Prednisolone 0.5 mg/kg/day for 4 weeks; 0.25 mg/kg/day for 4 weeks; 0.125 mg/kg/day for 4 weeks. Then taper by 5 mg every 2 weeks and discontinue. All doses will be rounded off to the nearest 5 mg (maximum duration of therapy, 4 months)

Deflazacort 0.75 mg/kg/day for 4 weeks; 0.375 mg/kg/day for 4 weeks; 0.1875 mg/kg/day for 4 weeks. Then taper by 6 mg every 2 weeks and discontinue. All doses will be rounded off to the nearest 6 mg (maximum duration of therapy, 4 months)

Allergic Bronchopulmonary Aspergillosis

Deflazacort

Prednisolone

Chest Clinic, Dept. of Pulmonary Medicine

Deflazacort vs. Prednisolone in Acute-stage ABPA

A Randomized Controlled Trial of Deflazacort vs. Prednisolone in Acute-stage Allergic Bronchopulmonary Aspergillosis

Weight at 2 months minus the baseline weight

((Baseline IgE minus serum total IgE at 2 months)/Baseline IgE) * 100

((Baseline IgE minus serum total IgE at 4 months)/Baseline IgE) * 100

Decline in serum total IgE levels by ≥25% AND clinical improvement or partial/total clearance (>50%) of chest radiographic lesions after two months

Decline in serum total IgE levels compared to the value at 2 months AND clinical improvement or partial/total clearance (>50%) of chest radiographic lesions after two months

50% increase in the 'new' baseline serum total IgE levels along with clinical or radiological deterioration

Cushingoid habitus, acne, striae, hypertension, hyperglycemia

Ritesh Agarwal

Professor

Postgraduate Institute of Medical Education and Research

Weekly subcutaneous immunoglobulin therapy (0.1g/Kg) Cuvitru 20% Injectable Solution for 1 Year

Standard Care arm without immunoglobulin replacement therapy

Bronchiectasis

Immunoglobulin Subclass Deficiency

Cuvitru 20 % Injectable Solution

Royal Infirmary of Edinburgh

Immunoglobulin Replacement Therapy for Immunoglobulin G Subclass 2 Deficient Patients With Bronchiectasis

Immunoglobulin Replacement Therapy for Immunoglobulin G Subclass 2 Deficient Patients With Bronchiectasis- A Proof of Concept Study

With IgG replacement therapy, does the sputum bacterial load change in treated versus untreated group

With IgG replacement therapy, does the frequency with >=10(6) colony forming units/ml change in treated versus untreated group?

Does sputum colour change in treated versus untreated group?

Does 24 hour sputum volume change in treated versus untreated group

Does Sputum microbiome change in treated versus untreated group

Does forced expired volume in 1 second change in treated versus untreated group

Does forced expired volume in 1 second change % predicted in treated versus untreated group

Does forced vital capacity in treated versus untreated group

Does forced vital capacity % predicted in treated versus untreated group

Does the incremental shuttle walk test change in treated versus untreated group

Does cough related quality of life change in treated versus untreated group using the Leicester Cough Questionnaire mean score (range 3-21)

Does cough related quality of life change in treated versus untreated group using the Leicester Cough Questionnaire proportion that have 1.3 or more Unit improvement (minimum clinically important difference)

Does quality of life change in treated versus untreated group using the St George's Respiratory Questionnaire proportion that have 4 Unit or more improvement (minimum clinically important difference)

Does quality of life change in treated versus untreated group using the St George's Respiratory Questionnaire mean score (range 0-100)

Does sputum myeloperoxidase change in treated versus untreated group

Does sputum elastase change in treated versus untreated group

Does sputum interleukin 8 change in treated versus untreated group

Do serum IgG and subclasses change in treated versus untreated group

Do serum white cell count change in treated versus untreated group

Do serum C reactive protein change in treated versus untreated group

Do serum erythrocyte sedimentation rate change in treated versus untreated group

Do serum intercellular adhesion molecule 1 change in treated versus untreated group

Does peripheral blood neutrophil phagocytosis and killing of Pseudomonas aeruginosa change in treated versus untreated group

Does peripheral blood neutrophil phagocytosis and killing of Staphylococcus aureus change in treated versus untreated group

Is there antibody deficiency with pneumococcal vaccination

Is the time to first exacerbation requiring antibiotic therapy change in treated versus untreated group

Does the number of exacerbations change in treated versus untreated group

Does the mean exacerbations change in treated versus untreated group

What are the number of participants with side effects of IgG therapy

What are the compliance rates of IgG therapy

What are the completion rates of IgG therapy

No plan to share IPD- will present overall data

University of Edinburgh

NHS Lothian

1 Group receiving active immunoglobulin replacement therapy

This group will be comprised of healthy individuals without evidence of lung disease.

This group will be comprised of individuals who have been diagnosed with cystic fibrosis.

Samples of induced and expectorated sputum and blood for DNA and RNA extraction, plasma, and serum will be banked in the UCSF Airway Tissue Bank.

aspergillosis/carbohydrate

Deflazacort vs. Prednisolone in Acute-stage ABPA

Immunoglobulin Replacement Therapy for Immunoglobulin G Subclass 2 Deficient Patients With Bronchiectasis

CF And Effects of Drugs Mixed Ex Vivo With Sputum for Mucolytic Treatment

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