Spotter of the week

29 MAY 2015

What Renuka Says Spotter Series ...

Dr Ranjith R, MEM Y-II

29 year old male with 1 month history of Progressively increasing dyspnoea, Cough with mucoid sputum

Working in a petrol pump, but no obvious exposure to dust, smoke, pets

Clinically stable :
ABCs normal, Vitals stable, AEBE, B/L Creps +

Pulmonary Alveolar Proteinosis (PAP)

• Diffuse lung disease characterized by the accumulation of amorphous, periodic acid-Schiff (PAS)- positive lipoproteinaceous material in the distal air spaces
• There is little or no lung inflammation, and the underlying lung architecture is preserved
• Types :

              Congenital
              Acquired
              Secondary

• Pathology :

              Impaired processing of surfactant by alveolar macrophages contributes to the pathogenesis of PAP

• Clinical Manifestations :

            Age : 30 – 50
            Sex : Male – Female (2 : 1)
            Symptoms :
               Progressive dyspnea on exertion
               Cough
               Fatigue
               Weight loss
               Low-grade fever
            Signs :
               Normal
               Clubbing and cyanosis
               Crackles

• Labs :

              Polycythemia
              Hypergammaglobulinemia
              Increased LDH levels
              Increased serum anti-GM-CSF titers
              Sputum : PAS-positive material in macrophages

• CXR  :

              Bilateral symmetric alveolar opacities located centrally in mid and lower lung zones are typical, often in a "bat wing" distribution

HRCT :
Ground-glass opacification, predominantly in a homogeneous distribution.
Thickened intralobular structures and interlobular septa in typical polygonal shapes, referred to as "crazy-paving“

BAL :
An opaque or milky appearance due to the abundant lipoproteinaceous material

Treatment :
Whole lung lavage
Subcutaneous GM-CSFc