Details

Autor(en) / Beteiligte

• Handa, Tomohiro
• Nakatsue, Takeshi
• Baba, Motoo
• Takada, Toshinori
• Nakata, Koh
• Ishii, Haruyuki

Titel

Clinical features of three cases with pulmonary alveolar proteinosis secondary to myelodysplastic syndrome developed during the course of Behçet's disease

Ist Teil von

• Respiratory investigation, 2014-01, Vol.52 (1), p.75-79

Ort / Verlag

Netherlands: Elsevier B.V

Erscheinungsjahr

2014

Quelle

MEDLINE

Beschreibungen/Notizen

• 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.