title: Clinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease.
abstract: We have previously reported that myelodysplastic syndrome (MDS) is the most common underlying disease in cases of secondary pulmonary alveolar proteinosis (PAP). Here, we present 3 MDS cases in which PAP developed during the course of Behçet's disease (BD). All patients carried trisomy 8 in the bone marrow. Chest HRCT scans showed variable distribution of ground glass opacities, but none of the scans showed so called "crazy paving appearance". Two patients with intestinal BD who underwent potent immunosuppressive therapy died of sepsis. These findings demonstrate that PAP secondary to MDS may be occasionally associated with BD.
authors.affiliation: Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, 54 Kawaharacho Shogoin, Sakyo-ku, Kyoto 606-8507, Japan. Electronic address: hanta@kuhp.kyoto-u.ac.jp.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Handa
authors.affiliation: Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, 1-754 Asahimachi-dori, Chuo-ku, Niigata 951-8520, Japan. Electronic address: nakatsue@med.niigata-u.ac.jp.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nakatsue
authors.affiliation: Department of Respiratory Medicine, Tomishiro Chuo Hospital, 25, Ueda Tomishiro, JP-47, 901-0243, Japan. Electronic address: mbaba@yuuai.or.jp.
authors.forename: Motoo
authors.initials: M
authors.lastname: Baba
authors.affiliation: Division of Respiratory Medicine, Niigata University Graduate School of Medical and Dental Sciences, 1-754 Asahimachi-dori, Chuo-ku, Niigata 951-8520, Japan. Electronic address: ttakada@med.niigata-u.ac.jp.
authors.forename: Toshinori
authors.initials: T
authors.lastname: Takada
authors.affiliation: Bioscience Medical Research Center, Niigata University, 1-754 Asahimachi Dori, Chuoku, Niigata 951-8520, Japan. Electronic address: radical@med.niigata-u.ac.jp.
authors.forename: Koh
authors.initials: K
authors.lastname: Nakata
authors.affiliation: Department of Respiratory Medicine, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, 181-8611, Japan. Electronic address: h141@ks.kyorin-u.ac.jp.
authors.forename: Haruyuki
authors.initials: H
authors.lastname: Ishii
mesh_terms: D000328:Adult; D001528:Behcet Syndrome / Q000150:complications* / Q000188:drug therapy; D001853:Bone Marrow; D002898:Chromosomes, Human, Pair 8; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000150:complications*; D011649:Pulmonary Alveolar Proteinosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000235:genetics; D013902:Radiography, Thoracic; D018805:Sepsis / Q000209:etiology; D014057:Tomography, X-Ray Computed; D014314:Trisomy
chemical_list: D007166:Immunosuppressive Agents
keywords: BAL; BD; Behçet's disease; GGO; HRCT; Intestinal ulcer; MAC; MDS; Mycobacterium avium complex; Myelodysplastic syndrome; PAP; RA; RA with excess blasts; RAEB; SLB; SPAP; Sepsis; Trisomy 8; WLL; bronchoalveolar lavage; ground glass opacity; high-resolution computed tomography; myelodysplastic syndrome; pulmonary alveolar proteinosis; refractory anemia; secondary PAP; surgical lung biopsy; whole lung lavage