Remarkable Improvement in Clinical Course and Serum KL-6 Levels after Initiation of High-Dose Inhaled Budesonide in Pulmonary Sarcoidosis.

We present a pulmonary sarcoidosis patient with specific elevation of serum Krebs von den lungen-6 (KL-6) levels, who was successfully treated with inhaled corticosteroids. Pulmonary sarcoidosis was initially identified as a chest radiograph abnormality during a routine medical examination, and subsequently confirmed by a high serum level of soluble interleukin 2 receptor. The patient was started on high-dose inhaled budesonide because of high serum levels of angiotensin-converting enzyme (ACE) and KL-6. Following treatment, radiographic findings improved, ACE levels normalized, and serum KL-6 levels markedly decreased. No recurrence was detected at 100 months with a budesonide dosage of 800 μg/day. This case demonstrates the efficacy of highdose inhaled corticosteroids for the initial treatment of pulmonary sarcoidosis.