Single-cell transcriptomics unveil profiles and interplay of immune subsets in rare autoimmune childhood Sjögren's disease.

Childhood Sjögren's disease represents critically unmet medical needs due to a complete lack of immunological and molecular characterizations. This study presents key immune cell subsets and their interactions in the periphery in childhood Sjögren's disease. Here we show that single-cell RNA sequencing identifies the subsets of IFN gene-enriched monocytes, CD4+ T effector memory, and XCL1+ NK cells as potential key players in childhood Sjögren's disease, and especially in those with recurrent parotitis, which is the chief symptom prompting clinical visits from young children. A unique cluster of monocytes with type I and II IFN-related genes is identified in childhood Sjögren's disease, compared to the age-matched control. In vitro regulatory T cell functional assay demonstrates intact functionality in childhood Sjögren's disease in contrast to reduced suppression in adult Sjögren's disease. Mapping this transcriptomic landscape and interplay of immune cell subsets will expedite the understanding of childhood Sjögren's disease pathogenesis and set the foundation for precision medicine.

The Florida Scoring System for stratifying children with suspected Sjögren's disease: a cross-sectional machine learning study.

BACKGROUND: Childhood Sjögren's disease is a rare, underdiagnosed, and poorly-understood condition. By integrating machine learning models on a paediatric cohort in the USA, we aimed to develop a novel system (the Florida Scoring System) for stratifying symptomatic paediatric patients with suspected Sjögren's disease. METHODS: This cross-sectional study was done in symptomatic patients who visited the Department of Pediatric Rheumatology at the University of Florida, FL, USA. Eligible patients were younger than 18 years or had symptom onset before 18 years of age. Patients with confirmed diagnosis of another autoimmune condition or infection with a clear aetiological microorganism were excluded. Eligible patients underwent comprehensive examinations to rule out or diagnose childhood Sjögren's disease. We used latent class analysis with clinical and laboratory variables to detect heterogeneous patient classes. Machine learning models, including random forest, gradient-boosted decision tree, partial least square discriminatory analysis, least absolute shrinkage and selection operator-penalised ordinal regression, artificial neural network, and super learner were used to predict patient classes and rank the importance of variables. Causal graph learning selected key features to build the final Florida Scoring System. The predictors for all models were the clinical and laboratory variables and the outcome was the definition of patient classes. FINDINGS: Between Jan 16, 2018, and April 28, 2022, we screened 448 patients for inclusion. After excluding 205 patients due to symptom onset later than 18 years of age, we recruited 243 patients into our cohort. 26 patients were excluded because of confirmed diagnosis of a disorder other than Sjögren's disease, and 217 patients were included in the final analysis. Median age at diagnosis was 15 years (IQR 11-17). 155 (72%) of 216 patients were female and 61 (28%) were male, 167 (79%) of 212 were White, and 20 (9%) of 213 were Hispanic, Latino, or Spanish. The latent class analysis identified three distinct patient classes: class I (dryness dominant with positive tests, n=27), class II (high symptoms with negative tests, n=98), and class III (low symptoms with negative tests, n=92). Machine learning models accurately predicted patient class and ranked variable importance consistently. The causal graphical model discovered key features for constructing the Florida Scoring System. INTERPRETATION: The Florida Scoring System is a paediatrician-friendly tool that can be used to assist classification and long-term monitoring of suspected childhood Sjögren's disease. The resulting stratification has important implications for clinical management, trial design, and pathobiological research. We found a highly symptomatic patient group with negative serology and diagnostic profiles, which warrants clinical attention. We further revealed that salivary gland ultrasonography can be a non-invasive alternative to minor salivary gland biopsy in children. The Florida Scoring System requires validation in larger prospective paediatric cohorts. FUNDING: National Institute of Dental and Craniofacial Research, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Heart, Lung, and Blood Institute, and Sjögren's Foundation.

Xanthoma of the Jaw Bones: Cases Series and Review of Literature.

