A String of Pearls: Linear Immunoglobulin A (IgA) Bullous Dermatosis in the Setting of Imipramine Use and Newly Diagnosed Ulcerative Colitis.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune disease affecting children or adults that leads to subepithelial vesiculobullous lesions on the skin and/or mucosa. Due to the histologic and clinical appearance of the disease with tense and pruritic blisters, direct immunofluorescence is required for diagnosis, which features the characteristic linear deposition of IgA autoantibodies along the basement membrane zone. LABD can be idiopathic, drug-induced, or associated with a systemic disease such as inflammatory bowel disease. Many drugs have been implicated, such as antibiotics, anti-hypertensives, anti-epileptics, analgesics, and immunosuppressive medications. Treatment of LABD centers on discontinuation of the offending drug, if applicable, as well as pharmacotherapy with dapsone as the first-line treatment. Adjunctive therapy with sulphonamides, systemic corticosteroids, cyclosporine, colchicine, intravenous immunoglobulins, tetracyclines, erythromycin, and dicloxacillin has also shown benefits. We report the case of a young adult patient who developed LABD with a background of recent initiation of treatment with imipramine and newly diagnosed ulcerative colitis.

Predictors of remission in rheumatoid arthritis patients treated with biologics: a systematic review and meta-analysis.

Biologics have emerged as an effective treatment of rheumatoid arthritis (RA). However, there is a significant proportion of patients who fail to respond to biologics. Identifying the predictors that affect the response to biologics remains challenging. A comprehensive literature search of PubMed, Embase, and Web of Science databases was conducted through May 1, 2022. We included all studies that used a multivariate model to assess for the predictors of remission in RA patients treated with biologics. We calculated pooled odds ratios (OR) with 95% confidence intervals (CI) for risk factors reported in ≥ 3 studies using a random-effects model. A total of 16,934 patients with RA who were treated with biologics were included in twenty-one studies. Our study showed that old age (OR 0.98 (0.97, 0.99), P < 0.00001), female gender (OR 0.66 (0.56, 0.77), P < 0.00001), smoking history (OR 0.86 (0.75, 0.99), P 0.04), obesity (OR 0.95 (0.91, 0.99), P 0.02), poor functional status (OR 0.62 (0.48, 1.27), P < 0.00001), high disease activity (OR 0.90 (0.85, 0.96), P 0.0005), and elevated erythrocyte sedimentation rate (OR 0.99 (0.98, 1.00), P 0.009) were poor predictors of remission. On the other hand, positive anti-citrullinated protein antibodies (OR 2.52 (1.53, 4.12), P 0.0003) was associated with high remission rate. Old age, female gender, obesity, smoking history, poor functional status, high disease activity, and elevated ESR at the time of diagnosis have been associated with poor response to biologics. Our findings could help establish a risk stratification model for predicting the remission rate in RA patients receiving biologics.

Atypical Gradenigo Syndrome in an Elderly Man Resolved with Mastoidectomy and Petrous Apicectomy.

Gradenigo syndrome (GS) was described primarily in the paediatric population, especially in the pre-antibiotic era. GS is rarely reported in the elderly population, especially in the post-antibiotic era. We present the rare case of a 67-year-old man who presented with an incomplete triad of symptoms (without abducens nerve palsy) of GS that failed medical therapy and was successfully treated with surgical intervention (mastoidectomy and petrous apicectomy). Physicians should be familiar with atypical presenting symptoms of GS as it can lead to life-threatening complications, especially in the elderly. GS cases resistant to medical therapy may require prompt appropriate imaging studies and surgical intervention. LEARNING POINTS: Gradenigo syndrome may present with an incomplete triad (without abducens nerve palsy), especially in the post-antibiotic era.Gradenigo syndrome can rarely affect immunocompromised elderly patients.Gradenigo syndrome cases resistant to medical therapy may require prompt appropriate imaging studies and surgical intervention (mastoidectomy and petrous apicectomy).

Clinical significance of micronutrient supplements in patients with coronavirus disease 2019: A comprehensive systematic review and meta-analysis.

