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Respiratory Diseases Dataset – Lung & Breathing Disorders Database
Respiratory Diseases Dataset
The Respiratory Diseases Dataset is a structured medical database containing a comprehensive list of conditions affecting the lungs and respiratory system.
Respiratory diseases such as asthma, chronic obstructive pulmonary disease (COPD), and pneumonia are major global health concerns, impacting millions of people worldwide. This dataset provides organised information to support medical research, healthcare analytics, and application development.
Each record includes key clinical details such as disease descriptions, affected respiratory structures, common symptoms, severity levels, and treatment approaches.
The dataset has been cleaned and structured for easy integration into spreadsheets, databases, and data analysis tools.
It is ideal for healthcare developers, researchers, educators, and data scientists working with respiratory health data.
Dataset Contents
The dataset includes fields such as:
- Disease / Condition Name
- Description
- Affected Area (Lungs, Airways, Respiratory System)
- Common Symptoms
- Severity Level
- Disease Category
- Risk Factors
- Treatment / Management
Example Conditions Included
- Asthma
- Chronic Obstructive Pulmonary Disease (COPD)
- Pneumonia
- Tuberculosis
- Bronchitis
- Lung Cancer
- Pulmonary Fibrosis
- Emphysema
- Sleep Apnea
- Acute Respiratory Distress Syndrome (ARDS)
...and many more respiratory conditions.
Data Preview
12 columns
25 rows shown
| No. | Disease Name | Category | Primary Cause / Etiology | Prevalence | Age of Onset | Key Symptoms | Affected Respiratory Structure | Diagnostic Method | Treatment Approach | Prognosis | ICD-10 Code | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | ▸ OBSTRUCTIVE AIRWAY DISEASES (16 disorders) | |||||||||||
| 2 | 1 | Allergic Bronchopulmonary Aspergillosis (ABPA) | Obstructive Airway Diseases | Hypersensitivity to Aspergillus fumigatus colonizing airways; Th2-driven IgE response; occurs in asthma (1-2%) and CF (7-9%); mucoid impaction and bronchial damage | Affects 1-2% of asthmatics and 7-9% of CF patients; geographic variation; more common in tropical regions | Usually 20-40 years; in CF patients, adolescence to young adulthood | Worsening asthma, recurrent pulmonary infiltrates, productive cough with brown mucus plugs, hemoptysis, fever during exacerbations, progressive bronchiectasis | Proximal (central) bronchiectasis pathognomonic; mucoid impaction with eosinophilic mucin containing fungal hyphae; airway destruction over time | Elevated total IgE >1000 IU/mL; positive Aspergillus skin test or specific IgE; serum precipitins; blood eosinophilia >500; CT showing central bronchiectasis and mucoid impaction; Rosenberg criteria | Oral prednisone 0.5mg/kg for 2 weeks then taper over 3-5 months; itraconazole 200mg BID for 4-6 months (reduces steroid need); omalizumab for refractory cases; monitoring total IgE for relapse | Chronic relapsing course; 5 stages from acute to fibrotic; early treatment preserves lung function; fibrotic stage (Stage V) irreversible; long-term monitoring of IgE levels essential | B44.81 |
| 3 | 2 | Alpha-1 Antitrypsin Deficiency Emphysema | Obstructive Airway Diseases | Genetic deficiency of alpha-1 antitrypsin (SERPINA1 gene); ZZ phenotype most severe; uninhibited neutrophil elastase destroys alveoli; smoking dramatically accelerates disease | 1 in 2,500-5,000 of European descent; only 5-10% diagnosed; accounts for 1-3% of COPD | 30-50 years (earlier if smoking); panlobular emphysema predominantly basal | Progressive dyspnea, wheezing, reduced exercise capacity; lower lobe predominant emphysema (unlike smoking-related); hepatic disease in 10-15%; panniculitis rare | Panlobular emphysema predominantly lower lobes; hepatic inclusions of polymerized AAT; accelerated FEV1 decline | Serum AAT level <11 μmol/L (57 mg/dL); Pi typing or SERPINA1 genotyping; CT chest showing basal emphysema; spirometry; liver function tests | IV augmentation therapy (Prolastin, Zemaira) 60mg/kg weekly; standard COPD treatment; smoking cessation critical; lung transplant for severe disease; liver transplant if needed | Without augmentation: FEV1 decline 70-100ml/year; with augmentation: decline reduced to 40-50ml/year; never-smokers have better prognosis; median survival 60+ years if never-smoked | E88.01 |