BACKGROUND: Intraosseous xanthomas are rare benign lesions sometimes associated with excess lipid production. Xanthoma of the jaw bones (XJB) was first reported in 1964, and fewer than 50 cases have been reported in the English literature to date. The etiopathogenesis of XJB is highly suggestive of a reactive process or a metabolic condition. METHOD: Seven cases of XJBs were retrieved from the archives of 4 oral and maxillofacial pathology services. Clinical, radiographic and histopathologic features of all these cases were retrospectively analyzed. Immunohistochemical (IHC) stains for S100 and CD68 were performed. RESULTS: All seven cases involved the mandible. Patients' age ranged between 13 and 69 years with an evenly distributed female to male ratio. One patient had a medical history of hyperlipidemia, but the medical and dental histories of the others were unremarkable. For most cases, XJB was an incidental finding discovered during routine radiographic examination. Swelling and cortical expansion were noted in a few cases. Radiographically, cases typically presented as either well-defined multilocular or unilocular lesions, which were either radiolucent or mixed radiolucent/radiopaque. All the lesions were treated with surgical curettage and no recurrence was observed during subsequent follow-ups. Each of the seven cases exhibited sheets of foamy macrophages. The diagnosis is established by exclusion of entities with overlapping microscopic features and involved correlation with the clinical, histological, radiographic and IHC profiles. Immunohistochemically, all the cases expressed diffuse positivity for CD68 and were negative for S100. CONCLUSION: XJB is a rare lesion of unknown etiology, which may mimic other benign or reactive jaw lesions. Due to its rarity and the potential diagnostic challenges it presents, clinicians must remain vigilant and consider CXJ in their differential when assessing radiolucent jaw anomalies.

Increased frequency of oral plasma cell mucositis/plasma cell gingivitis after the COVID-19 pandemic: a 23-year retrospective analysis and review of diagnostic challenges relating to this condition.

OBJECTIVE: Oral plasma cell mucositis (PCM) or localized plasma cell gingivitis (PCG) is an idiopathic inflammatory condition often associated with hypersensitivity reactions. This study aimed to evaluate the frequency and features of PCM/PCG in a large biopsy service over a time period of more than 20 years. STUDY DESIGN: The biopsy archives at University of Florida College of Dentistry were searched from 2000 through the first quarter of 2023 for cases of oral PCM or PCG. Case data were aggregated and analyzed. RESULTS: A total of 107 cases were included. Between 2000 and 2019, PCM/PCG was diagnosed in 0.03% of all biopsy cases. Starting in 2020 through 2023, the percentage of biopsies diagnosed as PCM/PCG increased threefold to 0.10% of all biopsy cases, and the mean patient age increased by 3 years. There were no statistically significant differences between cases diagnosed from 2000 to 2019 and those from 2020 to 2023 regarding age, sex, location, or histology. CONCLUSIONS: A significant increase in PCM/PCG was identified in this study at one institution coinciding with the start of the COVID-19 pandemic. Further investigation is recommended to determine if this is a widespread phenomenon and identify possible etiologic mechanisms.

COVID-related mucormycosis mimicking dental infection.

A recent increase in the prevalence of mucormycosis of the head and neck in patients who have recovered from COVID-19 following hospitalization has been reported. A Majority of the cases have been reported from India. Conditions such as diabetes mellitus, use of corticosteroids for other autoimmune conditions, organ transplant, immunosuppression, immunodeficiency, and malignancies especially hematologic ones, are all known risk factors for mucormycosis. Recently, hospitalization for COVID-19 has been added to the list of risk factors for opportunistic mucormycosis infection. This is likely attributable to the high doses and prolonged use of corticosteroids in the treatment of hospitalized COVID-19 patients. Case Description: Two patients with post-COVID-19 associated rhinocerebral mucormycosis presented with profound unexplained dental disease including tooth mobility and dental abscess mimicking periodontal disease. The patients were previously hospitalized for COVID-19 and received prolonged treatment with high doses of corticosteroids. The patients responded well to the surgical debridement with or without antifungal therapy. Conclusion: Oral healthcare providers including oral and maxillofacial surgeons, dentists, dental hygienists, and other dental practitioners can play a vital role in the recognition and early diagnosis of rhinocerebral mucormycosis given the large number of patients with severe COVID-19 infection who have recovered following hospitalization and/or received long-term high doses of immunosuppressive treatment.

