Mixed cryoglobulinemia is the most prevalent extrahepatic manifestation of chronic HCV infection. It is usually a benign lymphoproliferative disorder which presents as vasculitis affecting different organs. Although life-threatening cryoglobulinemic vasculitis (CryoVas) is rare, it is sometimes the first and possibly lethal complication. Its treatment depends on the severity of vasculitis and can be challenging. High dose of corticosteroids, immunosuppressive agents and plasma exchange represent the first-line treatment, which should be followed by antiviral therapy. Rituximab is an effective and safe treatment option. However, the data about its use in life-threatening conditions are scarce. We report the case of a patient with severe, relapsing and life-threatening HCV-related CryoVas resistant to standard therapy who had had an initial beneficial response to rituximab added to plasma exchange that was later compromised by the development of sepsis. We also review the literature and discuss manifestations and therapy of life-threatening Cryovas with focus on rituximab use.

Background: Behçet’s disease (BD) and granulomatosis with polyangiitis (GPA) are distinct vasculitides. PR3-ANCA is considered specific for GPA, yet rare BD cases demonstrate positivity, creating diagnostic dilemmas. Case Presentation: We describe a young man fulfilling criteria for BD, presenting with recurrent oral and genital ulcers, ocular inflammation, catastrophic jejunal perforations, pulmonary embolism, and myocardial infarction with non-obstructive coronary arteries. Despite strong PR3-ANCA positivity, the global phenotype was consistent with BD. Management required a complex, multimodal immunosuppressive regimen that included corticosteroids, cyclophosphamide, therapeutic plasma exchange, and rituximab. Conclusions: PR3-ANCA positivity may represent a severe BD phenotype rather than true GPA overlap, underscoring the need for individualized treatment strategies.

Background: Drug desensitization allows for safe administration of a drug to a patient with a previous hypersensitivity reaction. Successful desensitization protocols have been described for different medications, including protocols for oncology patients. Few cases of desensitization to sorafenib and imatinib have been described in the literature so far. Objective: The objective of this paper is to describe the process of the sorafenib and imatinib drug hypersensitivity diagnosis and desensitization process in two patients. Methods: Two oncology patients who experienced non-immediate hypersensitivity reactions to sorafenib and imatinib underwent desensitization to these drugs. We designed a protocol for the first patient and used a modified protocol from the literature for the second patient. Results: By using a slow desensitization technique and gradual tapering of corticosteroids and antihistamines, both patients reached the target dose of the incriminated drug. Conclusions: Desensitization to sorafenib and imatinib can be an effective therapeutic option in patients with hypersensitivity to those medications, without alternative treatment options.

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Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis affecting predominantly small vessels, typically associated with PR3-ANCA positivity. Central nervous system involvement beyond cranial nerve palsies is rare, with intracerebral granulomas being exceptionally rare and lacking specific imaging characteristics, often mimicking malignancies or other intracranial pathologies. This significantly complicates the diagnostic process, particularly in the absence of active systemic disease. We report a case of a 71-year-old woman in whom neurological symptoms and magnetic resonance imaging revealed intracerebral granulomas and led to the diagnosis of GPA. Early recognition of tumor-like brain lesions in GPA and prompt initiation of immunosuppressive and anti-edematous therapy are crucial to prevent progression and potentially life-threatening compressive complications.