COVID-19 Exposure Unmasking Systemic Amyloidosis With Hepatic Predominance.

Amyloidosis is characterized by depositing insoluble fibrillar proteins that misfold into beta-pleated sheets. This phenomenon occurs on a systemic or local level and may interfere with the function of various organs, including the heart, kidneys, and liver. Among those presenting with amyloidosis, hepatic, gastrointestinal, renal, cardiac, vitreous, and immunological involvement may occur. These manifestations are linked to several clinical presentations, varying from abdominal pain and hepatomegaly to restrictive cardiomyopathy and chronic renal failure. The two most common types of amyloid proteins are amyloid light chain (AL) and serum amyloid A (AA) proteins. AL produced by immunoglobulin light chains kappa and lambda (κ, λ) circulate systemically and accumulate in organs. At the same time, serum AA proteins are acute-phase reactants seen in infectious, chronic inflammatory states. In an immune-mediated infection such as COVID-19, serum AA levels may be a predictive factor of disease severity and a valuable biomarker to monitor the clinical course of COVID-19 patients. This report highlights a case in which infection with COVID-19 provoked an effective immune response that may have contributed to the accelerated progression of systemic amyloidosis with hepatic involvement. The study further investigates the involvement of AL and AA proteins in COVID-19 infections, including their role in synergistically exacerbating an already grueling clinical course.

Hemi-central retinal vein occlusion as a rare manifestation of the hypercoagulable state in COVID-19.

To date, COVID-19 has no definite effective targeted therapy, and management is primarily supportive. Central retinal vein occlusion (CRVO) is frequently caused by systemic risk factors posing hypercoagulable states. In April 2020, a female patient with a history of hypertension, diabetes mellitus and chronic kidney disease presented with 2 days of loose, watery stools, nasal congestion and severe lethargy. The patient denied dyspnoea or fever. A week after the initial symptoms, the patient reported decreased vision from the left eye. Dilated funduscopy and fluorescein angiography suggested hemi-CRVO. The patient refused intravitreal antivascular endothelial growth factor agents because of non-severe visual loss. Testing was positive for COVID-19 IgG antibodies; reverse transcription PCR was not available. Vision improved within 3 weeks of presentation. We recommend that clinicians keep a high suspicion for acute onset of thrombotic events in patients with COVID-19 and thrombotic predisposing risk factors.

Fulminant Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis After 10 Years of Hydralazine Use.

Vasculitis, by definition, causes changes in the walls of blood vessels, including thickening, weakening, narrowing, and scarring, leading to inflammation and necrosis of the blood vessel walls. Small-vessel vasculitis is commonly associated with anti-neutrophil cytoplasmic antibodies (ANCA), which activate cytokine-primed neutrophils and monocytes that express ANCA antigens proteinase 3 (PR3) and myeloperoxidase (MPO) on their surface. The continuous injury and inflammation of these small vessels characterized by circulating immune complexes and antinuclear antibodies result in clinical features standard in all types of vasculitis. When a 59-year-old male with a history of heart failure, hypertension (on hydralazine 100 mg every eight hours for more than ten years), diabetes mellitus, and dyslipidemia presented to the hospital, he was complaining of hematuria, intermittent periumbilical abdominal pain, and 40-lb weight loss over four months. Initial evaluation showed symptomatic anemia and large blood cells with proteinuria on urine analysis. During his clinical course, the patient developed a new diffuse purpuric rash. Imaging showed systemic involvement with ground-glass opacities, diffuse alveolar hemorrhage, and peripancreatic inflammatory changes, consistent with small-vessel vasculitis. Immunological tests confirmed ANCA-associated vasculitis, and kidney biopsy showed ANCA-mediated pauci-immune glomerulonephritis supported by the salvage technique used by pronase immunofluorescence, which provides evidence against the glomerular disease of the complex immune type in the setting of MPO-ANCA seropositivity. Despite the withdrawal of hydralazine and prompt initiation of immunosuppressive therapy and alternating sessions of plasmapheresis, the patient succumbed to acute massive pulmonary hemorrhage and subsequent demise. We recommend that patients on the common antihypertensive, hydralazine, should be monitored with non-specific inflammatory markers and, if warranted, with qualitative and quantitative assessment tools to measure inflammatory disease activity for possible complications of hydralazine drug-induced vasculitis or hydralazine ANCA-associated vasculitis (HAAV). Furthermore, cumulative dosages may be a predisposing factor for HAAV to present as a pulmonary-renal syndrome, which can be fulminant and fatal, despite aggressive efforts. Therefore, screening, revisiting therapy, early diagnosis, and prompt discontinuation of the drug are imperative.

"Two for One", Novel Dual Left Anterior Descending Artery (LAD) Variant: Type XIII.

Dual left anterior descending artery (LAD) is a rare phenomenon that occurs in less than one percent of the population. To date, 12 variants have been identified. Proper identification of coronary vessels is crucial in emergent situations that require prompt action, such as percutaneous coronary intervention (PCI). We propose that our case highlights a novel 13th (type XIII) variant. We present the case of a 57-year-old African American woman with a past medical history of hypertension, glaucoma, cerebral vascular accident, dyslipidemia who presented to the ED complaining of atypical chest pain for one day duration. Electrocardiography showed normal sinus rhythm at 60 beats per minute (bpm), normal axis, normal intervals, no acute ischemic changes, and an isolated T wave inversion in DIII. Cardiac markers were within normal limits. The patient was started on aspirin 81mg, atorvastatin 40mg, and restarted on amlodipine 5mg. Echocardiography showed a left ventricular ejection fraction (LVEF): 65%, normal right ventricular size and systolic function, mild mitral valve regurgitation, and mild aortic regurgitation. Computed tomographic (CT) angiography showed a novel subtype of dual LAD, the left circumflex and right coronary arteries were patent. The patient was discharged once stabilized and advised to follow up with cardiology. Dual LAD describes a rare anatomic variant in which two coronary branches, known as short and long LAD arteries, supply the territory normally supplied by the solitary LAD artery. To date, 12 variants of dual LAD, classified by origin and course of the short and long LAD arteries, have been described in the literature. To the best of our knowledge, the current case describes a novel subtype of dual LAD, variant XIII. The LAD originates as usual from the left main coronary artery (LMCA) and initially runs in the anterior interventricular groove for a short course before bifurcating into two long LADs which both leave the interventricular groove and course out to the apex. One of the vessels courses laterally and the other courses medially of the interventricular groove. It is pertinent to identify the coronary vessels accurately before certain interventions are taken. Acknowledgement of this phenomenon can help guide accurate management in the future for patients with this condition.