Localization and characterization of human papillomavirus-16 in oral squamous cell carcinoma.

OBJECTIVES: The role of Human papillomavirus (HPV) in the oral squamous cell carcinoma (OSCC) has not been completely elucidated. The purpose of the present study was to investigate the prevalence and localization of HPV-16 virus in OSCC and to correlate HPV-16 positivity and p16INK4A expression with the clinical and pathological features of OSCC. METHODS: The archives of Oral Pathology at the University of Florida, College of Dentistry were accessed for demographic, clinical, histopathological data, and slides of 114 OSCC patients. HPV-16 positivity of OSCC was evaluated by p16INK4A immunohistochemistry (IHC) and HPV-16 E6/E7mRNA by in situ hybridization (ISH). RESULTS: Out of 114 consecutive pathological slides of OSCC, 16 samples (14%) showed positivity for p16INK4A by IHC and 14 samples (12%) were positive for HPV-16 E6/E7mRNA ISH and the Positivity showed a significant correlation with the patients' age, alcohol consumption, and the degree of OSSC differentiation. The hard palate showed the highest positivity of p16INK4A IHC and HPV-16 mRNA ISH (38%, 36% respectively). CONCLUSION: HPV-16 is a significant factor in oral carcinogenesis. We recommend using p16INK4A as a surrogate marker for HPV detection in OSCC, which can be complemented by RNA ISH for the identification of HPV subtypes.

Oral Melanoacanthoma: Case Series of 33 Cases and Review of the Literature.

BACKGROUND: Oral melanoacanthoma (OM) is a rare, reactive, and benign proliferation of two cell types: keratinocytes and melanocytes. Biopsy is mandatory to not only confirm the diagnosis but also, rule out other entities, as clinical correlation simply, is not definitive. AIM OF THE STUDY: We present a large series of OM with analysis of demographics, clinical appearance, histologic presentation, and review of the literature. To the best of our knowledge, this is the largest series of oral OM reported to date. MATERIALS AND METHODS: Following IRB approval, cases diagnosed as OM within the archives of the University of Florida Oral Pathology Biopsy Service (1998-2020) were included. Patient age, gender, location, clinical appearance, clinical impression, and duration of each lesion was collected. RESULTS: A total of 33 cases were included with a mean age of 38.7 years (range of 5-73), and a female: male ratio of 2.6:1. The most common location in descending order was the buccal mucosa (n = 16, 48%), followed by palate (n = 11, 33%), tongue and labial mucosa (n = 2 each, 6%), maxillary and mandibular gingiva (n = 1 each, 3%). The lesions were most frequently brown/black in color, and most often described as macular. All cases were asymptomatic and reported duration was ranged from one week to twelve months. Clinical impression in descending order was pigmented macules (n = 15, 45%), melanosis (n = 4, 12%), nevus (n = 3, 9%), melanoma (n = 2, 6%), melanoacanthoma (n = 1, 3%), and racial pigmentation (n = 1, 3%). Ethnicity was only documented in only 6 out of 33 cases, of which 5 cases were African-American and one Caucasian. The majority of cases (n = 28, 84%) demonstrated hyperplastic/acanthotic surface epithelium with less common, atrophic (n = 4, 12.1%) or spongiotic epithelium (n = 2, 6.06%). CONCLUSION: The demographics and clinical presentation of OM in our series was similar to previous findings but encompasses wider variability of histologic presentation. Awareness of OM in the clinical and histologic differential diagnosis of pigmented lesions should be reinforced as many patients are concerned for melanoma and clinicians are often unware of this condition.

