BACKGROUNDChronic periaortitis is a rare entity characterized by a fibroinflammatory tissue surrounding most commonly abdominal aorta and iliac arteries, which can spread to retroperitoneum, and is of unknown etiology. Other vascular districts which may be involvedalbeit rarely-are the coronary, renal, mesenteric and celiac arteries. Firstly, it was thought to be caused by a local inflammatory reaction to antigens in the atherosclerotic plaques in the aorta, but now it has been considered a primary inflammatory or immune mediated disorder because of its association with immunoregulatory diseases such as systemic lupus erythematosus (SLE), Sjögren's syndrome and systemic vasculitis.
BACKGROUNDRemitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare inflammatory disease of unknown etiology, characterized by edema of the extremities and arthritis, which mainly affects patients over 50 years of age.Rheumatoid factor and anti-CCP are usually negative. The syndrome can be isolated or related with other diseases. There are reports of paraneoplastic association with prostate, lung, lymphoproliferative and gastrointestinal tumors. This patient was diagnosed with gastrointestinal stromal tumor (GIST), for which there are no publications with a direct relation with RS3PE. Patients diagnosed with RS3PE usually present good response to corticosteroids, as was the case of our patient. CASE REPORTA 63-year-old man, previously hypertensive and heavy drinker was admitted for severe COVID-19 infection, needing intubation and hemodialysis. During hospitalization, he developed knee monoarthritis, and an arthrocentesis revealed cloudy synovial fluid, 7,429 leukocytes/µL, with negative gram stain and culture. He was treated as possible septic arthritis due to prolonged hospitalization, multiple infections and use of broad-spectrum antibiotic therapy, with improvement After medical discharge, the patient developed symmetrical polyarthritis of the hands, feet, ankles and knees, prolonged morning stiffness, pitting edema of hands and feet. An extensive investigation was carried out in the outpatient clinic and reactive arthritis, gout, and rheumatoid arthritis were excluded. Investigation of possible associated neoplasia was initiated, due to persistent iron deficiency anemia, weight loss, and melena. Diagnosis of GIST was made by computed tomography of the abdomen and upper digestive endoscopy, and it was surgically resected. The patient was treated with prednisone 10 mg/day and showed improvement in the joint condition.
BACKGROUNDA 27-year-old female patient, complaining of migraine with aura for the least 2 months, developed sudden vertigo, headache, tinnitus, ear fullness with hearing loss, and visual loss. There was no mental confusion. Fundoscopy demonstrated retinal detachment and central retinal artery branches occlusion. CASE REPORTFemale, 27 years old, with a previous history of hypothyroidism, was admitted with hearing and visual loss, headache and vertigo. She denied fever, alopecia, arthritis, rash, livedo or weakness. Ophthalmological investigation showed campimetry with altitudinal deficit to the right, accompanied by ipsilateral visual deficit. Fundoscopy demonstrated retinal detachment and central retinal artery branches occlusion. Audiometry demonstrated sensorineural loss on the right, and with this, the Susac's syndrome triad (encephalopathy, retinal arterial occlusion and sensorineural hearing loss) was confirmed. Cerebrospinal fluid (CSF) examination was unremarkable, echocardiogram did not show vegetations, and carotid Doppler scan was normal. Imaging of central nervous system revealed a typical Susac's syndrome in CNS MRI ("snowball pattern''). CONCLUSIONSusac's syndrome is a rare entity, with less than 400 cases described worldwide. It comprises the triad of encephalopathy, sensorineural hearing loss and retinal artery occlusion. It is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina and inner ear, with antiendothelial cell antibodies playing an important role in the lesion. It may also present with myalgia, arthralgia, livedo reticularis, livedo racemosa, which makes this diagnosis of importance for the rheumatologist. It can be misdiagnosed as multiple sclerosis, disseminated encephalomyelitis, central nervous system vasculitis, and systemic lupus erythematosus. Treatment requires potent immunosuppression, with pulses of methylprednisolone associated with rituximab, cyclophosphamide or mycophenolate.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.