Pulmonary artery involvement in Behçet's disease: A challenging case and comprehensive management approach.

A 26-year-old male with Behçet's disease (BD) presented with recurrent oral and genital ulcers, bilateral pneumonia, and a left lower pulmonary artery aneurysm. Endovascular coil embolization was initially performed, followed by treatment with prednisone, colchicine, and azathioprine. Despite treatment, disease progression occurred, requiring additional embolization, intravenous pulse methylprednisolone, and cyclophosphamide. Ultimately, a combination of medical and endovascular interventions resolved the pulmonary arterial aneurysms. This case highlights BD's systemic inflammatory nature and vascular complications like pulmonary artery aneurysms. It emphasizes the importance of early detection and individualized, multidisciplinary care for such complications.

Giant Cell Arteritis Presenting as Bilateral Cotton Wool Spots.

An 81-year-old Afro-Caribbean woman presented with a two-week history of a dull headache in her temples, jaw claudication especially when chewing food, and reduced vision in her eyes, more pronounced in the right eye. There was no past medical or family history of hypothyroidism or autoimmunity. On examination, the vision was counting fingers in the right eye and 6/36 in the left eye, best corrected. Dilated fundus examination revealed multiple peripapillary cotton wool spots in both eyes though more pronounced in the right. Her erythrocyte sedimentation rate (ESR) was 120 mm/h, and her C-reactive protein (CRP) level was 79 mg/L. A temporal artery ultrasound scan was undertaken immediately which demonstrated a halo sign around both temporal arteries and so a giant cell arteritis (GCA) diagnosis was made. The patient was commenced on daily high-dose IV methylprednisolone 1 g for three days and referred to the rheumatology team. Her vision improved to 1/60 right and 6/9 left eye best corrected at three days post-treatment. At 12 months after the initial presentation, her vision stabilized at 6/60 in the right and 6/6 with complete visual fields in the left eye. Cotton wool spots can be a sign of GCA. Their appearance with or without characteristic systemic symptoms should prompt urgent evaluation.

Paediatric Behçet's Disease: A Comprehensive Review with an Emphasis on Monogenic Mimics.

Behçet's disease (BD) is a polygenic condition with a complex immunopathogenetic background and challenging diagnostic and therapeutic concepts. Advances in genomic medicine have provided intriguing insights into disease pathogenesis over the last decade, especially into monogenic mimics of BD. Although a rare condition, paediatric BD should be considered an important differential diagnosis, especially in cases with similar phenotypes. Emerging reports of monogenic mimics have indicated the importance of genetic testing, particularly for those with early-onset, atypical features and familial aggregation. Treatment options ought to be evaluated in a multidisciplinary setting, given the complexity and diverse organ involvement. Owing to the rarity of the condition, there is a paucity of paediatric trials; thus, international collaboration is warranted to provide consensus recommendations for the management of children and young people. Herein, we summarise the current knowledge of the clinical presentation, immunopathogenetic associations and disease mechanisms in patients with paediatric BD and BD-related phenotypes, with particular emphasis on recently identified monogenic mimics.

Giant cell arteritis: A case report and review of literature.

Giant cell arteritis, the most common form of vasculitis in the elderly, is characterized by granulomatous inflammation of arteries, which can lead to serious, life-threatening conditions including aortic aneurysms, ruptures, and dissections as well as blindness. Since GCA can be treated by immunosuppressant therapy, such as corticosteroids, early diagnosis and treatment may reduce the risk of serious disability and morbidity. While temporal artery biopsy is considered the gold standard to diagnosis giant cell arteritis, it is intrusive with inherent risks as well as unreliable due to tissue sampling. Imaging studies, such as computerized tomography, are nonintrusive and have been shown to identify vasculitis including giant cell arteritis. We present a case of a 72-year-old male patient who was diagnosed with giant cell arteritis by temporal artery biopsy during surgery for aortic aneurysm and coronary artery bypass graft. Computerized tomography imaging studies, prior to the surgery and biopsy, were suggestive of vasculitis. This case serves to emphasize the beneficial role of imaging studies to assess vasculitis, including giant cell arteritis, that can be done prior to the progressive development of more serious debilitating and potentially fatal pathology.

