Evaluation Of Vasculitis Research Articles

Imaging in vasculitis.

Systemic vasculitides are characterized by inflammation of blood vessels. Their classification is based on the size of the blood vessels involved - large, medium, or small. Vasculitis early diagnosis and reliable monitoring are crucial to establish a treatment plan and prevent serious complications. Based on these considerations and depending on the location of the affected vessels, the importance of imaging modalities including ultrasonography (US), magnetic resonance Imaging (MRI), magnetic resonance angiography (MRA), computed tomography (CT), computed tomography angiography (CTA), and [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (FDG-PET/CT) has progressively increased. In addition to physical exam and laboratory data, these imaging tools offer complementary information about vascular changes occurring in vasculitis.This review summarizes the different imaging modalities being utilized to diagnose and monitor vasculitis. The most recent update for the use of imaging in vasculitis is referenced in the 2023 European Alliance of Associations for Rheumatology (EULAR) recommendations and the American College of Rheumatology (ACR) guidelines in 2021. Recent advances in PET imaging in large vessel vasculitis include improved technological imaging acquisition and the use of novel radiotracers for cellular and immune targets. FDG-PET has now been demonstrated to have high sensitivity and specificity to detect temporal arteritis. Imaging plays a significant role in the evaluation of vasculitis and continues to gain importance in the diagnosis and monitoring of disease activity. Differences exist between the ACR guidelines, which advocates for temporal artery biopsy, and the EULAR guidelines, which favors imaging modalities for the initial evaluation and diagnosis of large vessel vasculitis (LVV). Prerequisites for appropriate clinical management utilizing imaging in patients with vasculitis are the availability and access to skilled clinicians to interpret the images and the cost of these techniques not being prohibitive.

Clinical value of [18F]FDG-PET/CT and 3D-black-blood 3T-MRI for the diagnosis of large vessel vasculitis and single-organ vasculitis of the aorta.

We aimed to investigate the clinical value of a 3D-T1w turbo-spin-echo (TSE) sequence and [18F]fluorodeoxyglucose positron emission tomography/computed tomography ([18F]FDG-PET/CT) for the diagnosis of active large vessel vasculitis (LVV) and single-organ vasculitis (SOV) of the aorta. Twenty-four patients with suspected vasculitis who underwent MRI and PET/CT were retrospectively evaluated. MRI was analyzed for concentric contrast enhancement and wall thickening, and flow artifact intensity (4-point-scales). PET/CT analysis comprised qualitative, quantitative and semiquantitative methods. Imaging findings were correlated with final diagnosis derived from the clinical follow-up data. Fifteen of 24 patients had a clinically confirmed active vasculitis, two had inactive vasculitis and 7 no vasculitis. [18F]FDG-PET/CT and 3D-T1w TSE-MRI revealed both a high diagnostic accuracy of 88% and 83%, respectively. In patients in whom both PET/CT and MRI showed concordant findings (19 patients), the accuracy increased to 95% with a high positive predictive value (92%) and negative predictive value (100%); thus, a correct diagnosis was obtained in 18 of 19 patients. Among the five patients with discordant findings PET/CT correctly identified the two patients without active vasculitis while rated false positive on MRI. Of the three remaining patients with active vasculitis, two were correctly identified by MRI and one by PET/CT. 3D-T1w TSE-MRI and [18F]FDG-PET/CT are both useful in the diagnosis of active vasculitis with high diagnostic accuracies. The diagnostic accuracy was even optimized by combining the two analysis methods. Therefore, there might be substantial potential for the application of whole-body hybrid PET/MRI in the evaluation of vasculitis in future studies.

