A 65-year-old man who was occupationally exposed to asbestos for 40 years was admitted to our hospital with fever and cough. Chest CT revealed paraseptal emphysema, subpleural fibrosis in both lungs, and pleural plaques. On bronchoalveolar lavage fluid contained elevated levels of lymphocytes and neutrophils (15% and 17%, respectively), and asbestos bodies were also found. Because serum MPO-ANCA titer was elevated to 188 EU, we suspected ANCA-associated disease with interstitial pneumonia. Prednisolone was begun at 30 mg/day and his lung opacities partially disappeared. Six weeks later, he complained of headache, dysphagia and hoarseness, and was admitted to the neurology department of the hospital. Under a diagnosis of either hypertrophic pachymeningitis or neuritis due to angiitis of the lower cranial nerves, steroid pulse therapy was performed. Asbestos exposure may have been a contributing factor for ANCA generation in this case. Furthermore, the fact that cranial nerves palsy occurred in spite of steroid therapy may also be important.
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