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Case Report

Case Report. 3rd Department of Respiratory Disease. Introduction of case. Female, 44 ys, farmer, from Fuyang city , A nhui Province Chief Complaint : Recurrent cough, sputum, dyspnea for more than 1 year History of present illness ：

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Case Report

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  1. Case Report 3rd Department of Respiratory Disease

  2. Introduction of case • Female, 44 ys, farmer, from Fuyang city,Anhui Province • Chief Complaint: Recurrent cough, sputum, dyspneafor more than 1 year • History of present illness： April 2009:the patient began to have a recurrent cough, sputum, dyspnea,intermittent treated with anti-infection, symptomatic treatment. no significant improvement.

  3. Introduction of case • December 2009: Cough, sputum, dyspnea aggravated, with fatigue, diagnosed as interstitial pneumonia in the local hospital, received anti-infection and symptomatic treatment, and intravenous methylprednisolone 40mg qd for 4 days and oral prednisone 30mg qd, no significant improvement. • 2010.1.11: Admitted to the General Hospital of Chinese People's Liberation Army with increased dyspnea, diagnosis of ILD, treat with intravenous methylprednisolone 40mg and anti-infection, dyspnea improved. • 2010.2.8 discharged, oral methylprednisolone 32mg qd, • 2010.2.18 self-withdrawal methylprednisolone.

  4. Introduction of case • 2010.3.13: Cough, sputum, dyspnea aggravated for 3 days, methylprednisolone 40mg qd ivgttand anti-infection, no significant improvement in local hospital. So came to our hospital. • 2010.3.23:First admission to our department, diagnosis of "interstitial lung disease, PAP ?, Lung secondary infection , hypoxemia.”

  5. Introduction of case • Treated with Methylprednisolone 40mgqd and meropenem, azithromycin. • Symptoms improved, chest CT: lesions slightly improved. • Then oral prednisone 35mg qd, reduction of 5mg qd each half month,dyspneaimproved.

  6. Introduction of case • After communication with the patient and her families about the advantages and disadvantages of FOB and BALF, they rejected. • But they agree with further examinations such as: VATS lung biospy. • 2010.6.7:Prednisone was reduced to 15mg qd for surgery.

  7. Introduction of case • Without history of hypertension, diabetes, tuberculosis. • Non-smoking. • No history of raw crabs, pet. • Non-toxic, dust exposure, no history of long-term medication history. • No family history of genetic disease.

  8. Physical examination • A little Velcro sound in lower lungs

  9. Laboratory tests • Arterial blood gas analysis：No oxygen: PaO2:58 mmHg、SaO2:89.6 %。 Oxygen:nomal。 • Blood Rt:WBC:16.0 ×109/L、N%:83.80 % • LDH:359↑IU/L. • LDL-C:4.07↑mmol/L,TCH:6.61↑mmol/L. • ESR、CRP、SACE、BNP(-) • Rheumatic markers : （-） • Allergen(-) • Cancer markers（-）

  10. Rheumatic markers • anti-nuclear antibodies, • anti-double-stranded DNA antibodies, • anti-RNP antibodies, • anti-Sm antibodies, • anti-Scl-70 antibodies, • anti-Jo-1 antibodies, • anti-SSA, anti-SSB, • anti-neutrophil cytoplasmic antibodies (ANCA: MPO, PR3)

  11. Laboratory tests Sputum culture: No bacteria. No mycobacterium tuberculosis No fungus

  12. Special examination • Nomal EKG. • Ultrasound: mild fatty liver.

  13. Chest imaging 2010-3-23 2010-6-18 significant hilar infiltration mimicking cardiogenic pulmonary edema

  14. Characteristic “Geographic” or “Crazy-Paving” signs 2010-5-19 胸部影像学检查 2010-3-22

  15. 2010-3-22 胸部影像学检查 Characteristic “Geographic” or “Crazy-Paving” signs

  16. Pathological examination • 2010.6.7:partial resection of left upper lobe in our hospital • Pathological special dye：PAS(+) • Pathology：Left upper lobe pulmonary alveolar proteinosis

  17. Diagnosis

  18. Diagnosis • pulmonary alveolar proteinosis,PAP

  19. Discussion • PAP is a rare disease • Characterized by intermittent alveolar accumulation of phospholipid-rich protein substances( PAS positive) that affect the alveolar gas exchange, leading to dyspnea, hypoxemia and a series of clinical syndrome. • PAP can be divided into primary, secondary, and congenital types. • 90% primary，unknow causes.

