A 47-year-old woman was referred to our team for WLL 10 years ago (2008). At that time, she presented some symptoms, mainly increasing dyspnea, for 6 months. An open lung biopsy had been performed at her hospital to establish the diagnosis of PAP. Pulmonary function tests performed in our center showed a light restrictive syndrome, a DLCO at 58%, and a PaO2 of 68 mmHg. Radiological investigation revealed a homogeneous distribution with the involvement of bilateral superior lobes, middle lobe, and bilateral supero-dorsal segment of the inferior lobe. BAL confirmed the diagnosis of PAP.
A first bilateral WLL was performed, and moderately effective results were obtained. During the next 2.5-year period, the patient underwent six bilateral WLL, at intervals varying between 4 and 12 months, without good improvement in the clinical status, laboratory results, and radiological imaging. During the last WLL, BAL was performed as the radiological infiltrations were localized mainly in the left superior lobe and the supero-dorsal segment of the bilateral inferior lobes.
Nine months later, the patient complained about the same symptoms without marked improvement following any of the performed WLL. The dosage of GM-CSF was measured, and the dosage result, 203 μg/mL (N < 3 μg/ml), confirmed the diagnosis of primary PAP. In the following months, the patient received GM-CSF, but this treatment was ended because no improvement occurred and many secondary effects were observed. A few months later, the patient was placed on rituximab (Rituxan®), but the treatment was also ended after a few cycles since there was clinical and radiological deterioration.
Given this situation, we performed a new WLL, associated with specific BAL. The sediment recuperation was increased following the BAL. In the following days after the recovery from the WLL, we began GM-CSF by inhalation, once daily. At 1- and 3-month follow-up, the patient presented a significant improvement of her clinical status, for the first time since the first WLL. The radiologic images were completely cleared with this associated therapy, but the laboratory investigation remained stable.
We followed her to evaluate the long-term effect of this therapy. Up to 4 years after the last WLL, she received GM-CSF by inhalation following the yearly CT scan imaging, since the images reported small infiltrations. The last follow-up was done 8 years following the last WLL, and she was still asymptomatic. The annual CT scan was clean, as for the last four ones, and she did not take any GM-CSF inhalation.
This case report promotes the usefulness of a multimodal therapy that should be carried out to efficiently treat patients suffering from PAP.