A 43 year-old nonsmoker female with progressive dyspnea and hypoxic respiratory failure referred to our transplant center at Imam Khomeini General Hospital with a primary diagnosis of pathologically proven idiopathic interstitial lung fibrosis (IPF) and no response to classic treatment with high dose prednisolone and azathioprine.
She was healthy until 10 months before referral when she developed progressive dyspnea and cough with scant sputum. Primary evaluation at that time showed mild restrictive pattern on spirometry, and interstitial process in high resolution CT scan without honey combing suggestive of interstitial process like pulmonary alveolar proteinosis or nonspecific interstitial Pneumonitis (NSIP). Further evaluation with FOB and TBLB was nondiagnostic. An open lung biopsy at that time showed interstitial process compatible with NSIP. Based on the diagnosis of IPF prednisolone one milligram/kg plus azathioprine 150 mg were administered for the patient. No improvement was observed after 3 months of therapy and she got worse. She was put on long term oxygen therapy (5 liters/min) by nasal prong. However, she got worse and became wheelchair bound during the 6 months period of treatment and her physician decided to transfer her to our center for lung transplant.
At the time of first visit she had dyspnea and tachypnea on bed despite 5 liters of O2 by nose prong. She had a Cushingoid face with cyanotic lips. Thoracic examination showed no crackles on auscultation and a partial reduction in expansion. No other important findings were seen except for obesity. No clubbing was seen.
The patient was put on 12 liters of O2 with mask to improve oxygenation and further evaluations were carried out.
Laboratory data showed normal CBC and differential count, hemoglobin = 16.5 mg/dl, hematocrit = 50.7 percent and normal ALT, AST, and Alkaline Phosphatase levels. LDH was minimally elevated. Other lab test results were within the normal range. ESR and CRP were also within the normal range. Antibody for HIV, HCV, and HBS and also HBS antigen were negative. Evaluation of pre-transplant viral and parasitic markers showed positive antibody of IgG type for Epstein-Barr virus, cytomegalovirus and toxoplasmosis with low titer of IgM antibodies.
New CT scan of the lungs showed interstitial process with crazy paving pattern and no honey combing despite clinical progression of disease. Plethysmography showed well preserved lung volumes and minimal restriction (Table 1). Diffusion of the lung for CO (DLCO) was severely decreased. 
Reevaluation of the previous lung biopsy and new section of pathological block by the same and a new expert pathologist did not change the primary diagnosis of NSIP. Despite the pathologic report, re evaluation with FOB and bronchoalveolar lavage (BAL) ruled out PAP as a treatable cause. The patient was admitted to the hospital and FOB was done and BAL was obtained. BAL fluid had a completely turbid and milky appearance, highly suggestive of PAP. Positive PAS staining confirmed PAP and whole lung lavage was considered. Because of very severe hypoxia it was impossible to do the whole lung lavage with the usual technique under general anesthesia and therefore segmental bronchial washing with FOB was considered.
FOB was done under local anesthesia at the bronchoscopy unit. Midazolam and morphine sulfate were administered for sedation. Cardiac, blood pressure, and SPO2 monitoring was done at the time of bronchoscopy in the bronchoscopy unit. To preserve oxygenation throughout the procedure, the bronchoscope was passed through a small fit hole in the reservoir mask. Each time the bronchoscope was wedged into segmental bronchi 200 cc of warm saline was injected with 50 milliliter syringe and the fluids were then suctioned out. No balloon was used for blockage of bronchi. Both lungs were washed by warm (34 C) saline solution in 14 sessions, each time 3-5 segments with 3 liters of normal saline, based on the patient's tolerance. After the first 2 sessions, bronchoscopic washing was done as outpatient every 2-3 days.
She got better with progression of bronchoalveolar washing and no longer needed oxygen support and function class improved dramatically. At the end of therapy she was symptom free and six-minute walk test was 650 meter. New CT scan showed resolution of the interstitial process ([Figures 1A, B] and [Figures 2A, B]) and the new DLCO and plethysmography results were close to the normal range (Table 1); 12 months after lung washing she was still in good condition with no symptoms at rest or exertion and pulmonary function tests remained unchanged.
HRCT of the patient before segmental lung lavage at the level of the bronchus intermedius.