Current perspective on infections and mitigation strategies in primary systemic vasculitis.

PURPOSE OF REVIEW: The purpose of this review is to summarize and evaluate most recent evidence on the epidemiology of infections and associated risk factors in patients with primary systemic vasculitides (PSV), as well as discuss mitigation strategies including the risk of antibiotic prophylaxis. RECENT FINDINGS: Infections remain one of the leading causes of mortality in patients with PSV, with rates of severe infection ranging from 16 to 40% in different cohorts. Older age, frailty, renal and pulmonary involvement, and higher burden of comorbidities have been recognized as important patient-associated risk factors. Treatments including higher cumulative doses of glucocorticoids are associated with an increased risk of infections, and recent studies show the potential benefit of interventions such as reduced-dose glucocorticoid regimens. Existing mitigation strategies include screening, vaccination, and infection prophylaxis. The latter remains particularly important for Pneumocystis jirovecii pneumonia; however, the benefit-risk ratio seems to be less clear outside of induction phase (i.e., high dose of glucocorticoids) and optimal treatment duration remains less clear. Patients with PSV are at increased risk of infections, due to disease itself, comorbidities, and treatment side effects. Awareness of the timing and types of infection, as well as mitigation strategies are imperative to ensure treatment success and survival for patients.

The risk and severity of stroke in patients with atrial fibrillation and gout: A National Representative Database study.

BACKGROUND: It has been shown that gout is associated with left atrium remodeling and a pro-inflammatory state leading to the development of atrial fibrillation (AF). There is limited evidence whether gout increases the risk of stroke in patients with AF. We assessed the incidence of gout and the risk of stroke in patients with AF. METHODS: This is a retrospective cohort study using the 2016 US National Inpatient Sample (NIS) based on ICD-10 codes. The outcomes of the study were the risk and severity of new stroke in patients with pre-existing AF and gout. RESULTS: In 2016, we identified 3 844 057 patients admitted to the hospitals in NIS with history of AF, of which 240 875 had history of gout. Patients with AF and gout have higher risk of new stroke (OR 2.07 [1.97-2.19], P < .001), and this risk remains significantly elevated after adjusting for CHADS2VASC score variables, chronic kidney disease, dyslipidemia, obesity, and race (OR 1.10 [1.01-1.11], P = .041). However, presence of gout in patients with AF was not associated with all-cause in-hospital mortality, need for mechanical ventilation, percutaneous gastrostomy tube insertion, or discharge to skilled nursing facility. CONCLUSION: Subjects with AF and gout compared to AF alone had an increased risk of new stroke, but presence of gout was not associated with stroke severity. There is a potential role of gout as a risk factor or a risk marker for stroke in subjects with AF.

Lupus Never Fails to Deceive US: A Case of Rowell's Syndrome.

BACKGROUND: Rowell's syndrome is comprised of the presentation of erythema multiforme- (EM-) like lesions in association with lupus erythematosus (LE), along with serologies of speckled antinuclear antibodies (ANAs), positive rheumatoid factor (RF), positive anti-La/anti-Ro, and the clinical finding of chilblains. As per the redefined criteria by Zeitouni et al., three major criteria in addition to at least 1 minor criterion are necessary for diagnosis. Case Presentation. A 20-year-old male presented with a one-week history of worsening nonpruritic erythematous maculopapular skin rash (resembling EM) which appeared on the face and subsequently spread to the trunk, arms, legs, palms, and soles. There was no mucosal involvement. At the onset of rash, the patient reported headaches, associated with photosensitivity and intermittent fevers. Workup for viral meningitis yielded negative results. Laboratory investigation revealed mild anemia, elevated inflammatory markers, a positive ANA with speckled pattern, a positive anti-Ro/SSA, anti-La/SSB antibodies, and a positive rheumatoid factor (RF). Lupus anticoagulant antibody was positive along with a low-positive anticardiolipin IgM antibody and a negative beta-2 glycoprotein antibody. Anti-dsDNA, anti-Smith, anti-Jo-1, anti-centromere, and anti-Scl-70 antibodies were negative. Hepatitis serologies, herpes simplex virus 1 and 2, mycoplasma, Epstein-Barr virus, HIV, and parvovirus B19 were negative. Left arm skin biopsy demonstrated vacuolar interface dermatitis and positive colloidal iron stain suggestive of dermal mucin deposition, favoring the diagnosis of cutaneous collagen vascular disease. Cutaneous lesions improved with administration of oral prednisolone. CONCLUSION: Rowell's syndrome should be considered in patients who present with cutaneous LE and lesions resembling EM. Further serological markers should be pursued in the absence of obvious EM-precipitating factors.

Acute myocardial infarction on Nongated chest computed tomography.

Contrast-enhanced chest computed tomography (CT) is not considered part of the evaluation of myocardial infarction. However, acute myocardial infarction has been detected on contrast-enhanced chest CT as areas of decreased myocardial enhancement in patients evaluated for other indications, such as pulmonary embolism and aortic dissection. We present a case of acute myocardial infarction on a nongated chest CT in a 67-year-old male who presented with atypical chest pain and initial nondiagnostic electrocardiogram. This case highlights that acute myocardial infarction may be detectable on contrast-enhanced CT. When evaluating contrast-enhanced chest CT's for other etiologies of chest pain, radiologists should look for potential myocardial perfusion abnormalities that can provide clues to the presence of myocardial infarction.

Bullous Lupus: An Atypical Case of Refractory Disease in a Patient with Sulfa Allergy.

Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous autoimmune disorder characterized by rapid, widespread vesiculobullous lesions in patients with Systemic Lupus Erythematosus (SLE). BSLE can present as the initial manifestation of SLE and may be a marker of severe disease. In this case report, we present a case of a 22-year-old African American woman with BSLE and impaired renal function with subsequent nephrotic range proteinuria concerning for lupus nephritis and autoimmune hemolytic anemia, refractory to systemic corticosteroids, immunoglobulin, and mycophenolate mofetil, requiring dapsone after careful desensitization due to prior history of angioedema with sulfa drugs. This case highlights the importance of the prompt recognition of BSLE as the initial manifestations of SLE and illustrates the association of BSLE with severe disease and the benefit of concomitant use of dapsone with corticosteroids and other immunosuppressant drugs, even in patients with a history of sulfa allergy.

A Case of Sudden Death: Subarachnoid Hemorrhage, Pheochromocytoma, Berry Aneurysm.

Subarachnoid hemorrhage is a medical emergency. Berry aneurysm rupture is the second most common cause following trauma. Diagnosis is often challenging. Neurogenic heart syndrome often complicates subarachnoid hemorrhage. A concomitant pheochromocytoma can be deadly causing sudden cardiac arrhythmia. Here, we describe a case of subarachnoid hemorrhage with concomitant incidental pheochromocytoma in a relatively young female who died suddenly, diagnosed during autopsy. A 57-year-old Hispanic woman with past medical history of asthma, prediabetes, and uncontrolled hypertension collapsed unexpectedly. She initially had ventricular tachycardia, followed by pulseless electrical activity and finally asystole without response to resuscitation. In the emergency department she was on epinephrine, calcium, naloxone, and tPA with suspected thrombotic stroke. Despite measures, she was pronounced dead. Autopsy revealed subarachnoid hemorrhage due to a ruptured berry aneurysm. Additionally, pheochromocytoma was detected in the right adrenal gland. Subarachnoid hemorrhage has a grave prognosis by itself. This case describes the uncommon detection of pheochromocytoma in the setting of subarachnoid hemorrhage.