A 46-year-old registered nurse presented with a two-month history of intermittent episodes of arthralgias, with occasional swelling of her wrists or knees. These episodes lasted from one to three days and were helped by over-the-counter ibuprofen.

One month ago a red rash appeared on her legs that worsened when she was up during the day.  Although not painful, the rash looked like “blood blisters.” She also developed painful, purple lesions on her ankles. The knee pain worsened, and her fingers became sore and stiff.

She then began having low-grade fevers, myalgias, and recurrent frontal headaches, which she assumed were flu symptoms. These were causing her to miss a great deal of work. After a week or so, the rash on her legs subsided spontaneously, but it then reappeared one week ago. At that time she began to lose her appetite, her headaches worsened, and two days ago she developed mid-abdominal pain, with nausea and vomiting. She has lost 10 pounds over the past two months.

A. Rheumatoid arthritis
B. Systemic lupus erythematosus
C. Systemic necrotizing vasculitis
D. Disseminated gonococcemia arthritis
E. Infectious mononucleosis
F. Viral hepatitis
G. HIV infection
H. Subacute bacterial endocarditis

This patient has no history of photosensitive rash, hair loss, oral or ocular dryness, Raynaud’s phenomenon, pleurisy, or convulsions. She is two years postmenopause and is taking no medications except the ibuprofen for her joint pains. She has no history of tick bites, and she denies risk factors for HIV infection. She works on a dialysis unit and has missed work for the past three days.

She stopped smoking five years ago and drinks only occasionally. She is married with three grown children. Both her medical and family history are unremarkable.  The patient is an ill-appearing white woman. Blood pressure is 140/85, pulse is 110 and regular, and temperature is 100°F. She has no adenopathy. The mucosae are dry and without lesions. Funduscopic exam is normal. Chest is clear, and heart sounds are normal with no murmurs noted. Mid-abdominal and right upper quadrant tenderness is present, but without guarding. There is no liver or spleen enlargement, and bowel sounds are active. Rectal examination is unremarkable, and stool guaiac is negative. Neurological exam is normal.

She has mild swelling and tenderness of both wrists, and small bilateral knee effusions. Discrete palpable, purpuric lesions are present on both legs, along with a few tender, hemorrhagic lesions over both ankles.  There are no ulcerating skin lesions.

Results of routine laboratory testing revealed the following:

A. Active hepatocellular disease
B. Gallbladder disease
C. Active inflammatory renal disease
D. Acute urinary tract infection
E. GI bleeding

A. Anti-dsDNA
B. Antineutrophil cytoplasmic antibody (ANCA)
C. Anti-HIV antibodies
D. Hepatitis B antigen, hepatitis C antibody
E. C3, C4
F. Cryoglobulins
G. Serum protein electrophoresis
H. Blood cultures

A. 24-hour urine protein and creatinine clearance
B. Abdominal ultrasound
C. Renal biopsy
D. Abdominal angiogram
E. Colonoscopy
F. Skin biopsy
G. Liver biopsy

Anti-dsDNA, anti-HIV antibodies, and hepatitis C antibodies are negative. HBsAg is present. ANCA is positive. Complement levels are slightly decreased, at 45 mg/dl for C3 (normal 50-100) and 13 mg/dl for C4 (normal 15-30); cryoglobulins are negative. Serum protein electrophoresis demonstrates a polyclonal rise, with normal gamma globulins. A 24-hour urine collection shows 1.20 gm protein, and the creatinine clearance rate is 75 ml/min. Blood cultures are negative.

Ultrasound examination of the abdomen and colonoscopy up to the terminal ileum are unremarkable. The renal biopsy revealed pauci-immune focal necrotizing glomerulonephritis.  The angiogram demonstrates multiple areas of stenosis and dilation with saccular aneurysms in the superior mesenteric artery.  Leukocytoclastic vasculitis is demonstrated on skin biopsy. The patient declined the liver biopsy.

A. Systemic lupus erythematosus
B. Cryoglobulinemia associated with hepatitis B
C. Polyarteritis nodosa associated with hepatitis B
D. Henoch-Schönlein purpura
E. Rheumatoid arthritis
F. Sjögren’s syndrome
G. HIV-related arthritis

After working this case, the student will be able to:

1. List at least three organ systems commonly affected in polyarteritis nodosa.
   
   
2. List at least four cutaneous findings in patients with necrotizing vasculitis.
   
   
3. Name three musculoskeletal syndromes associated with hepatitis B virus infection.
   
   
4. Recommend appropriate work-up of the patient with clinical findings suggesting systemic vasculitis.

Conn DL. Polyarteritis. Rheum Dis Clin North Am 1990;16:341-362.

Gibson LE, Su WPD. Cutaneous vasculitis. Rheum Dis Clin North Am 1990;16:309-324.

Guillevin L, Lhote F, Cohen P, Sauvaget F, Jarrousse B, Lortholary O, Noel LH, Trepo C. Polyarteritis nodosa related to hepatitis B virus: a prospective study with long-term observation of 41 patients. Medicine (Baltimore) 1995;74:238-53.

Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore) 1996;75:17-28.

Lie JT. Diagnostic histopathology of major systemic and pulmonary vasculitic syndromes. Rheum Dis Clin North Am 1990;16:269-292.

Copyright© 2002 American College of Rheumatology