Rheumatologic disorders in patients undergoing esophageal manometry: prevalence, symptom characteristics, and manometric findings.

Rheumatologic disorders (RDs) can have gastrointestinal (GI) manifestations. Systemic sclerosis (SSc) patients often have upper GI symptoms from absent esophageal contractility (AC). Upper GI symptom characteristics and high-resolution esophageal manometry with impedance (HREMI) findings of other RDs have not been well studied. We aimed to: (i) determine the prevalence of RD in patients undergoing HREMI and (ii) assess the symptom characteristics and manometric findings of these patients. Patients undergoing HREMI (July 2018 to March 2020) rated their GI symptoms' severity. Healthy volunteers (HVs) also underwent HREMI. Of the 1,003 patients, 90 (9%) had RD (mean age: 55.3 ± 1.4 years, 73.3% females), most commonly SSc (n = 27), rheumatoid arthritis (RA, n = 20), and systemic lupus erythematosus (SLE, n = 11). The most severe upper GI symptoms in patients with RD were heartburn, regurgitation, nausea, and dysphagia, with no significant differences in their severities between SSc, RA, and SLE. RD patients had higher upper esophageal sphincter (UES) pressures, lower distal contractile integral (DCI), lower bolus clearance, and more frequent hiatal hernia (HH) on HREMI (all P < 0.05) than HVs. Over half (61.1%) of patients with RD had esophageal motility disorders, most commonly AC (n = 25), ineffective esophageal motility (IEM; n = 18), and esophagogastric junction (EGJ) obstructive disorders (n = 11). Among patients undergoing HREMI, 9% had RD. Upper GI symptom severities did not distinguish different RDs. Patients with RD had higher UES pressures, weaker DCI, lower bolus clearance, and more frequent HH than HVs. Although AC and IEM were most common motility disorders, a considerable minority (12.2%) of our RD patients had EGJ obstructive disorders.

Acute vascular insufficiency of intestine: incidence highest in summer, outcomes worst in winter.

BACKGROUND: The incidence of acute vascular insufficiency of intestine (AVII) is on the rise in the USA and is associated with significant morbidity and mortality. Seasonal variations have been observed in the onset of several gastrointestinal diseases. It is thus far unknown whether the incidence, in-hospital mortality rates, and length of hospital stay (LOS) of AVII vary in different seasons. AIMS: The aims of this study were to study the seasonal variations in the (1) incidence, (2) in-hospital mortality, and (3) LOS of AVII in the USA. METHODS: We used the Nationwide Inpatient Sample to identify patients aged ≥ 18 years hospitalized from the years 2000-2014. We used the Edwards recognition with estimation of cyclic trend method to study the seasonal variation of AVII hospitalizations and z test to compare the seasonal incidences (peak-to-low ratio), mortalities, and LOS. RESULTS: A total of 1,441,447 patients were hospitalized with AVII (0.3% of all hospitalizations). Patients with AVII were older (69.0 ± 0.1 vs 56.9 ± 0.1) and more commonly females (65.4% vs 35.5%) than patients without AVII (p < 0.001). The incidence of AVII increased through the summer to peak in September (peak/low ratio 1.028, 95% CI 1.024-1.033, p < 0.001). Patients with AVII hospitalized in winter had the highest mortality (17.3%, p < 0.001) and LOS (9.2 ± 0.7 days, p < 0.001). CONCLUSIONS: The incidence of AVII in the USA peaks in late summer. The in-hospital mortality rates and LOS associated with AVII are the highest in winter. Physicians could be cognizant of the seasonal variations in the incidence, in-hospital mortality, and LOS of AVII.

Sex and Racial Differences in Systemic Lupus Erythematosus Among US Adults in the All of Us Research Program.

OBJECTIVE: Men with systemic lupus erythematosus (SLE) are an understudied population. The present study characterized differences between men and women with SLE. METHODS: We examined cross-sectionally participants with SLE in the All of Us Research Program, a US cohort with a participant survey at enrollment (May 2018 to June 2022) and linked electronic health record (EHR) data. We described and compared characteristics of men and women with SLE encompassing disease manifestations and prescribed medications from EHR data and socioeconomic factors, including health literacy and health care access and utilization, from surveys. We reported racial variations stratified by sex. RESULTS: Of 1,462 participants with SLE, 126 (9%) were male. Men reported lower educational attainment and less fatigue than women. Myocardial infarction was significantly more common in men. Men had significantly less confidence in completing medical forms than women and exhibited a trend toward requiring more help in reading health-related materials. Barriers to health care access and utilization were common in both men and women (40% versus 47%, respectively, reporting some reason for delay in care; P = 0.35). Women of race other than Black or African American or White more often reported delaying care due to cultural differences between patient and provider. CONCLUSION: Our study demonstrated major clinical and health literacy differences in men and women with SLE. Socioeconomic factors were significant barriers to health care in both sexes. Our study suggests men have disproportionately poorer health literacy, which may exacerbate preexisting disparities. Further large prospective studies, focusing on recruiting men, are needed to better characterize racial differences in men with SLE.