Primary mucosal melanomas of the head and neck are characterised by overexpression of the DNA mutating enzyme APOBEC3B.

AIMS: Primary head/neck mucosal melanomas (MMs) are rare and exhibit aggressive biologic behaviour and elevated mutational loads. The molecular mechanisms responsible for high genomic instability observed in head/neck MMs remain elusive. The DNA cytosine deaminase APOBEC3B (A3B) constitutes a major endogenous source of mutation in human cancer. A3B-related mutations are identified through C-to-T/-G base substitutions in 5'-TCA/T motifs. Herein, we present immunohistochemical and genomic data supportive of a role for A3B in head/neck MMs. METHODS AND RESULTS: A3B protein levels were assessed in oral (n = 13) and sinonasal (n = 13) melanomas, and oral melanocytic nevi (n = 13) by immunohistochemistry using a custom rabbit α-A3B mAb (5210-87-13). Heterogeneous, selective-to-diffuse, nuclear only, A3B immunopositivity was observed in 12 of 13 (92.3%) oral melanomas (H-score range = 9-72, median = 40) and 8 of 13 (62%) sinonasal melanomas (H-score range = 1-110, median = 24). Two cases negative for A3B showed prominent cytoplasmic staining consistent with A3G. A3B protein levels were significantly higher in oral and sinonasal MMs than intraoral melanocytic nevi (P < 0.0001 and P = 0.0022, respectively), which were A3B-negative (H-score range = 1-8, median = 4). A3B levels, however, did not differ significantly between oral and sinonasal tumours (P > 0.99). NGS performed in 10 sinonasal MMs revealed missense NRAS mutations in 50% of the studied cases and one each KIT and HRAS mutations. Publicly available whole-genome sequencing (WGS) data disclosed that the number of C-to-T mutations and APOBEC3 enrichment score were markedly elevated in head/neck MMs (n = 2). CONCLUSION: The above data strongly indicate a possible role for the mutagenic enzyme A3B in head/neck melanomagenesis, but not benign melanocytic neoplasms.

A rare case of peripheral adenoid ameloblastoma with dentinoid.

Adenoid ameloblastoma with dentinoid (AAD) is an extremely rare central neoplasm of the gnathic bones with no reported case of peripheral AAD. Adenoid ameloblastoma with dentinoid was first reported by Slabbert et al. in 1992 under the term "dentinoameloblastoma." However, the Armed Forces Institute of Pathology introduced the name "adenoid ameloblastoma with dentinoid" in 1994. Histologically, AAD shows features of ameloblastoma and adenomatoid odontogenic tumor along with a calcified product resembling dentin. The biological behavior of central AAD is not well established, though, in the literature, several reports consider it to be among the more aggressive odontogenic neoplasms, with a propensity for local invasion and recurrence. The demographic characteristics, clinical features, behavior, and prognosis of the peripheral AAD (PAAD) are unknown. To the best of our knowledge, this is the first reported case of PAAD in a 62-year-old woman.

Changing Trends in Benign Human Papillomavirus (HPV) Related Epithelial Neoplasms of the Oral Cavity: 1995-2015.

