Periaortitis in eosinophilic granulomatosis with polyangiitis

Area of Science:

• Rheumatology
• Neurology
• Vascular Medicine

Background:

• Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis characterized by asthma, eosinophilia, and vasculiticならラクマ.
• Large-vessel involvement, such as periaortitis, is an uncommon but recognized manifestation of EGPA.
• Distinguishing EGPA from other eosinophilic or vasculitic disorders, including IgG4-related disease, can be diagnostically challenging.

Purpose of the Study:

• To report a case of EGPA presenting with radiological periaortitis and mononeuritis multiplex.
• To highlight the diagnostic considerations and management strategies for EGPA with large-vessel involvement.
• To emphasize the importance of recognizing thromboembolic risks in EGPA patients.

Main Methods:

• Case report of a patient in his late 60s with EGPA.
• Clinical presentation: acute mononeuritis multiplex, constitutional symptoms.
• Diagnostic workup: eosinophilia, MPO antibodies, imaging (periaortitis), nerve conduction studies, sural nerve biopsy.
• Treatment: corticosteroids, cyclophosphamide, azathioprine.

Main Results:

• The patient exhibited marked eosinophilia, positive MPO antibodies, and imaging evidence of periaortitis.
• Nerve conduction studies revealed axonal mononeuropathies; biopsy confirmed vasculitic neuropathy with eosinophilic infiltrates.
• Treatment led to substantial neurological improvement and remission of periaortitis.
• A pulmonary embolism complicated the patient's course.

Conclusions:

• EGPA can manifest with periaortitis and significant neurological deficits.
• Vigilance is required to differentiate EGPA from mimics like IgG4-related disease.
• Recognizing rare large-vessel involvement and managing thromboembolic complications are critical in EGPA care.