Mysore/Mysuru: A 42-year-old male patient had reported to the Department of Pulmonology in Manipal Hospital, Mysuru, with chief complaint of breathlessness. A lung CT scan had revealed a rare lung condition called ‘Pulmonary Alveolar Proteinosis’ (PAP).

The patient, an electrician by profession, had started developing breathlessness and fatigue for a past few months. He had no other significant medical history or habit history that could have caused breathlessness.

However, in the last two months his condition worsened and was finding it difficult to manage his professional work and day-to-day activities.

He had earlier consulted a doctor elsewhere and had done a lung CT scan that revealed he was suffering from a very rare lung condition called PAP. Later the patient consulted Dr. Lakshmi Narasimhan, Consultant – Pulmonolgy at Manipal Hospital.

Dr. Lakshmi Narasimhan said, “PAP is a rare lung condition typically caused by accumulation of pulmonary surfactant within the alveoli of the lung. Pulmonary surfactant is a proteinaceous substance with traces of fat. Its main function is to coat the alveoli and prevent alveoli from collapsing during exhalation.” Speaking about the causes he said, “The condition can be congenital, secondary to any pulmonary infections that reduce the number of functionally effective alveolar macrophages, or may develop as an autoimmune condition. In the current case, the cause was determined as autoimmune.”

Discussing about the treatment for PAP, Dr. Narasimhan added that “The standard treatment for PAP is GM-CSF injection. However, currently, in India GM-CSF injection is unavailable and hence they had to consider Lung Lavage to remove the accumulated pulmonary surfactant from the patient’s lungs.”

Lung Lavage or bronchoalveolar lavage was performed by Dr. Narasimhan and team. During the procedure the doctors removed 9 litres of accumulated proteinaceous fluid from the patient’s lungs. There was a significant improvement in the patient’s condition and his oxygen saturation levels also improved from 75% before the procedure to 98% following the procedure.

Dr. Narasimhan said the patient was perfectly fine to resume his daily activities and professional work. He is maintaining oxygen saturation of 98% even 2 months after the procedure and has stopped using supplemental oxygen completely.

Lung Lavage is not a routine procedure and it was performed for the first time ever in Mysuru because the Pulmonology Department at Manipal Hospital had the advanced technology required for the procedure and a team of eminent doctors.

‘Make GM-CSF injections available in India’

Although the patient recovered well after the procedure, failure to procure and administer GM-CSF injections may mean that the procedure may have to be repeated after six months or so. Administration of GM-CSF injection was the gold standard treatment for PAP. Timely unavailability of this injection may lead to severe complications and cases may even worsen to the extent of requiring a lung transplant. Hence, the State and Central Government health bodies should take note of this and work towards making GM-CSF injections readily available in the Indian market.” —Dr. Lakshmi Narasimhan