Human papillomavirus (HPV)-related benign papillary epithelial neoplasms are common lesions affecting any region of the oral cavity. This study evaluated the trends in frequency, location, and demographics of these lesions over 20 years in a large biopsy service. Following IRB approval, the archives of UF Oral Pathology Biopsy Service between 1995 and 2015 were queried. Cases diagnosed as squamous papilloma, verruca vulgaris, and condyloma acuminatum were included. Extraoral locations, inconclusive diagnoses, or syndrome-related HPV lesions were excluded. Age, gender, location, clinical presentation, and diagnoses were recorded. Data from one calendar year per 5-year span was assessed including the years 1995, 2000, 2005, 2010, and 2015. A total of 1458 cases were identified over the total 5 calendar years assessed. Papilloma as a percentage of total biopsies per year was as follows: 1995 (2.6%), 2000 (3.3%), 2005 (3.6%), 2010 (4.0%) and 2015 (4.5%), representing a 73% (1.9×) percentage increase. Males (56%) were affected more commonly; however, in patients under 19 years, a female predominance was observed. The overall percentage of lesions in females increased by 30.6% over the time frame. The mean age was 54 years (range 1-93 years) with an increase of 10 years over time. About 1.1% of patients had multifocal lesions and 0.2% had a recurrence. In descending order of frequency, the tongue, soft palate, and mandibular gingiva were most involved. Maxillary gingiva and lower lip were the most common locations in patients under 19. Location varied over time, however, the biggest increase was noted for lesions on the gingiva. Squamous papilloma was the most common histologic variant (93.6%). The incidence of benign HPV-related oral lesions increased substantially over the 2 decades studied. This increase was statistically significant with a p-value <0.00045. Other trends noted included increase in the following: the average age, female involvement, and gingival location. Our results indicate a trend for the overall increase in the prevalence of benign oral HPV lesions in our population.

Buccal bifurcation cyst: A case series and review of the literature.

INTRODUCTION: Buccal bifurcation cyst is an inflammatory odontogenic cyst and constitutes up to 5% of all odontogenic cysts. The aim of this study was to report a series of cases, review the recent literature, and facilitate recognition and proper treatment of this entity. METHODS: With institutional review board approval, the authors retrieved all archival cases of buccal bifurcation cyst from the oral pathology biopsy service from 1994 through 2018. Patient age and sex, cyst location, clinician's impression, radiographic appearance, diagnosis, and treatment data were recorded. RESULTS: A total of 10 cases were identified. Average patient age was 9 years. A slight male predilection was observed (n = 6, 60%). One hundred percent of cases were in the mandible, including 3 (30%) bilateral cases. CONCLUSIONS: Mandibular buccal bifurcation cyst is an important entity in pediatric patients but may be less likely to be recognized by clinicians not regularly treating children. The results of this study are mostly consistent with the literature. Treatment is typically via enucleation or even more conservative modalities, and extraction should be avoided if possible. PRACTICAL IMPLICATIONS: Buccal bifurcation cysts should be treated via enucleation or even more conservative methods. If possible, the affected teeth should be preserved.

Essentials of oral manifestations of leukemia for the dental practitioner.

Oral lesions may be the initial or only manifestation of leukemia and can be the key to early diagnosis. The varied nature of presenting signs and dentists' general lack of familiarity with oral presentations makes diagnosis challenging. This retrospective review reports a series of cases of leukemia to familiarize dentists with the oral manifestations and facilitate earlier diagnosis or recognition of relapse of this life-threatening disease. Following institutional review board approval, the University of Florida Oral Pathology Biopsy Service archive from 1994 to 2018 was queried for all oral biopsies resulting in a diagnosis of leukemia. Cases with insufficient diagnostic information or extraoral manifestations were excluded. Demographic, clinical, and histologic findings were tabulated. Ten cases with 12 biopsy sites were identified. Men (n = 6) were affected more commonly. The mean age of the patients was 58.4 years (range of 17 to 88 years). The gingiva was the most frequently biopsied site (n = 6; 50%). Importantly, 40% of the patients (n = 4) had no prior diagnosis of leukemia. A wide spectrum of clinical impressions was rendered, pyogenic granuloma being the most common, and the reported duration of lesions ranged from several weeks to 6 months. The rarity of patients presenting with leukemia may lead to low levels of clinical suspicion, misdiagnosis, and delays in treatment. However, oral lesions may be the first and only manifestation of leukemia, and clinicians should be aware of the clinical characteristics of these oral presentations to ensure early diagnosis and treatment, thereby helping to reduce disease-related morbidity and mortality.