Sector (partial) oculo(dermal) melanocytosis in 89 eyes.

PURPOSE: To describe sector (partial) involvement of the uvea with melanocytosis. DESIGN: Noninterventional, retrospective case series. PARTICIPANTS: A total of 89 eyes of 86 patients. METHODS: Review of medical records, color photographs, and ultrasound images. MAIN OUTCOME MEASURES: Clinical features and relationship with uveal melanoma. RESULTS: Approximately all patients were Caucasian (n = 83, 97%), and sector melanocytosis involved the right (n = 41, 46%) or left (n = 48, 54%) eye. The involved tissue included iris (n = 58, 65%), choroid (n = 48, 54%), and both iris and choroid (n = 17, 19%). The melanocytosis affected a mean of 6 clock hours of iris and 5 clock hours of choroid. Related melanocytosis involved the sclera (n = 39, 44%), eyelid (n = 4, 4%), temple (n = 4, 4%), scalp (n = 1, 1%), and palate (n = 1, 1%). Uveal melanoma was found at presentation in 7 patients (8%) and was multifocal in 2 of these patients. A comparison of eyes with versus without melanoma revealed clinically significant factors (odds ratio [OR] > 2) of male gender (71% vs. 43% [OR 3.36]); cutaneous/palate melanocytosis (14% vs. 7% [OR 2.11]); scleral melanocytosis heaviest in superior, temporal, or nasal quadrants (57% vs. 29% [OR 2.41, confidence interval, 2.24-3.92]); and any degree of choroidal melanocytosis (86% vs. 70% [OR 2.63]), particularly diffuse choroidal melanocytosis (29% vs. 16% [OR 3.85]). None of these factors reached statistical significance in this small cohort. Over a mean follow-up of 6 years, there was no metastatic event. CONCLUSIONS: Ocular melanocytosis can be sectoral (partial), affecting only a mean of 5 to 6 clock hours of the uvea and can manifest melanoma within the melanocytosis region. There were no specific features of melanocytosis statistically related to the presence of melanoma. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Anti-Signal Recognition Particle Antibody-Associated Severe Interstitial Lung Disease Requiring Lung Transplantation.

Anti-signal recognition particle (SRP) antibodies are typically associated with immune-mediated necrotizing myopathy. Some patients with anti-SRP antibodies may have extramuscular manifestations including mild respiratory symptoms secondary to interstitial lung disease. We present a case of a 40-year-old female presenting with acute hypoxic respiratory failure secondary to anti-SRP antibody-associated interstitial lung disease with mildly elevated creatinine kinase but without evidence of necrotizing myopathy on muscle biopsy. After a complicated six-month hospitalization, the patient successfully received double lung transplantation and was eventually discharged on room air. Unexplained worsening interstitial infiltrates leading to persistent hypoxic respiratory failure in the setting of nonspecifically elevated creatinine kinase should warrant consideration of an underlying connective tissue disease, including myositis with anti-SRP antibody-associated interstitial lung disease. In rare cases, interstitial lung disease may be severe requiring lung transplantation.

Porencephalic cyst: a rare cause of new-onset seizure in an adult.

We present a case of a 56-year-old male with a history of perinatal intracerebral hemorrhage who presented to the emergency department after a witnessed new-onset generalized tonic-clonic seizure. Computerized tomography and magnetic resonance imaging of the head revealed a large frontal lobe porencephalic cyst, with encephalomalacia in the right parietal lobe and temporal lobe (the patient did not have any prior cranial imaging). The patient has subsequently remained seizure-free on levetiracetam. Porencephalic cyst is a rare condition of cerebrospinal fluid accumulation in the brain parenchyma that is usually related to perinatal vascular events. These cysts can have a wide array of clinical presentations. This can include partial or generalized seizures, which are usually managed by antiepileptics.

A case report of emphysematous gastritis in a diabetic patient: favorable outcome with conservative measures.

