PULMONARY ALVEOLAR PROTIENIOSIS

1. DR ASHRAF AHMAD
PULMONOLOGIST
KAASH - TAIF
2015
PULMONARY ALVEOLAR
PROTIENIOSIS

2. INTRODUCTION
is a diffuse lung disease characterized by the
accumulation of amorphous, periodic acid-Schiff
(PAS)-positive lipoproteinaceous material in the
distal air spaces

4. pathogenesis
1- Role of GM-CSF: effects of GM-CSF on surfactant clearance by alveolar
macrophages are mediated, at least in part, by transcription factor peroxisome
proliferator-activated receptor-gamma (PPAR-gamma) dependent pathways.
Compared to normal controls, alveolar macrophages from patients with PAP
demonstrate decreased levels of PPAR-gamma and the macrophage scavenger
receptor CD36, which PPAR-gamma regulates; PPAR-gamma and CD36 levels can
be restored to normal following treatment with GM-CSF administered
subcutaneously . The interrelationship between GM-CSF and PPAR-gamma may
explain how GM-CSF deficiency could cause alveolar and macrophage accumulation
of surfactant lipoprotein.

5. Cont.
2-Macrophage dysfunction :
 Alveolar MQ obtained from patients with PAP after therapeutic
whole lung lavage have greater migratory response compared with
macrophages from the same patients prior to the lavage.
 Other causes of acquired macrophage dysfunction, such as
immunosuppressive drug therapy or hematologic malignancies,
may explain the occasional finding of PAP associated with these
disorders .

6. CP
 Symptoms: progressive dyspnea on exertion,
cough, fatigue, weight loss, and low-grade fever.
expectoration of "chunky" gelatinous material may
occasionally occur.
 Physical examination :often normal; clubbing
and cyanosis are present in about 25%. Crackles are
present in approximately 50 %.

7. INVESTIGATIONS
 1-Lab.:
 polycythemia, hypergammaglobulinemia, and increased LDH
levels
 Elevated levels of lung surfactant proteins A and D (SP-A and SP-D)
 Sputum examination may suggest PAP in the correct clinical setting,
based on the identification of PAS-positive material in macrophages
 Anti-GM-CSF titer: In the absence of any known 2ry cause of
PAP, an elevated serum anti-GM-CSF titer is 100 % sensitive and 91
to 98% specific for the diagnosis of acquired PAP. BAL fluid levels of
anti-GM-CSF antibodies correlate better with the severity of PAP
compared to serum titers.
2-PFTs: restrictive ventilatory defect or sometimes an isolated
decrease in DLCO.
3- Radiological:

10. Cont.
4-BAL:
 milky appearance due to the abundant lipoproteinaceous
material
 MQs are engorged with the PAS-positive material
 Large acellular eosinophilic bodies in a background of
eosinophilic granules
 PAS staining of proteinaceous material
5- Lung Biobsy:

16. TREATMENT
1- Conservative : for asymptpmatic or mild
symptoms(with regular FUP)
2- Whole lung lavage : For patients who have
moderate to severe symptoms and hypoxemia. After
whole lung lavage, patients often feel dramatically
better, with improvement in exertional dyspnea. The
clinical course is variable. 30-40%of patients require
only one lavage, while others require repeat lung
lavages at intervals of 6 to 12 ms.

17. Cont.
3- GM-CSF:Experimental therapy with GM-CSF has been
used in patients with PAP. Preliminary data suggests that
the proportion of responders to GM-CSF is less than with
whole lung lavage. Given the experimental nature of GM-
CSF therapy, we use lung lavage as primary therapy.
4-Other therapies — Lung transplantation has been
performed in patients who deteriorate despite whole lung
lavage, but recurrence in the allograft has been reported .
Treatment with plasmapheresis and rituximab have
yielded mixed results .