Peripheral ameloblastic fibro-odontoma-Report of 2 cases of a very rare entity.

Ameloblastic fibro-odontoma (AFO) is a rare benign mixed odontogenic tumor that affects children and young adults. AFO occurs mainly intraosseous. Extraosseous AFO is extremely rare. We report 2 cases of rare peripheral ameloblastic fibro-odontoma in 2- and 12-year-old female patients. Microscopic examination revealed a benign proliferation of odontogenic epithelium associated with a dentinoid material distributed within a cell-rich mesenchymal stroma resembling dental papilla. Simple surgical excision of the lesion is usually curative. There was no recurrence after a short period of follow-up. Clinicians should be cognizant of this rare entity, which can be considered in a differential diagnosis of gingival growths that are noted in early childhood.

Adenomatoid odontogenic tumor: a series of 28 cases from a biopsy service.

OBJECTIVE: Adenomatoid odontogenic tumor (AOT) is a benign odontogenic tumor with an excellent prognosis, often seen in children and young adults. The aim was to examine the spectrum of clinical, radiographic, and histologic attributes of AOT and assess clinician recognition of this entity. In addition, diagnostic considerations and treatment modalities were explored. METHOD AND MATERIALS: With Institutional Review Board approval, archival cases of AOT from the University of Florida Oral Pathology Biopsy Service (1994-2019) were examined. Clinical and demographic data along with accompanying radiographs and original slides were reviewed. RESULTS: A total of 28 cases of AOT were identified. These were all solitary in nature, with a mean age of 20.6 years (range 12-67 years). Most patients were under 20 (75.0%) with a definite female predilection (64.3%). Anterior jaws remained the most common location (85.2%), with a higher maxillary predilection (57.1%). Clinical impression included odontogenic lesions such as dentigerous cyst, lateral periodontal cyst, and odontogenic keratocyst. CONCLUSION: The spectrum of features of AOT is described. As clinicians were unfamiliar with AOT, highly characteristic features of AOT and more unique variants are discussed extensively to improve diagnostic aptitude. Clinicians must remain aware of this entity, as treatment is minimal compared to other odontogenic entities.

Angina bullosa hemorrhagica: report of two cases.

Angina bullosa hemorrhagica (ABH) is a rare benign condition that affects the oral and oropharyngeal mucosa. It is characterized by a rapid eruption of one or more red or magenta blood-filled bullae, which typically involves the soft palate. ABH is a self-limiting condition that heals spontaneously usually within 2 weeks without scarring. ABH is not related to any dermatologic, hematologic, systemic disorders, or other known causes. The etiopathogeneses of ABH are unknown, though several theories have been proposed. Trauma has been suggested as a potential cause for the development of ABH in susceptible individuals. Two cases are presented of ABH, and the differential diagnoses of oral vesiculobullous conditions is discussed. Cognizance and identification of this benign condition is important to prevent misdiagnosis and eventual unwarranted treatment.

Central odontogenic fibroma in association with brown tumor of hyperparathyroidism in a patient with neurofibromatosis type 1.

We present a patient with bone abnormalities and a myriad of lesions secondary to his redeveloping renal failure and neurofibromatosis type 1 (NF1). A 21-year-old male renal transplant recipient with NF1 presented with painless masses and large, irregular radiolucent lesions in the maxilla and mandible. After histologic examination, the lesion was diagnosed as a central odontogenic fibroma (COdF) in association with a central giant cell lesion, most consistent with brown tumor of hyperparathyroidism. The bone changes were interpreted to be highly suggestive of renal osteodystrophy. Around 30 cases of hybrid central giant cell granuloma-like lesion in association with central odontogenic fibroma have been reported. This, to our knowledge, is the first reported case of brown tumor in association with COdF. Our case provides further evidence of the giant cell component as the initiating entity in these hybrid lesions.

The Diagnostic Performance of Early Sjögren's Syndrome Autoantibodies in Juvenile Sjögren's Syndrome: The University of Florida Pediatric Cohort Study.