Emphysematous gastritis (EG) is a rare cause of abdominal pain, which should be differentiated from gastric emphysema. It is hypothesized to result from air-producing microorganisms in patients with underlying predisposing factors. Because of the non-specific presentation of EG, it is diagnosed radiographically. CT scan is the diagnostic modality of choice that typically reveals irregular, mottled appearance of the air in the thickened gastric wall and in the portal vein in the liver. We report a rare case of EG in a male with a history of diabetes mellitus who presented to the emergency department with diarrhea, nausea, vomiting, and epigastric pain. On examination, he was hypotensive and had mild tenderness in the epigastrium. Laboratory tests revealed leukocytosis, elevated lactate, anion gap metabolic acidosis, and acute kidney injury. A non-contrast CT abdomen revealed findings consistent with EG. Even though mortality rate in access of 60% have been reported without prompt surgical intervention in EG, recent literature suggests favorable prognosis with conservative measures in patients without an overt surgical indication. Our patient was also managed conservatively with IV antibiotics and gradual advancement of diet and had complete resolution of symptoms over the ensuing few days. The factors that correlate with a poor prognosis include elevated serum lactate, serum creatinine, and concomitant pneumatosis in small bowel and colon.

Group d salmonella urinary tract infection in an immunocompetent male.

A 62-year-old male with past medical history of benign prostatic hyperplasia presented to the emergency department with complaints of decreased urinary flow, inability to fully empty his bladder, and gross hematuria. Physical examination was unremarkable. Urinalysis revealed large amount of blood and more than 700 white blood cells suggesting a urinary tract infection. Urine culture grew group D Salmonella greater than 100,000 colony-forming units per mL. He was prescribed 6 weeks of trimethoprim/sulfamethoxazole and had resolution of symptoms. Retrospectively, he reported a 3-day history of watery diarrhea about a week prior to onset of urinary symptoms that was presumed to be the hematogenous source in this case. Urinary tract infection from nontyphoidal Salmonella (NTS) is rare and is usually associated with immunosuppression, chronic diseases, such as diabetes or structural abnormalities of the genitourinary tract. Genitourinary tract abnormalities previously reported in the literature that predispose to nontyphoidal Salmonella urinary tract infection include nephrolithiasis, chronic pyelonephritis, retrovesicular fistula, urethrorectal fistula, hydrocele, and post-TURP. We present an exceedingly uncommon case of 62-year-old male with group D Salmonella urinary tract infection predisposed by his history of benign prostatic hyperplasia.

IVC filter perforation through the duodenum found after years of abdominal pain.

BACKGROUND: The number of IVC filter-related complications has increased with their growing utilization; however, IVC filter perforation of the duodenum is rare. It can manifest with nonspecific abdominal pain, gastrointestinal bleeding, cava-duodenal fistula, or small bowel obstruction. CASE REPORT: A 67-year-old female presented with several years of right upper quadrant abdominal pain which was exacerbated by movement and food intake. She had a history of hepatic steatosis, cholecystectomy, and multiple DVTs with inferior vena cava filter placement. Physical exam was unremarkable. Laboratory tests demonstrated elevated alkaline phosphatase and transaminases. Esophagogastroduodenoscopy revealed a thin metallic foreign body embedded in the duodenal wall and protruding into the duodenal lumen with surrounding erythema and edema, but no active hemorrhage. Further evaluation with non-contrast CT scan revealed that one of the prongs of her IVC filter had perforated through the vena cava wall into the adjacent duodenum. Exploratory laparotomy was required for removal of the IVC filter and repair of the vena cava and duodenum. Her post-operative course was uneventful. CONCLUSIONS: In patients with history of IVC filter placement with non-specific abdominal pain, a high clinical suspicion of IVC filter perforation of the duodenum should be raised, as diagnosis may be challenging. CT scan and EGD are valuable in the diagnosis. Excellent outcomes have been reported with open surgical filter removal. Low retrieval rates of IVC filters have led to increased complications; hence, early removal should be undertaken as clinically indicated.

Thyroid follicular carcinoma presenting as metastatic skin nodules.

BACKGROUND: Follicular thyroid cancer (FTC) metastasizes most commonly to the lungs and non-cranial bones. Skull and skin are uncommon sites and usually manifest well after the diagnosis of primary malignancy. Metastasis to skull and skin as the presenting feature of FTC is infrequently reported in the literature. CASE PRESENTATION: A 65-year-old Caucasian woman with a history of thyroid nodule presented with the complaint of rapidly growing skull nodules which had been present for 3 years but were stable previously. She denied any fevers, chills, history of trauma, or weight loss. She denied any history of smoking or head and neck irradiation. On physical examination, she had two non-tender gray cystic lesions - one on her left temporal region and the other on the right parietal region. Biopsy was consistent with metastatic FTC. Magnetic resonance imaging of the brain demonstrated 7.1×3.8 cm and 3.7×4.5 cm fairly homogeneous, enhancing, relatively well-defined masses centered in the posterior and left anterior lateral calvarium with intracranial and extracranial extensions but without any vasogenic edema or mass effect on the brain. Thyroid ultrasound showed numerous nodules in both lobes. The patient underwent a total thyroidectomy. Histopathological studies of the thyroid gland revealed a well-differentiated FTC in the left lobe. Then she underwent resection of the tumor in multiple stages. She did not have any recurrence of the FTC or metastases during the follow-up period and will be receiving radioactive iodine treatment. CONCLUSION: Bone and lung are the common sites of metastasis from FTC, but involvement of skull or skin is unusual, particularly as the presenting feature. Metastases from FTC should be in the differential of patients with new osteolytic hypervascular skull lesions or cutaneous lesions in head and neck area.

