Multi-Modality Imaging in Vasculitis.

Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis.

Successful Treatment of Steroid-Refractory Checkpoint Inhibitor Myocarditis with Globulin Derived-Therapy: A Case Report and Literature Review.

Immune checkpoint inhibitor (ICI) monoclonal antibody drugs are an important interface of immunology and cancer biology with the intended goal to create cancer specific treatments with less systemic toxicity. Recognition of immune-related adverse events is critical and these include significant cardiovascular toxicity and myocarditis. Compared with other immune-related events, ICI associated myocarditis is rare but is associated with high mortality. The majority of cases present early in the course of therapy and patients can rapidly progress to fulminant myocarditis. Initially, the mainstay of treatment in patients with ICI-associated myocarditis is immunosuppressive therapy with glucocorticoids. For those who do not respond to steroids, the optimal treatment is unclear. This review summarizes the potential adjunctive treatment options for patients with steroid-refractory myocarditis by illustrating a case of myocarditis that was treated with Thymoglobulin and immunoglobulin.

Never Events in Radiology and Strategies to Reduce Preventable Serious Adverse Events.

The term never event in medicine was originally coined by Kenneth W. Kizer, MD, MPH, former chief executive officer of the National Quality Forum, to describe particularly shocking medical errors that should never occur, such as wrong-site surgery or death associated with introduction of a metallic object into the MRI area. With time, the National Quality Forum's list of never events, or "serious reportable events," has been expanded to include adverse events that are unambiguous, serious, and usually preventable. In this article, the never event framework has been used to describe (a) the errors that may occur in an imaging department that are serious and usually preventable with a review of the causative factors and (b) strategies to eliminate and reduce the adverse effects of these avoidable errors. These errors are often rooted in communication breakdowns and can only be eliminated with a true shift to a culture of open reporting and patient safety. ©RSNA, 2018.

Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus.

Bronchiolitis obliterans (BO) and paraneoplastic pemphigus are rare and ominous complications of Castleman disease. Collectively, these processes have been reported as part of paraneoplastic autoimmune multiorgan syndrome (PAMS), and they can occur in the setting of various hematologic malignant tumors, carcinoid tumors, and melanoma. Irrespective of the underlying malignancy driving PAMS, the clinical outcomes are uniformly poor, and there are no standard treatment regimens, given the clinical rarity of the syndrome. We describe 2 patients with unicentric Castleman disease complicated by paraneoplastic pemphigus and bronchiolitis obliterans. In addition to primary surgical resection for Castleman disease, we also used therapy from a treatment protocol used for bronchiolitis obliterans resulting from hematopoietic stem cell transplant (HSCT). We were able to treat the patients using intravenous immunoglobulin; rituximab; fluticasone, azithromycin, and montelukast (FAM); and rosuvastatin therapy. One patient demonstrated a favorable response, while the other demonstrated minimal response to this therapy.

Eosinophilia and Ulcerative Colitis Associated with Eosinophilic Myocarditis.

Reactive eosinophilia is associated with inflammatory bowel disease, but its association with eosinophilic myocarditis is rare. We report a case of a 42-year-old man who presented with hypovolemic shock secondary to diarrhea and recently diagnosed nonischemic cardiomyopathy (left ventricular ejection fraction, 0.29). Laboratory evaluation revealed marked peripheral eosinophilia. Cardiac magnetic resonance imaging showed evidence of subacute-to-chronic myocarditis, and endomyocardial biopsy results were consistent with eosinophilic myocarditis. Colonic biopsy specimens revealed ulcerative colitis and no eosinophils. Hematologic evaluation was negative for an alternative cause of eosinophilia. The patient was given corticosteroids; his diarrhea resolved, but there was no short-term improvement in his ejection fraction, so an implantable cardioverter-defibrillator was placed. Follow-up at one year showed that the patient's left ventricular ejection fraction had improved to 0.42.

Hereditary renal tumor syndromes: imaging findings and management strategies.

OBJECTIVE: It is not rare for the radiologist to identify multiple renal masses and be the first to raise the possibility of a hereditary renal tumor syndrome. Characteristic renal and extrarenal imaging findings aid in making the correct diagnosis. The imaging findings, screening guidelines, and management techniques for the most common hereditary renal tumor syndromes are reviewed. CONCLUSION: Hereditary renal tumor syndromes have specific screening guidelines and unique management techniques in which imaging plays a central role.