UNASSIGNED: Takayasu arteritis is a large-vessel vasculitis predominantly seen in young women. Lack of signs and symptoms in the early stage of the disease often delays the diagnosis and thus leads to significant morbidity and mortality. One severe complication that may arise is a significant narrowing of blood vessels, potentially leading to life-threatening ischemic repercussions.
UNASSIGNED: The authors present a case of a 29-year-old female who presented to our ER with features of left-sided hemiparesis and right-sided facial deviation. Computed tomography angiography and Carotid Doppler helped in making the diagnosis of Takayasu arteritis. She was managed with prednisolone and mycophenolate mofetil. She has been on a regular follow-up for the last year and is currently stable.
UNASSIGNED: Even being a rare scenario, stroke can be the initial presentation of Takayasu arteritis. Early diagnosis and management in young patients are vital in keeping the disease at bay and preventing physical, mental, and socio-economic adversities.

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A 33-year-old female was admitted to our department complaining of multifocal paresthesia and weakness of the upper and lower extremities that had developed over the previous three months. She had also been undergoing treatment for atopic dermatitis with dupilumab, an anti-interleukin 4/13 receptor antibody. A nerve conduction study revealed multifocal axonal sensorimotor neuropathy of bilateral limbs. On admission, a small erythema appeared on her right forearm, but it was atypical for vasculitic skin lesions due to its location and time course. Nonetheless, a biopsy revealed medium-sized vessel vasculitis. The patient was therefore diagnosed with vasculitic neuropathy caused by cutaneous arteritis. Methylprednisolone pulse therapy with prednisolone and azathioprine markedly improved her symptoms. A skin biopsy is useful when mononeuropathy multiplex is suspected, even if the skin findings are atypical for vasculitic rash.

Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis with a poor prognosis, characterised by inflammation and necrosis of medium-sized arteries. PAN patients can present with a wide range of systemic manifestations, whereas cutaneous arteritis (CA) is a restricted manifestation to skin of the disease with a more favourable prognosis. Thus, differentiation between PAN and CA is crucial. Here, we present two cases that were initially diagnosed as CA due to the limited presence of systemic symptoms, but were finally diagnosed as PAN through catheter-based angiography. Although contrast-enhanced computed tomography and computed tomographic angiography are increasingly used to diagnose PAN, neither case had any abnormal findings on these examinations. Our cases therefore underscore that catheter-based angiography is critical for differentiation between PAN and CA, even in cases with limited systemic symptoms.

Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions.

BACKGROUND: Vasculitis does not usually induce glandular tissue lesions. Herein, we present a case of vasculitis with a distribution similar to that of immunoglobulin-G4-related disease (IgG4-RD), including that of the submandibular and lacrimal glands, and periaortitis.
METHODS: A 70-year-old man presented with shortness of breath. Laboratory findings revealed an IgG4 level of 191 mg/dL, negative antineutrophil cytoplasmic antibody test, and C-reactive protein level of 8.33 mg/dL. Magnetic resonance imaging of the head and computed tomography of the neck revealed bilaterally enlarged submandibular and lacrimal glands. Neck-to-pelvis computed tomography revealed bilateral infiltrative shadows in the lower lobes of both lungs, mass shadows in both lungs, and periaortitis of the abdominal aorta extending to the common iliac artery. Thus, the patient was diagnosed with IgG4-related respiratory disease and periaortitis/periarteritis. Prednisolone was administered at a dose of 35 mg (0.6 mg/kg daily). The dose was gradually tapered while observing the effects of the treatment. Imaging findings indicated an improvement and the C-reactive protein and IgG4 levels decreased, indicating a successful treatment course. However, after reexamination of the pathological findings, the diagnosis changed from IgG4-RD to vasculitis. One year after treatment initiation, the patient symptoms have stabilized.
CONCLUSIONS: Vasculitis can present with lesions and pathological findings similar to those of IgG4-RD.

