Ocular and orbital manifestations of granulomatosis with polyangiitis: a systematic review of published cases.

Objective: Granulomatosis with polyangiitis (GPA) is an autoimmune disorder characterized by necrotizing granulomatous inflammation of small and medium-sized vessels. This systematic review aimed to highlight the most common ophthalmic manifestations and to uncover their associations with antineutrophil cytoplasmic antibody (ANCA) positivity and the presence of granulomas. Methods: A literature search of PubMed, Web of Science, and Scopus electronic databases was performed from journal inception to March 21, 2021, for case reports and a series of ophthalmic GPAs. Cytoplasmic-ANCA (c-ANCA), perinuclear-ANCA (p-ANCA), and granulomas were analyzed against many ophthalmic signs and symptoms. 306 patients with GPA were retrospectively studied. Results: Granulomas were present in 47.7% of our sample, c-ANCA in 59.2%, and p-ANCA in 10.8%. Scleritis was significantly associated with higher odds for c-ANCA positivity. Eye discharge, episcleritis, proptosis, and central nervous system (CNS) involvement were each significantly associated with lower odds for c-ANCA positivity. Orbital mass was significantly associated with lower odds for p-ANCA positivity. CNS involvement was significantly associated with higher odds for p-ANCA positivity (OR:3.08, 95% CI:1.02, 9.36, p=0.047) and orbital mass was significantly associated with lower odds for p-ANCA positivity. Conclusions: We recommend that clinicians should consider ocular or orbital GPA in patients presenting with non-specific eye complaints, such as vision impairment, orbital mass, or proptosis, and obtain further assessments to determine the possible presence of granuloma, c-ANCA, or p-ANCA. Abbreviations: GPA = Granulomatosis with Polyangiitis, ANCA = antineutrophil cytoplasmic antibody, c-ANCA = cytoplasmic-ANCA, p-ANCA = perinuclear-ANCA, CNS = central nervous system, AAVs = ANCA-associated vasculitides, SD = standard deviation, GU = genitourinary, ENT = ear nose and throat, OR = odds ratio, CI = confidence interval.

A Case of Concurrent Disseminated Coccidioidomycosis and Embryonal Carcinoma When Lice and Fleas Coexist.

Coccidioidomycosis (CM) is a fungal infection endemic to the southwestern United States with a wide range of clinical presentations depending on the infected organ systems. Most infections are asymptomatic. Coccidioidomycosis causes a primary pulmonary infection and when symptoms occur, they most often resemble community-acquired pneumonia. One percent of cases disseminate, typically via hematogenous or lymphatic spread. It is in these cases that more severe symptoms may present and potentially overlap with those characteristics of other systemic illnesses. This is a case of CM disseminated to lymph nodes in a 24-year-old man with concomitant metastatic embryonal carcinoma. It is difficult to identify the primary etiology for many components of this patient's presentation, including diffuse lymphadenopathy and multiple pulmonary nodules. Furthermore, the relationship between these 2 concurrent disease processes is not entirely clear. Factors that may contribute include the well-known phenomenon of locus minoris resistentiae (LMR) or potentially a shared immune failure between infectious organisms and malignant cells.

"To Fix a Broken Heart": An Unusual Case of Infective Endocarditis Involving the Mitral Valve With Perforation and Hemodynamic Instability.

Infective endocarditis (IE), commonly caused by Staphylococcus aureus, can affect multiple cardiac structures and lead to significant morbidity and mortality. We present a case of IE with extensive mitral valve involvement causing perforation and hemodynamic compromise. A 66-year-old Caucasian female presented to the emergency department for progressive altered mental status and lethargy. The patient and family denied history of intravenous drug use (IVDU) on interview. Physical exam revealed tachypnea, tachycardia, lethargy, and fluctuance in the right antecubital fossa draining serous fluid. Initial studies revealed a urinary tract infection, patchy bilateral opacities on chest x-ray, hypoxic respiratory failure, elevated lactate and cardiac markers, leukocytosis, and positive urine toxicology for opioid and benzodiazepine. She was intubated and admitted to the ICU, and later developed acute respiratory distress syndrome with requirement for vasopressors. Antibiotics were started, and blood cultures ultimately grew methicillin-sensitive S. aureus. Coronavirus disease 2019 (COVID-19) results were negative. Cardiology was consulted for elevated cardiac markers that were due to myocardial injury in the setting of septic shock. A transthoracic echocardiogram showed a large mobile mass on the anterior mitral leaflet. Further evaluation with transesophageal echocardiogram revealed a large, mobile, and centrally necrotic vegetation on the medial portion of the mitral annulus extending to both the anterior and posterior leaflets. Doppler of the valve showed holosystolic retrograde ejection into the left atrium confirming a perforation. The patient was transferred urgently to a cardiothoracic surgery capable center for operative intervention on the mitral valve. IE is most commonly caused by S. aureus and seen in highest rates among patients with a prosthetic valve, congenital heart disease, and intracardiac device. However, roughly 50% of IE occurs in patients without any valvular disease. Other risk factors include IVDU, valvular disease, and prior history of endocarditis. Clinical diagnosis of IE is made using the Duke's criteria, with echocardiogram and bacteremia playing a major role. The initial management involves empiric antibiotics until a pathogen is identified. Surgical consult is also suggested, and indications for surgery include heart failure due to valve dysfunction, uncontrolled infection, prevention of embolism, and hemodynamic compromise. Prompt recognition and intervention is crucial in the prevention of mortality in patients with IE leading to mitral perforation and hemodynamic compromise.