Systemic vasculitis

Case history #1

A 36-year-old woman with a history of chronic sinusitis presents with nasal deformity. She has had nonspecific muscle and joint aches for 2 years, diagnosed as fibromyalgia. For a few years she has regularly noted dark crusts from her nose, occasionally mixed with some blood. A few weeks ago the bridge of her nose started to collapse. She has a prominent saddle nose deformity and nasal septal defect. Sinus biopsy shows only chronic inflammation, but her cytoplasmic-pattern antineutrophil cytoplasmic autoantibody titer is 1:160, consistent with granulomatosis with polyangiitis.

Case history #2

A 75-year-old man with an unremarkable past medical history presents with a complaint of a new headache for the past 2 weeks. He notes that the headache is localized over the left temple. Two weeks prior to the onset of headache, he noted pain and stiffness in the shoulders and hips, which made it difficult to rise from bed in the morning, but progressively improved throughout the day. A few days prior to his evaluation, he noted jaw pain on chewing, and notes in retrospect that he had begun to avoid certain foods (e.g., steak) because of the associated discomfort. Laboratory evaluation demonstrates evidence of inflammation, including an elevated erythrocyte sedimentation rate, C-reactive protein, and platelets. The complaints of new headache and jaw claudication in the setting of systemic inflammation are consistent with a diagnosis of giant cell arteritis.

Other presentations

To some extent the manifestations of vasculitis are dictated by the size of blood vessel involved.[2]Seo P. Vasculitis. In: Bingham C, Bartlett SB, eds. Clinical care in the rheumatic diseases. 3rd ed. Atlanta, GA: American College of Rheumatology; 2006:199-202. Large-vessel vasculitis, for example, classically presents with claudication of the arm or jaw: that is, discomfort that occurs after repetitive motion due to compromised blood supply to the region being exercised. Involvement of the subclavian arteries may lead to asymmetric peripheral pulses.

Mononeuritis multiplex, or infarction of a named nerve, is a classic manifestation of a medium-vessel vasculitis. Clinically this may lead to an inability to dorsiflex the foot (foot drop) or an inability to hyperextend the hand (wrist drop). A medium-vessel vasculitis that affects the mesenteric arteries may lead to abdominal pain, mesenteric angina, or gut infarction.

Small-vessel vasculitis is associated with organs that have dense populations of small blood vessels. Small-vessel vasculitis in the kidneys leads to glomerulonephritis, in the lungs to pulmonary hemorrhage, and in the skin to palpable purpura.