Objectives: The aim of this study was to evaluate the clinical validity of early Sjögren's syndrome (SS) autoantibodies (eSjA), which were originally marketed for early diagnosis of SS, for juvenile SS (JSS) in a recently identified pediatric cohort. Methods: A total of 105 symptomatic subjects with eSjA results available were evaluated at the Center for Orphaned Autoimmune Disorders at the University of Florida and enrolled for this study. JSS diagnosis was based on the 2016 ACR/EULAR SS criteria. Demographic/clinical/laboratory parameters were compared between JSS (n = 27) and non-JSS (n = 78) for % positivity, sensitivity, and specificity of eSjA (SP1, anti-salivary protein; CA6, anti-carbonic anhydrase VI; PSP, anti-parotid secretory protein) and classic SS-autoantibodies (cSjA; ANA, SSA/SSB, RF, and others) either alone or in combination. Associations between eSjA and diagnostic/glandular parameters were also determined by Fisher's exact test. Results: Compared to non-JSS, JSS patients exhibited sicca symptoms demonstrating reduced unstimulated salivary flow rate (USFR) and abnormal glandular features revealed by salivary gland ultrasound (SGUS). Among cSjA, ANA demonstrated the highest sensitivity of 69.2%, while SSA, SSB, and RF showed around 95% specificities for JSS diagnosis. The % positive-SSA was notably higher in JSS than non-JSS (56% vs. 5%). Of eSjA, anti-CA6 IgG was the most prevalent without differentiating JSS (37%) from non-JSS (32%). Sensitivity and specificity of eSjA were 55.6 and 26.9%, respectively. Autoantibodies with potentially applicable specificity/sensitivity for JSS were seen only in cSjA without a single eSjA included. There were no associations detected between eSjA and focus score (FS), USFR, SSA, SGUS, and parotitis/glandular swelling analyzed in the entire cohort, JSS, and non-JSS. However, a negative association between anti-PSP and parotitis/glandular swelling was found in a small group of positive-SSA (n = 19, p = 0.02) whereas no such association was found between anti-PSP-positive compared to anti-PSP-negative. JSS and non-JSS groups differed in FS, USFR, and EULAR SS Patient Reported Index Dryness/Mean in CA6/PSP/ANA, SP1, and SSA-positive groups, respectively. Additionally, a higher FS was found in RF-positive than RF-negative individuals. Conclusions: eSjA underperformed cSjS in differentiating JSS from non-JSS. The discovery of clinical impact of eSjA on early diagnosis of JSS necessitates a longitudinal study.

Labial salivary gland assessment in idiopathic pulmonary fibrosis patients with sicca symptoms.

OBJECTIVE: Patients with idiopathic pulmonary fibrosis (IPF) commonly present with sicca symptoms. This study aimed to assess labial minor salivary glands (LMSGs) in those patients to rule out Sjögren's syndrome (SS), in which sicca symptoms are the clinical hallmark. STUDY DESIGN: Cases of patients with IPF with sicca symptoms referred to the oral medicine clinic at the University of Florida within the last 13 years were selected with institutional review board approval. Demographic characteristics, clinical findings, laboratory results, and histomorphologic parameters were retrospectively analyzed. RESULTS: A total of 12 patients (9 men and 3 women, ages 55-76 years) were identified. History of exposure to asbestos or chemicals, smoking, and medication information was obtained. All patients reported sicca symptoms with 57% of those exhibiting objective or borderline dryness. Anti-SSA/Ro and anti-SSB/La were positive in 25% and 8% of the cases, respectively. Microscopically, 1 out of 12 patients was biopsy positive in the absence of anti-SSA/Ro, fulfilling the 2016 SS criteria with positive sialometry. CONCLUSIONS: A LMSG biopsy is critical to identify SS in patients with diagnosed IPF and present sicca symptoms, especially those with negative serology, as revealed in our study.