Unilateral Facial Swelling and Exophthalmos in a Patient with Polyarteritis Nodosa.

Abstract

A patient with polyarteritis nodosa (PN) presenting with exophthalmos and facial swelling, which are rarely found in PN, is reported. The patient, a 27-year-old male, complained of painful facial swelling and diplopia. Physical examinations showed facial swelling around the right orbit and exophthalmos. After admission, he experienced myalgia in both calves. Laboratory studies disclosed leukocytosis and liver dysfunction. Celiac and renal angiograms showed aneurysms. A biopsy specimen of his left calf showed an arterial inflammatory process. The patient was diagnosed as having PN. He had an excellent response to corticosteroids, with prompt improvement. (Internal Medicine 31: 500-503, 1992)

Non-infectious orbital vasculitides

“…PAN can cause ischemia and dysfunction of the extraocular muscles, leading to ophthalmoplegia and diplopia. 45 PAN can also cause diffuse orbital inflammation, leading to exophthalmos, restriction of extraocular movements, conjunctival injection and chemosis, and decreased vision. There have been seven cases in the literature reporting exophthalmos and orbital inflammation associated with PAN.…”

“…There have been seven cases in the literature reporting exophthalmos and orbital inflammation associated with PAN. [45][46][47][48] These signs and symptoms are all secondary to the mass effect created by the vasculitis occurring within the orbit.…”

“…There have been isolated reports showing swelling and increased density of the extraocular muscles and soft tissues on CT, and enlargement of muscles and the retrobulbar space on MRI. 45,46 Orbital biopsies can exhibit a vasculitis of arteries and/or veins with fibrosis, although literature has not supported this as a reliable finding. 45,47 Treatment of orbital vasculitis secondary to PAN is identical to treatment of the systemic disease.…”

“…45,46 Orbital biopsies can exhibit a vasculitis of arteries and/or veins with fibrosis, although literature has not supported this as a reliable finding. 45,47 Treatment of orbital vasculitis secondary to PAN is identical to treatment of the systemic disease. The mainstay of therapy is corticosteroids and cyclophosphamide with case reports showing good response of orbital disease to corticosteroids alone.…”

“…The mainstay of therapy is corticosteroids and cyclophosphamide with case reports showing good response of orbital disease to corticosteroids alone. 45,46 Other immunomodulatory agents such as azathioprine and methotrexate have also been used. 4 There have been case reports of disease remission with the use of rituximab in PAN, although not specifically in orbital disease.…”