Multicenter International Cross-Sectional Evaluation of Pulmonary Alveolar Proteinosis (MICEPAP) Trial

1. Please change “Date of Visit” to “Date of Chart Review”

1. The third criteria listed under PAP diagnosis should read

   Elevated GM-CSF Ab concentration ◯ Yes ◯ No

1. Delete “Incomplete chart records in which >25% of the requested data is unable to be confirmed”

1. Please change “Date of Visit” to “Date of Chart Review”

1. Clinical form of PAP present: GM-CSF Ab ◯ Positive ◯ Negative ◯ Unknown

1. For Place of birth and Current Residence

   A. Can you please put the countries and the other option on one line

   Country: ◯ Germany ◯ US ◯ Italy ◯ Japan ◯ Other ____________

   B. Can you please list the option “Unknown” after State, Provence, Prefecture

   State:________ Provence:_________ Prefecture:___________ ◯ Unknown

1. Please change environmental smoke exposure to environmental (secondary) smoke exposure

1. Add the category “unknown” after the duration of exposure (years)

1. Please add (see section 9 to record data regarding infections) after the heading for this section

Bone Marrow

1. Add Myelofibrosis between Multiple Myeloma and Non-Hodgkin’s Lymphoma

Autoimmune Disorders

1. Add Bechet’s Disease between Autoimmune thyroiditis and Cryoglobulinemia
2. Combine Polymyositis/Dermatomyositis under the autoimmune disorders

Miscellaneous Disorders

1. Please make this section a subsection of section 5. Medical Illnesses
2. Add a space for the indication of “Other Medical History”

1. Please change the options in the category “Clinical form of PAP present:” to the following
   • GM-CSF Ab ◯ Positive ◯ Negative ◯ Unknown

     ◯ Surfactant Metabolic Dysfunction Disorder: due to mutations in

     • ◯ SP-B ◯ ABCA3 ◯ SP-C

1. Please add the following information after the heading for this section (defined as when the patient first went to the doctor with symptoms)
2. Please add the following to the list of symptoms and signs present at onset of PAP
   • Crackles ◯Yes ◯ No ◯ Unknown
   • Weight Loss ◯Yes ◯ No ◯ Unknown
3. Please add Vital Signs after the list of symptoms and signs present at onset of PAP

   Temperature	F or C
   Respiration Rate
   Pulse	bpm
   BP	sitting standing or supine
   % Saturation	sitting standing or supine

4. Arterial Blood Gas Please add FiO₂ ◯ Room Air ◯ O₂ _____L/min

1. Please add the following statement after the heading for this section

   If the diagnosis was made at the same time as the clinical presentation, please see instructions for section 8 in the manual of operations.

2. Please verify that the following question is indicated under the title for this section-

   At the time of diagnosis, which of the following symptoms and signs were present?

3. Please add the following to the list of symptoms and signs present at onset of PAP
   • Crackles ◯Yes ◯ No ◯ Unknown
   • Weight Loss ◯Yes ◯ No ◯ Unknown
4. Please add Vital Signs after the list of symptoms and signs present at onset of PAP

   Temperature	F or C
   Respiration Rate
   Pulse	bpm
   BP	sitting standing or supine
   % Saturation	sitting standing or supine

5. Please verify that the following question is asked after the Vital Signs and before the data for the Chest X-ray -

   At the time of diagnosis, what studies were done?

6. Complete Blood Count please add the option to include platelet (Plt) data

   Complete blood count ◯Yes ◯No◯Unknown Result(1000’s):WBC:___ Hb:___Hct:___Plt: ___

7. Arterial Blood Gas Please add FiO₂ ◯ Room Air ◯ O₂ _____L/min

1. To the question “What organism was identified?
   • Please change
     • Mycobacterium (Not TB) to Non-tuberculosis Mycobacteria
     • TB to Mycobacterium tuberculosis
   • Please add
     • Pneumocystis carinii

1. Please subscript the “2” in the following category
   • Long Term O₂ therapy: ◯ Yes ◯ No ◯ Unknown
2. Please add a section to indicate the duration of the GM-CSF treatment when dose and frequency are administrated
   • Duration__________ (month)