A 34-year-old man comes to the physician reporting one week of inability to extend his right wrist and several of his fingers on the same hand. He first began having difficulty while trying to type his thesis for graduate school on his computer. He is right-handed and does not recall any trauma. He has a history of asthma that was diagnosed while he was in college. Physical examination demonstrates impaired dorsiflexion of the right wrist with normal strength of the left wrist. Laboratory studies show: Serum antibodies against neutrophil myeloperoxidase are positive. This patient most likely has which of the following conditions?

Question

A 34-year-old man comes to the physician reporting one week of inability to extend his right wrist and several of his fingers on the same hand.  He first began having difficulty while trying to type his thesis for graduate school on his computer.  He is right-handed and does not recall any trauma.  He has a history of asthma that was diagnosed while he was in college.  Physical examination demonstrates impaired dorsiflexion of the right wrist with normal strength of the left wrist.  Laboratory studies show:  Serum antibodies against neutrophil myeloperoxidase are positive.  This patient most likely has which of the following conditions?

Quizplus · Accepted Answer

This patient most likely has eosinophilic granulomatosis with polyangiitis (Churg-Strauss). This small to medium vessel vasculitis is characterized by late-onset asthma, rhinosinusitis, and eosinophilia, though it can involve many other organ systems including the kidneys, gastrointestinal tract, and cardiovascular system. Asymmetric multifocal neuropathy (mononeuritis multiplex) is particularly common due to the vasculitis affecting the epineural vessels (eg, wrist drop due to radial nerve involvement). Other common manifestations include skin nodules, migratory/transient pulmonary infiltrates, and paranasal sinus abnormalities. In addition to peripheral eosinophilia, a frequent laboratory finding is antibodies against neutrophil myeloperoxidase, which most commonly have a pattern of perinuclear staining (p-ANCA). (Choice A) Allergic bronchopulmonary aspergillosis (ABPA) can produce an asthma-like picture with eosinophilia and elevated IgE and IgG serum antibodies to Aspergillus fumigatus. However, systemic vasculitis and p-ANCA are not commonly associated with ABPA. (Choice B) Pulmonary involvement in anti-glomerular basement membrane disease presents with cough, pulmonary infiltrates, and hemoptysis (not asthma-like symptoms). Patients have circulating autoantibodies against collagen IV, which damage alveolar and glomerular basement membranes. (Choice C) Carpal tunnel syndrome is characterized by paresthesias and pain in the distribution of the median (not radial) nerve. Sensory symptoms are more prominent than motor symptoms during the initial presentation. (Choice E) Idiopathic pulmonary fibrosis is a chronic fibrosing interstitial lung disease. There is no known association with p-ANCA, eosinophilia, or systemic vasculitis. (Choice F) Pulmonary parenchymal involvement by systemic sclerosis (scleroderma) results in a chronic restrictive interstitial fibrosis. Antitopoisomerase I (Scl-70), anticentromere, and anti-RNA polymerase III antibodies are highly specific for systemic sclerosis. Educational objective: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) is a small to medium vessel vasculitis characterized by late-onset asthma, rhinosinusitis, and eosinophilia. Mononeuritis multiplex due to involvement of the epineural vessels of peripheral nerves is common. __________ References: Churg-Strauss syndrome: evolving concepts. (http://www.ncbi.nlm.nih.gov/pubmed/20350492) ANCA-associated small-vessel vasculitis. (http://www.ncbi.nlm.nih.gov/pubmed/11989638)

A 34-Year-Old Man Comes to the Physician Reporting One Week