Microscopic Polyangiitis Masquerading as Pancreatitis

A 72-year-old female with a history of diverticulitis status post partial sigmoid colectomy, hypertension, hyperlipidemia, and osteoarthritis on chronic NSAIDs, presents with 2 months of progressive nausea, vomiting, early satiety, unintentional weight loss and abdominal pain. During an initial hospitalization she was noted to have a lipase of 1200 U/L and amylase of 130 U/L, but abdominal ultrasound did not demonstrate any gallstones or abnormality of the liver, pancreas, gallbladder or bile ducts. Laboratory findings included leukocytosis, elevated AST, ALT, alkaline phosphatase and GGT. The patient was taking acyclovir for herpes infection and was presumed to have druginduced pancreatitis, and she was discharged off acyclovir. However, her symptoms recurred along with cough and subjective fevers, and she continued to have elevated lipase and liver blood tests. CT abdomen and pelvis revealed no acute abnormalities except for a small lesion within the tail of the pancreas. Chest x-ray demonstrated diffuse bronchial wall thickening and mild bronchial opacities. The patient was admitted to the Gastroenterology service for work-up, and treated for presumed hospital-acquired pneumonia. EGD revealed no abnormalities and duodenal biopsies were negative for pathology. She was discharged, yet she again returned with similar symptoms and lab findings, except creatinine had elevated from 1.1 to 2.3 mg/dL and inflammatory markers were markedly elevated. Evaluation for vasculitis was positive for MPO and p-ANCA. Renal biopsy demonstrated pauci-immune necrotizing vasculitis. The patient was treated with steroids and rituximab, but developed alveolar hemorrhage requiring cyclophosphamide. Review of the literature reveals that abdominal pain (˜30-60%) and abnormal liver blood tests (˜85%) are common in patients with microscopic polyangiitis and other ANCA associated vasculitides. However, gastrointestinal signs and symptoms can be quite variable. There are scattered case reports of ANCA associated vasculitides mimicking inflammatory bowel disease, isolated bowel necrosis, cholecystitis and pancreatitis. Depending on the type of vasculitis, either pulmonary or renal involvement is almost always present. This case illustrates that the initial presentation of microscopic polyangiitis may be gastrointestinal, and that a broad differential should be considered in patients with vague complaints and multiorgan findings.

Abstract T P312: Utility of Early MRI in Intracerebral Hemorrhage

Introduction: Intracerebral hemorrhage (ICH) causes significant morbidity and mortality. Utility of early magnetic resonance imaging (MRI) is not fully understood. Identifying patients in whom early MRI changes clinical management may improve outcomes in select patients and reduce costs overall by avoiding inpatient MRIs in patients who are unlikely to benefit. Hypothesis: We assessed the hypothesis that early MRI in patients with spontaneous ICH does not alter management acutely in older patients. Methods: We conducted a retrospective chart review of all ICH admitted to our institution from 2006-2012. Patients were excluded if they 1) had a known underlying lesion, 2) were < 18 years, 3) suffered ICH as a result of trauma or 4) did not undergo MRI. Data were evaluated for clinical or radiographic characteristics that resulted in a change in clinical management, including surgical intervention or future avoidance of antithrombotic medications, among others. Results: In total, 248 patients with a median age of 70 years (IQR 58-78) were included. MRI changed management in 79 patients (31.9%). Initial MRI was obtained an average of 10 days after the hemorrhage. Possible structural abnormalities requiring emergent intervention were found in 14 (17.7%) patients, while 64 (81.0%) had findings concerning for other non-emergent/non-surgical anomalies (possible tumor (n=6), cavernoma (n=7), alternate diagnosis (n=1), probable amyloid angiopathy (n=21), evaluation for embolic phenomenon (n=13), evaluation for vasculitis (n=2), abnormal edema/enhancement (n=8), MRI led to additional non-invasive vascular imaging (n=6)). One patient (1.3%) was found to have a venous sinus thrombosis prompting anticoagulation. MRI was more likely to result in a change in management in patients < 55 years of age (47.1% vs 27.9%, p=0.009). Conclusions: MRI after ICH changed management in approximately one-third of patients and resulted in an immediate change in management in a minority of patients. Younger patients are more likely to benefit from early MRI. In older patients suspected of amyloid angiopathy that may preclude future antithrombotic therapy, delayed MRI in the outpatient setting may be reasonable.

Potential diagnostic pitfall in direct immunofluorescence evaluation for vasculitis in skin biopsies: fluorescent secretory granules within eccrine glands

Potential diagnostic pitfall in direct immunofluorescence evaluation for vasculitis in skin biopsies: fluorescent secretory granules within eccrine glands