BACKGROUND AND AIMS: Micronutrient supplements such as vitamin D, vitamin C, and zinc have been used in managing viral illnesses. However, the clinical significance of these individual micronutrients in patients with Coronavirus disease 2019 (COVID-19) remains unclear. We conducted this meta-analysis to provide a quantitative assessment of the clinical significance of these individual micronutrients in COVID-19. METHODS: We performed a comprehensive literature search using MEDLINE, Embase, and Cochrane databases through December 5th, 2021. All individual micronutrients reported by ≥ 3 studies and compared with standard-of-care (SOC) were included. The primary outcome was mortality. The secondary outcomes were intubation rate and length of hospital stay (LOS). Pooled risk ratios (RR) and mean difference (MD) with corresponding 95% confidence intervals (CI) were calculated using the random-effects model. RESULTS: We identified 26 studies (10 randomized controlled trials and 16 observational studies) involving 5633 COVID-19 patients that compared three individual micronutrient supplements (vitamin C, vitamin D, and zinc) with SOC. Nine studies evaluated vitamin C in 1488 patients (605 in vitamin C and 883 in SOC). Vitamin C supplementation had no significant effect on mortality (RR 1.00, 95% CI 0.62-1.62, P = 1.00), intubation rate (RR 1.77, 95% CI 0.56-5.56, P = 0.33), or LOS (MD 0.64; 95% CI -1.70, 2.99; P = 0.59). Fourteen studies assessed the impact of vitamin D on mortality among 3497 patients (927 in vitamin D and 2570 in SOC). Vitamin D did not reduce mortality (RR 0.75, 95% CI 0.49-1.17, P = 0.21) but reduced intubation rate (RR 0.55, 95% CI 0.32-0.97, P = 0.04) and LOS (MD -1.26; 95% CI -2.27, -0.25; P = 0.01). Subgroup analysis showed that vitamin D supplementation was not associated with a mortality benefit in patients receiving vitamin D pre or post COVID-19 diagnosis. Five studies, including 738 patients, compared zinc intake with SOC (447 in zinc and 291 in SOC). Zinc supplementation was not associated with a significant reduction of mortality (RR 0.79, 95% CI 0.60-1.03, P = 0.08). CONCLUSIONS: Individual micronutrient supplementations, including vitamin C, vitamin D, and zinc, were not associated with a mortality benefit in COVID-19. Vitamin D may be associated with lower intubation rate and shorter LOS, but vitamin C did not reduce intubation rate or LOS. Further research is needed to validate our findings.

Triglyceride Glucose Index for the Prediction of Subclinical Atherosclerosis and Arterial Stiffness: A Meta-analysis of 37,780 Individuals.

Insulin resistance (IR), which can be assessed by triglyceride-glucose (TyG) index, is a major contributor to the pathogenesis of cardiovascular diseases. Arterial stiffness is an index of subclinical atherosclerosis. We conducted this systematic review and meta-analysis to summarize the existing studies and provide a quantitative assessment of the significance of the TyG index in predicting the incidence of subclinical atherosclerosis and arterial stiffness. A comprehensive literature search in PubMed, EMBASE, and Web of Science databases from inception until April 30, 2022 was conducted. Published observational studies that evaluated the association between TyG index and arterial stiffness among the adult population and reported odds ratio (OR) for this association after multivariate analysis were included. The random-effects model was used for the estimation of pooled ORs with the corresponding confidence intervals (CIs). A total of 9 observational studies, including 37780 participants, were included. Seven out of the 9 studies analyzed the TyG index as a categorical variable and showed a statistically significant association between TyG index and incident arterial stiffness (pooled OR 1.96, 95% CI 1.52-2.53, P<0.00001, I2=82%). Additionally, similar results were in the 3 studies that analyzed TyG index as a continuous variable (pooled OR 1.37, 95% CI 1.26-1.49, P<0.00001, I2=0%). In conclusion, our meta-analysis demonstrates that a higher TyG index is associated with higher odds of subclinical atherosclerosis and arterial stiffness. TyG index may be used as an independent predictor of an increased risk of subclinical atherosclerosis and arterial stiffness.

Esophagopericardial Fistula After Esophagectomy.

