoimmune, and endocrine disorder registers and neurological and mental health or neuropsychological registers with mold-exposed individuals being monitored for prolonged follow-up times.

Risk of new onset autoimmune disease in 9- to 25-year-old women exposed to human papillomavirus-16/18 AS04-adjuvanted vaccine in the United Kingdom.

To assess the risk of autoimmune disease (AD) in 9-25 year-old women within 1 year after the first AS04-HPV-16/18vaccine dose, a retrospective, observational database cohort study was conducted using CPRD GOLD. From CPRD GOLD 4 cohorts (65,000 subjects each) were retrieved: 1 exposed female cohort (received ≥1 AS04-HPV-16/18 vaccine dose between Sep2008-Aug2010) and 3 unexposed cohorts: historical female (Sep2005-Aug2007), concurrent male, and historical male. Co-primary endpoints were confirmed neuroinflammatory/ophthalmic AD and other AD, secondary endpoints were confirmed individual AD. Risk of new onset of AD was compared between cohorts (reference: historical cohort) using Poisson regression. The main analysis using confirmed cases showed no neuroinflammatory/ophthalmic AD cases in the female exposed cohort. Incidence rate ratio (IRR) (95% CI) of other AD was 1.41 (0.86 to 2.31) in female and 1.77 (0.94 to 3.35) in male cohorts when compared to the female and male historical cohort, respectively. Secondary endpoints were evaluated for diseases with >10 cases, which were Crohn's disease (IRR: 1.21 [0.37 to 3.95] for female and 4.22 [0.47 to 38.02] for male cohorts), autoimmune thyroiditis (IRR: 3.75 [1.25 to 11.31] for female and no confirmed cases for male cohorts) and type 1 diabetes (IRR: 0.30 [0.11 to 0.83] for female and 2.46 [1.08 to 5.60] for male cohorts). Analysis using confirmed and non-confirmed cases showed similar results, except for autoimmune thyroiditis in females, IRR: 1.45 (0.79 to 2.64). There was no evidence of an increased risk of AD in women aged 9 to 25 years after AS04-HPV-16/18 vaccination.

Associations of non-Hodgkin Lymphoma (NHL) risk with autoimmune conditions according to putative NHL loci.

Autoimmune conditions and immune system-related genetic variations are associated with risk of non-Hodgkin lymphoma (NHL). In a pooled analysis of 8,692 NHL cases and 9,260 controls from 14 studies (1988-2007) within the International Lymphoma Epidemiology Consortium, we evaluated the interaction between immune system genetic variants and autoimmune conditions in NHL risk. We evaluated the immunity-related single nucleotide polymorphisms rs1800629 (tumor necrosis factor gene (TNF) G308A), rs1800890 (interleukin-10 gene (IL10) T3575A), rs6457327 (human leukocyte antigen gene (HLA) class I), rs10484561 (HLA class II), and rs2647012 (HLA class II)) and categorized autoimmune conditions as primarily mediated by B-cell or T-cell responses. We constructed unconditional logistic regression models to measure associations between autoimmune conditions and NHL with stratification by genotype. Autoimmune conditions mediated by B-cell responses were associated with increased NHL risk, specifically diffuse large B-cell lymphoma (odds ratio (OR) = 3.11, 95% confidence interval (CI): 2.25, 4.30) and marginal zone lymphoma (OR = 5.80, 95% CI: 3.82, 8.80); those mediated by T-cell responses were associated with peripheral T-cell lymphoma (OR = 2.14, 95% CI: 1.35, 3.38). In the presence of the rs1800629 AG/AA genotype, B-cell-mediated autoimmune conditions increased NHL risk (OR = 3.27, 95% CI: 2.07, 5.16; P-interaction = 0.03) in comparison with the GG genotype (OR = 1.82, 95% CI: 1.31, 2.53). This interaction was consistent across major B-cell NHL subtypes, including marginal zone lymphoma (P-interaction = 0.02) and follicular lymphoma (P-interaction = 0.04).

Idiopathic thrombocytopenic purpura and oral surgery: case report / Púrpura trombocitopênica idiopática e cirurgia bucal: relato de caso

OBJECTIVES: To review the function of platelets in the blood clotting mechanism. To address,conceptualize and classify thrombocytopenic purpura, especially idiopathic thrombocytopenic purpura (ITP),emphasizing the immunological aspects involved in the etiology of the disease. To address the clinicalmanifestations of the disease and the appropriate therapy. To present the case study of a patient with ITPwho requires oral surgical intervention. DISCUSSION: Leukopenia in ITP can be subclinical. The firstmanifestation of the disease can be severe hemorrhaging due to small lacerations or minor medical anddental surgical procedures. The cause of the platelet reduction is idiopathic; an autoimmune reaction in whichthe antibodies destroy the platelets appears to participate in the process. A variety of situations can occur,leading to mild to severe thrombocytopenia. One frequent aspect is the instability of the platelet count,which oscillates inconsistently and may be related to infections and other factors that have not been clearlydetermined, including stress. In the case presented here, the leukopenia in the patient was mild in presentation.However, it was decided that the patient should be referred to a hematologist for preparation and clearance.Oral surgery that compromises the bone, such as exodontia, can present significant difficulties in localizingand clamping intraosseous vessels, which does not only occur in small soft tissue surgeries. CONCLUSIONS:Platelet destruction in ITP occurs from a complex process that is comprised of multiple components of theimmune system. The platelets are prematurely destroyed by antibodies that are aimed at the platelet glycoprotein,which can results in serious, even fatal consequences. It is important to emphasize the significance of themedical history and the appropriate physical examination during the diagnostic process, as well as collaborationwith the patient’s medical...

OBJETIVOS: Revisar a função das plaquetas no mecanismo de coagulação sanguínea. Conceituare classificar a púrpura trombocitopênica, especialmente a púrpura idiopática, enfatizando osaspectos imunológicos envolvidos na etiologia da doença. Enfocar as manifestações clínicas dadoença e a terapia apropriada. Apresentar um caso de manifestação clínica da doença e a terapiaapropriada. Apresentar o caso de uma paciente com púrpura que necessitava de intervençãocirúrgica bucal. DISCUSSÃO: A leucopenia na púrpura trombocitopênica idiopática pode sersubclínica. A primeira manifestação da doença pode ser uma hemorragia severa por causa depequenas lacerações ou pequenos procedimentos médicos e odontológicos. A causa da reduçãodas plaquetas é idiopática; uma reação autoimune na qual os anticorpos destroem as plaquetasparece participar do processo. Uma variedade de situações pode ocorrer, levando àtrombocitopenia discreta a severa. Um aspecto frequente é a instabilidade da contagem plaquetária,que oscila inconsistentemente e pode relacionar-se com infecções e outros fatores ainda nãoclaramente determinados, incluindo estresse. No presente caso, o paciente apresentava discretaleucopenia. Entretanto, decidiu-se que o paciente deveria ser avaliado por um hematologista,para preparação e liberação para cirurgia, pois a cirurgia bucal que compromete osso, como aexodontia, pode apresentar dificuldades significativas na localização e pinçamento de vasosintraósseos, o que não acontece em pequenas cirurgias de tecidos moles. CONCLUSÕES: Adestruição plaquetária na púrpura trombocitopênica ocorre por meio de um complexo processo,com múltipos componentes do sistema imune. As plaquetas são destruídas prematuramente poranticorpos dirigidos contra as glicoproteínas plaquetárias, o que pode resultar em sérias – emesmo fatais – consequências. É importante enfatizar o significado da história médica e o examefísico adequado no processo diagnóstico...