Rapid progression of multiple splanchnic artery aneurysms

A 62-year-old man with a complex medical history that included long-standing muscular dystrophy presented with moderate-to-severe epigastric and mid-back pain. Family history was noncontributory. The patient worked as a full-time computer instructor. He was a social drinker and denied a history of cigarette smoking or illicit drug use. At initial presentation, vital signs were normal except for a blood pressure of 148/105 mm Hg. Physical examination revealed moderate epigastric tenderness without rebound or guarding. Laboratory findings were unremarkable, and the hemoglobin was 12.4 g/dL (hematocrit, 37%). A computed tomography of the abdomen and pelvis demonstrated a retroperitoneal hemorrhage that involved the mesenteric circulation. A mesenteric arteriogram (Fig 1) was performed that revealed high-grade stenosis of the celiac artery origin with retrograde filling via superior mesenteric artery (SMA) collaterals as well as aneurysms of the celiac artery trifurcation and inferior pancreaticoduodenal artery (IPDA) without evidence of active bleeding. No therapeutic intervention was undertaken, and the patient was stabilized and discharged home. Three weeks later, he was admitted with recurrent abdominal pain, and a repeat arteriogram (Fig 2) showed significant increase in the size and number (13 in total) of splanchnic artery aneurysms that involved the celiac trunk, branches of the SMA that included the anterior and posterior branches of IPDA and gastroepiploic arteries, and splenic and right renal arteries. No risk for splanchnic artery aneurysms was identified, and evaluation for vasculitis or mycotic etiology was negative. Also, we could not identify any reports of an association between our patient's muscular dystrophy and the development of splanchnic artery aneurysms. Therapeutic intervention was neither feasible nor attempted given the patient's vascular anatomy and overall health. At follow-up, 4 months since the last arteriogram, the patient remains hemodynamically stable in his usual state of health. Fig 2Follow-up mesenteric arteriogram performed 3 weeks after the initial study (as observed in Fig 1), which revealed splanchnic artery aneurysms that involve the celiac trunk, branches of the SMA that include the anterior and posterior branches of IPDA, and gastroepiploic arteries, as well as splenic and right renal arteries. View Large Image Figure Viewer Download Hi-res image This section features outstanding photographs of clinical materials selected for their educational value or message, or possibly their rarity. The images are accompanied by brief case reports (limit 2 typed pages, 4 references). Our readers are invited to sumit items for consideration.

Giant cell arteritis in a patient with acute aortic insufficiency with thyrotoxicosis

Acute aortic insufficiency in the setting of thyrotoxicosis can mask the presentation of vasculitis. We report a case of a 38-year-old woman with a 22 weeks gestation pregnancy who was known to be hyperthyroid for 4 months prior to conception. She presented with thyrotoxicosis and acute respiratory failure. Echocardiogram revealed severe acute aortic regurgitant flow. Following medical treatment for aortic insufficiency and thyrotoxicosis, the patient underwent ascending aorta replacement with aortic valve repair. Pathological exam revealed giant cell arteritis. Both giant cell arteritis and thyrotoxicosis share a common major histocompatibility antigen which may facilitate concomitant disease presentation. Following immunosuppression for giant cell arteritis, valve repair, and treatment for thyrotoxicosis, the patient made a complete recovery. A rise in human chorionic gonadotropin (HCG) during the first trimester of pregnancy is known to have a stimulatory effect on the thyroid gland and may result in hyperthyroidism. Although HCG may have exacerbated the existing hyperthyroidism, in this case it was not causal, as the diagnosis preceded her pregnancy by several months. Diagnosis of vasculitis may be overshadowed by the presence of thyrotoxicosis. Significant vascular compromise in the setting of thyrotoxicosis must prompt an evaluation for vasculitis. This may prevent unnecessary surgery with attendant morbidity and mortality.

Diagnosis and evaluation of vasculitis.

Diagnosis and evaluation of vasculitis.

Cutaneous Vasculitis: Approach to Diagnosis and Systemic Associations

The starting point in the evaluation of vasculitis is the clinical examination. The character and location of the primary lesions determine the type of biopsy specimens and additional tests needed to classify vasculitis. The most common causes of cutaneous vasculitis are drug reactions, infectious diseases, reactions to inflammatory diseases such as rheumatoid arthritis or inflammatory bowel disease, or association with malignant disease, especially lymphoproliferative disorders. Direct immunofluorescent techniques and leukocyte monoclonal antibody studies are useful for the diagnosis of selected cases of vasculitis. The clinical and histopathologic data help delineate an approach for further investigation of potentially associated systemic disorders or underlying causes. Although some cases of cutaneous vasculitis are not associated with systemic disorders, this possibility should never be assumed but considered only as a diagnosis of exclusion after careful examination of each patient.