Esophagectomy is the mainstay surgical treatment for esophageal carcinoma. The operation can be complicated by an anastomotic stricture, anastomotic leak, recurrent laryngeal nerve injury, conduit ischemia, and chylothorax. Rarely, esophagectomy can be complicated by fistula formation between the gastrointestinal tract and the nearby structures. We describe a case of esophagopericardial fistula after esophagectomy. A 50-year-old man presented to the emergency room with a chest pain of two-week duration associated with sweating, chills, and poor appetite. He was diagnosed with stage III esophageal adenocarcinoma four months ago. He had received neoadjuvant chemotherapy followed by distal esophagectomy, partial gastrectomy, and placement of a jejunostomy tube one month before presentation. Cardiovascular examination was significant for jugular venous distention and distant heart sounds. Chest CT angiography showed pneumomediastinum concerning for anastomotic leak. Esophagram finally confirmed an esophagopericardial fistula. A drain was placed into the pericardial space followed by emergent esophageal stent placement. Eventually, he underwent a cervical esophagostomy and placement of a jejunostomy tube. The patient was later discharged home in a stable condition. In conclusion, esophagopericardial fistula is a rare adverse event of esophagectomy. Esophageal stenting could be useful as a temporary or definite treatment.

Henoch-Schonlein Purpura Presenting as Upper Gastrointestinal Bleed in an Adult Patient.

Henoch-Schonlein purpura (HSP) is a multi-system autoimmune disease that is relatively common in pediatric patients. HSP usually manifests as palpable purpura, arthralgia, abdominal pain, and acute kidney injury. Here, we present a case of an adult male with hematemesis as the initial presenting symptom of HSP. A previously healthy, 18-year-old Caucasian male presented with a one-day history of hematemesis associated with abdominal pain and non-bloody diarrhea. He also reported bilateral knee and ankle arthralgias with a painless skin rash on both lower extremities. Physical exam was positive for palpable, purpuric, non-blanchable skin rash involving bilateral lower extremities. Notable labs on admission included a white cell count of 10.8 x 109/L and C-reactive protein of 4.8 mg/L. Upper endoscopy showed non-bleeding erosive gastropathy and duodenal erosions. Skin biopsy of the left leg showed immunoglobulin A (IgA) deposition within the walls of the superficial dermal vessels. The patient was started on intravenous methylprednisolone 500 mg daily followed by a steroid taper. Due to incomplete clinical response to steroids, mycophenolate mofetil 1000 mg twice daily was added and maintained for three months. His symptoms improved significantly, and he no longer complained of abdominal pain or diarrhea. Gastrointestinal manifestations are common in HSP patients. However, the diagnosis will be challenging when these symptoms precede other classical manifestations of HSP. History and physical exam are key components in accurately diagnosing HSP; nevertheless, skin biopsy remains the gold standard to confirm the diagnosis.

Gastric amyloidosis in a patient with dysphagia.

Dysphagia is a symptom with a broad differential diagnosis. Usually, the etiology of dysphagia is benign, but it is essential to rule out serious causes. It is also critical to think outside the box when the etiology is not obvious. Herein, we describe a case of multiple myeloma that initially presented with dysphagia. An 81-year-old male patient presented with dysphagia to solid food associated with anorexia and weight loss of 22 kg over the last 6 months. The patient looked chronically ill and cachectic. Upper endoscopy showed patchy erythema in the gastric antrum. Gastric biopsy was consistent with gastric amyloidosis. Although serum and urine protein electrophoresis did not show a monoclonal (M) band, immunofixation did show elevated free kappa light chains and elevated free Kappa/Lambda ratio. Bone marrow biopsy was consistent with multiple myeloma. Although gastrointestinal involvement is common in amyloidosis, it is unusual for amyloidosis to initially present in the gastrointestinal tract. Identification and treatment of the underlying condition, e.g., multiple myeloma, can lead to regression of gastrointestinal amyloidosis.

Efficacy and safety of anticoagulation in non-malignant portal vein thrombosis in patients with liver cirrhosis: a systematic review and meta-analysis.

