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Two clinical experts independently documented the segmental anatomy of the vascular obstructions by reviewing clinical records, pulmonary and CT angiograms, and surgical specimens. A nuclear medicine expert documented the segmental anatomy of the perfusion defects observed by planar and SPECT scans independently. Results:? Clinical/pathological evaluation disclosed 241 obstructed and 99 unobstructed lung segments in 17 patients. Sensitivity for detecting obstructed segments was significantly higher for SPECT than for planar scanning (63.5?��?3.1% vs 42.7?��?3.2%, respectively; P? of saline are instilled into the lungs to remove the proteinaceous material. However, not all patients respond to this treatment. Thus, new treatment modalities, such as subcutaneous or inhaled granulocyte macrophage colony-stimulating factor (GM-CSF), and the CD20 antibody rituximab and plasmapheresis, have been investigated. Based on the current literature, a stepwise treatment plan is suggested starting with WLL, continuing to inhaled GM-CSF, and then to rituximab if the former treatment regimes are unsuccessful. Pulmonary alveolar proteinosis (PAP) was first described by Rosen in 1958,1 and since then fewer than 1000 cases have been described. PAP is a rare lung disease characterized by accumulation of eosinophilic periodic acid Schiff (PAS)-positive material in the distal airways. The accumulation of PAS-positive material causes restrictive pulmonary function and decreased diffusion capacity (DLCO), and can progress to respiratory failure and death. The lung architecture is preserved, and there are few or no signs of lung inflammation. It is now recognized that PAP can be divided into two categories: (i) autoimmune PAP accounting for approximately 90% of all PAP cases, and (2) non-autoimmune PAP, which can be further subdivided into secondary PAP and congenital PAP.2�C4 The secondary form is found in association with high level of dust, mineral and metal particles exposures (e.g. aluminium, titanium, indium, silica, titanium). It is further associated with haematological malignancies and has been seen after allogeneic bone marrow transplantation for myeloid malignancies.