Jugulotympanic paraganglioma: a rare cause of vertigo.

BACKGROUND: Jugulotympanic paraganglioma generally presents in the 5th or 6th decades of life with tinnitus and hearing loss. In this manuscript, we present a rare case of jugulotympanic paraganglioma presenting in the 9th decade with vertigo as the most bothersome symptom. CASE REPORT: An 83-year-old woman presented with worsening episodes of dizziness of a few months duration. She also complained of tinnitus and hearing loss, more severe on the left side. Examination revealed a red bulging left-sided tympanic membrane, conductive hearing loss, and a bruit at the base of the skull. Dix-Hallpike test was negative. CT head and MRI brain revealed findings consistent with a large left-sided jugulotympanic paraganglioma, which was found to be hormonally inactive on laboratory tests. The patient underwent treatment with radiotherapy, which resulted in partial improvement of symptoms. CONCLUSIONS: Jugulotympanic paraganglioma may manifest in the elderly with the chief complaint of intermittent vertigo, as in our case. A red bulging mass on otoscopy raises the suspicion, necessitating further investigations, including CT and MRI.

Recurrent C. difficile in a Patient with IgG Deficiency.

IgG deficiency can predispose to recurrent pyogenic infections. The association of IgG deficiency with Clostridium difficile infection has been infrequently reported in the literature. We present a case of a middle-age woman with multiple hospitalizations for recurrent C. difficile in a short span of time which prompted consideration of a possible fecal transplant. On evaluation, she was found to have low total IgG, with subclass analysis revealing low IgG1 and IgG3. She was started on monthly infusions of immunoglobulins and one year after her last episode of C. difficile she has not had any recurrence. The role of immunoglobulin infusion in the treatment of recurrent C. difficile is controversial, with some studies revealing no clear evidence of benefit. Our case report suggests that the patients who have underlying IgG deficiency may benefit from immunoglobulin, as this can significantly reduce the incidence of recurrent infections and hence save the healthcare costs.

A rare initial presentation of gastric cancer with multiple osteolytic lesions.

Gastric cancer is the 12th leading cause of cancer-related deaths in the United States and commonly metastasizes to the bones. However, the presentation of gastric cancer as bony metastases without preceding gastrointestinal symptoms is rare which has been infrequently reported in the literature. Moreover, leptomeningeal carcinomatosis is an unusual complication of gastric cancer accounting for less than 1 percent of these patients. We present a unique case of a middle aged male who presented to the emergency department with worsening backache which started one month priorly. The only abnormal laboratory test was an elevated alkaline phosphatase of 154 IU/L. The imaging of his spine showed osteolytic lesions which on biopsy revealed signet ring cells. A small 2 cm ulcerated mass was found on esophagogastroduodenoscopy at the gastric cardia which on biopsy revealed signet ring gastric carcinoma. The patient received chemotherapy with capecitabine and oxaliplatin as well as radiation and showed a good response initially. A few months later, he presented with persistent worsening headaches and on brain imaging was found to have leptomeningeal carcinomatosis. Ten months after the diagnosis of gastric carcinoma, he passed away.

Epibulbar osseous choristoma in 8 patients.

PURPOSE: To describe the clinical features and histopathology of epibulbar osseous choristoma in a series of patients. METHODS: Noncomparative case series with chart review of 8 patients. RESULTS: At presentation, the mean age of the patients was 19 years (median, 19 years; range, 0.8-38 years), with 6 women and 2 men (6 Caucasian, 1 Hispanic, and 1 African American). There were no related systemic syndromes, and visual acuity was unaffected in all cases. The choristoma was superotemporal (n = 8, 100%), with epicenter located in the fornix (n = 8, 100%), deep to Tenon fascia (n = 8, 100%), and of yellow (n = 2, 25%), white (n = 2, 25%), or pink (n = 4, 50%) color. The mean basal dimension was 9 mm (median, 10 mm; range, 3.5-14 mm), and mean thickness was 4 mm (median, 4 mm; range, 2-5 mm). Four cases were managed with observation and 4 with surgical excision, revealing tumor base adherent to the episclera (n = 2, 50%) or loose within Tenon fascia (n = 2, 50%). There were no cases to demonstrate intrascleral involvement, growth, or recurrence. CONCLUSIONS: Epibulbar osseous choristoma is a congenital lesion of mature bone located superotemporally in the fornix at the level of Tenon fascia or episclera in young patients.