A 45-year-old white man seeks medical care because of hemoptysis of 1-week duration. He has not felt well for approximately 4 months and has lost 10 lb (4.5 kg) during this time. He has been receiving various antibiotics for the treatment of chest radiographic abnormalities thought to represent pneumonia. Although these changes have varied in presentation, they have not disappeared. A few weeks earlier,he noted some bloody nasal discharge. He started coughing up blood 1 week ago but attributed it to his bloody nose. The patient also complains that his
left knee has been hurting and that red spots have appeared on his arms and legs. He denies fever, purulent sputum, allergies or asthma, known tuberculosis, or chest pain.
On physical examination,
there is a curious depression in his upper nose(saddle-nose deformity),
bloody discharge in his nasal cavity,
a painless ulcer on his soft palate,
and
a slightly warm and swollen left knee.
Chestfindings are normal.
There are many small, purpuric, raised lesions on the skin of his lower extremities that are painless.
What are four possible diagnoses in this patient?
What diagnostic studies or procedures might be of value in this patient?
Which disorders are associated with p-ANCA?
What constitutes appropriate therapy for this patient with Wegener's
granulomatosis?
Case Discussion
What are four possible diagnoses in this patient?
Four possible diagnoses in this patient are Wegener's granulomatosis, Churg-Strauss syndrome (allergic granulomatosis), intranasal drug abuse, or a lung tumor. Churg-Strauss syndrome occurs primarily in patients with a history of allergies or asthma and is often associated with peripheral eosinophilia. ANCA reacting with human neutrophil elastase can occur in cocaine-induced midline
destructive lesions. The saddle-nose deformity and palpable purpura would be uncommon manifestations of a primary lung carcinoma.
What diagnostic studies or procedures might be of value in this patient?
Nasopharyngeal examination with biopsy, CT scan of the sinuses and chest,creatinine and urinalysis, and bronchoscopy with biopsy or open lung biopsy would all be helpful in the evaluation of this patient's disorder. An ANCA should be ordered because most patients with systemic Wegener's granulomatosis are c-ANCA positive and have antiproteinase 3 antibodies. In approximately 60% of patients, c-ANCA titers correlate with Wegener's disease activity.
Which disorders are associated with p-ANCA?
A p-ANCA may be present due to a variety of different antibodies directedagainst myeloperoxidase, elastase, cathepsin, and lactoferrin, and can occur in many different diseases. Diseases associated with p-ANCA directed against myeloperoxidase include Wegener's granulomatosis (10%), Churg-Strauss syndrome (50%), MPA (50% to 80%), and idiopathic crescentic
glomerulonephritis (65%). Nonspecific p-ANCAs directed against other various proteins can occur in CTDs, Crohn's disease, ulcerative colitis, sclerosing cholangitis, cystic fibrosis, chronic infections, and rare drug-induced vasculitic syndromes associated with propylthiouracil, hydralazine, and minocycline.
What constitutes appropriate therapy for this patient with Wegener's granulomatosis?
Standard therapy for Wegener's granulomatosis includes both high doses of corticosteroids and oral cyclophosphamide. Oral trimethoprim/sulfamethoxazole prophylaxis against Pneumocystis carinii should be considered while on the above therapy.
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