The Epidemiology and Clinical Manifestations of Takayasu Arteritis: A Descriptive Study of Case Reports.

Takayasu's arteritis is a rare form of chronic inflammatory disorder involving large vessels, with an unclear etiology. Common early signs and symptoms are weakness, malaise, and fever. Takayasu's arteritis mainly involves the aorta and its branches as well as the subclavian and carotid arteries. While radiologic methods can identify diseased vessels, they can't tell the difference between active and chronic lesions. This study reviews the characteristics of Takayasu's arteritis to identify any possible changes in the prevalence of symptoms of the disease. We conducted a literature review of case reports on Takayasu arteritis from PubMed and Google Scholar. Variables of interest were age, gender, symptoms, blood pressure (BP) measurement, diminished pulses, and radiological findings. Data were transferred to an Excel spreadsheet (Microsoft Corporation, Redmond, WA), and mean, median, and standard deviation, frequencies, and proportions were calculated using R version 1.1.456 (RStudio: Integrated Development for R. RStudio, PBC, Boston, MA). There were 43 cases, and females accounted for 88.3% of the presentations. The average age was 25 years, SD 12.5 years. Fever was the most frequent symptom (20.93%), followed by chest pain (13.95%), claudication (13.95%), and headache (13.95%). Less frequent complaints included shortness of breath (11.62%), weight loss (9.30%), syncope (6.98%), and night sweats (4.65%). On the right side, the average BP was 142/87 mmHg, and the left-sided finding averaged 115/72 mmHg. Decreased pulses were primarily seen in the radial artery with 15 cases. Radiological findings showed narrowing of the vessels in the following order: aorta (22), carotid (11), renal (10), subclavian (9), celiac (2), mesenteric (2), axillary (2), and tibial (1). The characteristics of Takayasu's arteritis were analyzed in this study. It identified several findings, ranging from fever symptoms to the signs of claudication, as well as the involvement of major vessels, such as the aorta and its branches, and a summary of radiological findings. This depicts the picture of Takayasu's arteritis and what physicians should expect when dealing with the disease.

Cerebral Vasculitis in a COVID-19 Confirmed Postpartum Patient: A Case Report.

COVID-19 has primarily been reported as a respiratory illness, but involvement of other organ systems has been reported. We describe a case of a postpartum with COVID-19 who had cerebral vasculitis. The patient presented with headache, blurring of vision, right-sided body weakness, and incoordination. Cranial magnetic resonance imaging (MRI) and angiography (MRA) showed a small acute hemorrhage on the left occipital lobe with associated acute subarachnoid hemorrhage along the parietal and occipital convexities and bilateral moderate to severe narrowing of the cerebral vessels. The patient was discharged asymptomatic. On follow-up, patient had no residual neurologic deficits, and repeat cranial MRI/MRA showed complete resolution of the vasculitis. This report was compatible with the pattern of viral-induced vasculitis and provides support to the mechanism of COVID-19-associated neurologic manifestation.

Neuro-Behcet's Disease Mimicking a Cerebral Tumor: A Case Report

We report a rare case of neuro-Behcet's disease (NBD) presenting as an inflammatory pseudotumor in the brain. A 52-yr-old woman was evaluated for subacute dizziness and headache. Brain magnetic resonance (MR) imaging showed a right cerebellar mass, which disappeared 2 weeks later. After a year, recurrent mucocutaneous manifestations of Beh et's disease were observed. Immunosuppressant and steroid maintenance treatment were started. She experienced two more neurologic attacks and brain MR imaging revealed an enhancing mass in the right temporal lobe. The second attack showed a good response to steroid pulse therapy, but the third attack did not respond to steroid and her neurologic signs suggested an impending transtentorial hernia. The right temporal lobectomy was performed for the purpose of life-saving. The pathologic finding of the mass was a chronic inflammatory vasculitis, compatible with NBD.