BACKGROUND: The role of anticoagulation in treating non-malignant portal vein (PV) thrombosis (PVT) in patients with liver cirrhosis remains unclear. In our meta-analysis, we aimed to evaluate the efficacy and safety of anticoagulation for the treatment of non-malignant PVT in these patients. METHODS: We conducted a meta-analysis to estimate the effects of anticoagulation on non-malignant PVT in patients with liver cirrhosis. We assessed the rates of PV recanalization, variceal bleeding, and any bleeding. RESULTS: We included 9 observational studies which involved 474 patients. The rate of PV recanalization was significantly higher in patients who received anticoagulation compared to those who did not: 65.2% vs. 25.2%; relative risk (RR) 2.31, 95% confidence interval (CI) 1.80-2.96; P<0.00001. Variceal bleeding was significantly lower in patients who received anticoagulation: 0.1% vs. 18.5%; RR 0.15, 95%CI 0.04-0.55; P=0.004. Any bleeding was similar between patients who received anticoagulation and those who did not: 10.3% vs. 22.7%; RR 0.43, 95%CI 0.09-1.99; P=0.28. CONCLUSIONS: Anticoagulation use increased the rate of PV recanalization in cirrhotic patients with non-malignant PVT. Anticoagulation decreased the rate of variceal bleeding and did not increase the rate of any bleeding.

Short versus standard peroral endoscopic myotomy for esophageal achalasia: a systematic review and meta-analysis.

BACKGROUND: Peroral endoscopic myotomy (POEM) is increasingly used to treat esophageal achalasia, but is associated with a high rate of gastroesophageal reflux disease (GERD). The aim of our meta-analysis was to compare short and standard POEM in terms of clinical success and postoperative GERD. METHODS: We conducted a systematic review and meta-analysis of studies that compared POEM using short myotomy with standard myotomy. The primary outcome was clinical success. Secondary outcomes were postoperative GERD, perioperative complications, operation time, and length of hospital stay. A random-effects model was used to calculate the risk ratios (RR), mean differences (MD), and confidence intervals (CI). A P-value <0.05 was considered statistically significant. RESULTS: We included 5 studies involving 474 esophageal achalasia patients. Short and standard myotomies were similar in terms of clinical success (RR 1.02, 95%CI 0.97-1.09), perioperative complications (RR 0.68, 95%CI 0.26-1.75), and length of hospital stay (MD 0.25 days, 95%CI -0.14-0.63). Operation time was shorter for short myotomy (MD -15.01 mins, 95%CI -20.34 - -9.67). Although reflux symptoms were similar (RR 0.94, 95%CI 0.51-1.74), short myotomy had a lower risk of reflux esophagitis on endoscopy (RR 0.61, 95%CI 0.39-0.98), and pathologic acid exposure on pH monitoring (RR 0.58, 95%CI 0.36-0.94). CONCLUSIONS: POEM using a shorter myotomy is comparable with standard myotomy in terms of efficacy and safety in the short-term setting. A short myotomy requires a shorter operation time and might reduce the occurrence of postoperative GERD.

Combined achalasia and cricopharyngeal achalasia in a patient with type 1 myotonic dystrophy: a case report.

Type 1 myotonic dystrophy (MD) is a rare inherited disease which presents with skeletal muscle weakness and myotonia. Involvement of smooth muscles is also common and mainly manifests in the gastrointestinal tract. We report a case of type 1 MD who presented with dysphagia and was found to have unique esophageal manometry findings. A 57-year-old male patient presented with dysphagia for the last few months. Past medical history was significant for type 1 myotonic muscular dystrophy, gastroesophageal reflux disease, diaphragmatic paralysis, and obstructive sleep apnea. Both his father and brother died in their 50s because of unclear respiratory problems. He was a former smoker and did not drink alcohol. Review of systems was unremarkable. His neurological examination was significant for bilateral facial muscle weakness and mild ptosis. He had atrophy and weakness of the distal upper and lower extremities. Deep tendon reflexes were absent. Upper endoscopy and 24-hour esophageal pH testing were non-diagnostic. Finally, esophageal manometry revealed elevated lower esophageal sphincter (LES) pressure, elevated upper esophageal sphincter (UES) pressure, and very week peristalsis of the esophageal body. Esophageal involvement is common in type 1 MD manifesting with dysfunction of UES, esophageal body, and LES. Manometry usually describes a reduced resting tone of the UES and LES. The patient had elevated LES pressure and week peristalsis of the esophageal body consistent with achalasia. He also had an elevated UES pressure consistent with cricopharyngeal achalasia. This is the opposite of what is expected in type 1 MD.