  20. Am J Respir Crit Care Med Vol 166. pp 215–235, 2002

  21. Discussion • Currently, the most commonly treatment for PAP is whole-lung lavage. • However, the therapy must be carried out under general anesthesia, high equipment requirement, risk, and the effect can not be sustained.

  22. Discussion • Untill 1994, GM-CSF knockout mice lungs appeared similar performance of PAP • Primary PAP may be associated with the lack of GM-CSF 1.Stanley E.Natl Acad Sci USA,1994,91:5592-5596 2.Dranoff G,Science,1994,264:713-716

  23. Lessons from murine studies Mice that lack the gene for GM-CSF still have normal steady-state hematopoiesis, but the animals develop pulmonary disease consistent with PAP. This finding led to speculation that GM-CSF may be important in surfactant homeostasis. These mice have too much surfactant in their lungs. Further work confirmed that the surfactant had normal bioactivity, but it was not being broken down.

  24. Chest 2006;130;227-237

  25. CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 68 • NUMBER 12 DECEMBER 2001

  26. Discussion • Bonfield suggested that anti-GM-CSF autoantibodies should be as a primary diagnostic criteria of PAP. • Sensitivity is 100%，Specificity is 91%. Bonfield.Am J Respir Cell Mol Biol,2002,27:481-486

  27. Discussion • In a clinical trial conducted by Kavuru, 4 patients with primary PAP were treated with whole-lung lavage , poor efficacy. • Then the patients were treated with 12 weeks of subcutaneous injection of GM-CSF. • Chest x-ray, pulmonary function, blood gas analysis and the 6-minute walk distance indicators of three patients were significantly improved. Kavuru MS. Am J Respir Crit Care Med, 2000,161:1143-1148

  28. Before treatment After treatment bilateral perihilar infiltrates withoutevidence of cardiomegaly, adenopathy, or effusion. clearing of the previously seen infiltrates after 4 months of GM-CSF therapy.

  29. Discussion • One patient with primary PAP was treated with 4 weeks of subcutaneous injection of GM-CSF, after whole-lung lavage treatment of poor efficacy, dyspneawas alleviated. • After three months, lesions in both lungs of chest CT, blood gas analysis and pulmonary function were signaficantly improved than before. Zhang Wei. Chin J Respir Crit Care Med.2008,7(4):298

  30. After WLL Before WLL • image no improvement of symptoms and image BeforeGM-CSF After subcutaneous GM-CSF×3months WBC PaO2

  31. Discussion • 2010.6.18: readmission after surgery, gradual reduction of prednisone, antibiotics, CT scan showed that lesions did not change significantly. • 2010.7.2: GM-CSF 75ug, ih, biw • 2010.7.16: GM-CSF 75ug,ih,qod

  32. Discussion • Presently, cough, sputum, chest tightness, dyspnea improved, no night sweats, fever, fatigue and discomfort. • 2010.7.22:lesions in chest CT and blood gas significantly improved. • GM-CSF 75ug,ih,qod,follow up

  33. After subcutaneous GM-CSF 75ug Biw ×1months 2010-5-19 2010-7-22

  34. 2010-5-19 2010-7-22

  35. treatment After subcutaneous GM-CSF 75ug q.o.d×5months before

  36. treatment After subcutaneous GM-CSF 75ug q.o.d×5months before

  37. treatment After subcutaneous GM-CSF 75ug q.o.d×5months before

  38. Follow-up 2010-5-19 2011-10-11 GM-CSF Withdrawal for 16 Months

  39. Follow-up 2010-5-19 2011-10-11 Drug Withdrawal for 16 Months

  40. Follow-up 2011-10-11 2010-5-19 Drug Withdrawal for 16 Months

  41. Discussion • Subcutaneous injection of GM-CSF is an alternative treatment for primary PAP • It still need large-scale clinical trials to evaluate the efficacy of GM-CSF treatment in PAP .

  42. Thank You!

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