This therefore leads to a build up of surfactant [16]. The mainstay of treatment is managing

Bortezomib mouse the underlying malignancy [1], [6] and [9]. Whole lung lavage and GM-CSF therapy are options. In conclusion, the literature mainly discusses treatments for primary and congenital PAP. As secondary PAP is extremely rare, there is no specific recommended therapy. Treatment is centred on treating the underlying cause [1], [5] and [6] and providing symptomatic relief. The prognosis of PAP can be worse with atypical infections such as pneumocystis carinii [2], mycobacterium and viruses [1], [5] and [8]. This leads to increased morbidity [1] and mortality for patients with secondary PAP [6]. The accumulation of surfactant encourages microorganisms to grow and the susceptibility is further increased by abnormal macrophages [6] and [7]. The use of steroids should therefore not be encouraged [5]. Ultimately, the prognosis of secondary PAP is dependent on the course of the underlying disease. There is no conflict of interest. “
“Patient no 1 is a man who was 49 years old at the time of admission. He is a never-smoker who has worked several years as a carpenter. He had been occupationally exposed to stone dust, but not GDC-0449 in vitro to asbestos. Patient no 2 is

his two years younger sister who was admitted to our hospital, also at the age of 49. She is a never-smoking woman, working in an office, and had no known exposure to harmful dusts. They were both previously healthy, the brother born deaf, though, assumingly because his mother was infected with rubella during pregnancy. They have no known hereditary diseases, and there are no other siblings. None of them have had any trauma or had a tracheostoma, nor have they had previous recurrent infections. Both presented with worsening dyspnoe, cough and non-characteristic very chest pain over a period of 4–6

months, and both had been treated by their GPs with antibiotics, without any improvement. At the time of admission, patient no 1 had spirometry values consistent with airway obstruction with FVC 4.5 l (98% of predicted), FEV1 2.7 l (73%). The sister’s initial spirometry showed FVC 2.6 l (77%), FEV1 1.2 l (41%), FEV1/FVC 0.46, and a considerable compromised inspiratory curve. They both had TLC between 70 and 80% predicted, RV 80–90% and DLCO within the normal range on serial measurements. Both had an elevated CRP, 61 for patient no 1 and 47 for patient no 2. Blood leukocyte and eosinophils were normal, there was no hypercalcemia, and ACE levels were in the lower normal area. Extensive diagnostic investigations were undertaken for both patients.