BACKGROUND: Eales\' disease is an idiopathic peripheral retinal vasculopathy characterized by retinal phlebitis, ischemia, retinal neovascularization, and recurrent vitreous hemorrhages. But CRAO is an unusual presentation.
METHODS: A 27-year-old healthy female nurse of Indian descent presented with sudden vision loss in her right eye upon awakening. Central retinal artery occlusion (CRAO), combined with mild central retinal vein occlusion (CRVO), was diagnosed. During the second of three consecutive sessions of hyperbaric oxygen treatments, her vision rapidly improved. One week later, she developed peripheral phlebitis in the same eye. Infectious, inflammatory, and hematologic etiologies were excluded. The systemic evaluation was normal except for a positive Mantoux tuberculin skin test. Following systemic steroidal treatment, she experienced gradual improvement of her vasculitis. Two weeks later, mild retinal phlebitis appeared in her left eye. Eales\' disease was diagnosed after the exclusion of other diseases.
CONCLUSIONS: This is an unusual Eales\' disease case, which presented as combined CRAO with mild CRVO. The association of CRAO and Eales\' disease is reported here for the first time, to our best knowledge.

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Cardiac wall rupture (CWR) is a serious and often fatal complication of myocardial infarction (MI). Despite an increase in the incidence of MI in patients with systemic lupus erythematosus (SLE), cases of CWR in these patients have been reported rarely. This study reports an SLE patient with CWR and pseudoaneurysm formation and reviews previously reported cases of CWR in SLE patients. An English language literature review of from the PubMed, EMBASE, and Scopus databases on published cases of CWR in SLE, up until January 2023, was performed and analyzed. The search identified 4 patients, including the present one, 5 cases altogether. All of them were female aged 27-40 years, and 3 of them had had SLE for 10 years or more. Chest pain and dyspnea were the common presentations. All had left ventricular (LV) wall rupture. Three patients had LV wall rupture with pseudoaneurysm formation (one had MI with normal coronary artery, one myocardial necrosis secondary from small coronary artery vasculitis and one MI from uncertain cause). The other 2 patients had LV free wall rupture (one had MI with extensive coronary atherosclerosis with coronary arteritis, and the other septic myocarditis with septic coronary arteritis) and these 2 patients died before the diagnosis was made. Three patients with pseudoaneurysm received surgical correction with good clinical outcomes in all. Cardiac wall rupture is a serious and often fatal cardiac complication. Emergency diagnosis and appropriate management with an experienced cardiology team is crucial. Surgical correction is the treatment of choice. Key Points • Cardiac wall rupture, a serious and often fatal cardiac complication, has rarely been described in SLE patients. • Emergency diagnosis and appropriate management with an experienced cardiology team is crucial. Surgical correction is the treatment of choice.

Thromboangiitis obliterans (TAO), or Buerger\'s disease, is a non-atherosclerotic inflammatory disease of the small and medium-sized arteries, veins, and nerves of the legs and arms, strongly associated with the use of tobacco products in young adults. Cannabis arteritis (CA), an entity with similar clinical and pathological features, has been described in marijuana users as a subtype of TAO. Distinction between TAO and CA is challenging, given that most patients use tobacco and marijuana products concomitantly. Herein, we report the case of a male in his late forties who was referred to rheumatology with a 2-month history of hand swelling and bilateral painful digital ulcers with blue discoloration on his fingers and toes. The patient reported daily use of marijuana in blunt wraps and denied tobacco use. His laboratory work-up was negative for scleroderma and other connective tissue diseases. His angiogram confirmed the diagnosis of thromboangiitis obliterans, which was attributed to cannabis arteritis. The patient was started on aspirin and nifedipine daily and discontinued marijuana use. His symptoms resolved within 6 months and have not recurred for more than a year with continued avoidance of marijuana. Our case is one of the few that features primarily marijuana-driven CA and highlights the importance of not only considering marijuana use but also blunt wrap use in patients presenting with Raynaud\'s phenomenon and ulcerations as cannabis use rises globally.