| 4 | 3 | Asthma (Allergic) | Obstructive Airway Diseases | Type I IgE-mediated hypersensitivity; genetic predisposition (ADAM33, ORMDL3); aeroallergens (dust mites, pollen, pet dander); atopic triad | 339 million worldwide; 8-10% of adults; prevalence rising in developed nations | Childhood onset typical (80% before age 6); can present at any age | Episodic wheezing, dyspnea, chest tightness, cough (worse at night); triggered by allergens, exercise, cold air; symptom-free intervals | Bronchial smooth muscle hyperresponsiveness; eosinophilic airway inflammation; mucous hypersecretion; reversible airflow obstruction | Spirometry with reversibility (FEV1 >12% and 200ml post-BD); methacholine challenge; FeNO >25ppb; skin prick testing; serum IgE and eosinophils | ICS (fluticasone, budesonide) cornerstone; SABA PRN (salbutamol); LABA add-on (formoterol); LTRA (montelukast); biologics for severe: omalizumab (anti-IgE), mepolizumab (anti-IL5), dupilumab (anti-IL4R) | Excellent prognosis with treatment; mortality <1% with proper management; 30-50% of children outgrow symptoms; uncontrolled asthma increases airway remodeling risk | J45.20 |
| 5 | 4 | Asthma (Non-Allergic/Intrinsic) | Obstructive Airway Diseases | Non-IgE-mediated; triggers include infections, exercise, cold air, irritants, stress, GERD, hormonal changes; neutrophilic airway inflammation predominant | 10-33% of all asthma cases; more common in adults; F:M 2:1 | Adult onset typical (>30 years); often more severe than allergic asthma | Persistent dyspnea, cough, wheezing; less episodic than allergic asthma; poor response to allergen avoidance; often associated with nasal polyps and aspirin sensitivity | Neutrophilic or pauci-granulocytic airway inflammation; airway remodeling more pronounced; subepithelial fibrosis | Spirometry with reversibility; negative skin prick tests and normal IgE; FeNO often normal; CT sinuses for polyps; aspirin challenge if suspected | Higher-dose ICS often required; LABA/ICS combination; tiotropium add-on; macrolide therapy (azithromycin) for neutrophilic; anti-IL5 biologics; aspirin desensitization if AERD | More persistent symptoms; less responsive to ICS; greater decline in lung function; 5-year remission rate lower (10%) vs allergic asthma (25%) | J45.30 |
| 6 | 5 | Bronchiectasis (Non-Cystic Fibrosis) | Obstructive Airway Diseases | Post-infectious (TB, pneumonia); immune deficiency (CVID, IgA deficiency); ciliary dyskinesia; ABPA; autoimmune (RA, IBD); idiopathic in 30-50% | Prevalence 350-566 per 100,000 in >65 years; increasing with CT availability; F:M 1.5:1 | Any age; post-infectious forms often childhood onset; idiopathic peaks at 60-70 years | Chronic productive cough with copious purulent sputum, recurrent respiratory infections, hemoptysis (50-70%), dyspnea, fatigue, rhinosinusitis | Permanent bronchial dilation; vicious cycle of infection, inflammation, and structural damage; typically lower lobe predominant | HRCT chest (gold standard): bronchial dilation, signet ring sign, lack of tapering; sputum culture; immunoglobulin levels; CF screening; ciliary function tests | Airway clearance techniques; long-term macrolides (azithromycin 250mg 3x/week); inhaled antibiotics (tobramycin, colistin) for Pseudomonas; mucolytics; bronchodilators; surgical resection for localized disease | Chronic progressive condition; Pseudomonas colonization worsens prognosis; annual FEV1 decline 50-55ml; 5-year mortality 10-16%; frequent exacerbations predict worse outcomes | J47.9 |
| 7 | 6 | Bronchiolitis Obliterans (Constrictive) | Obstructive Airway Diseases | Fibrotic narrowing of small airways; post-transplant (BOS in lung/HSCT); post-infectious; inhalational injury (diacetyl, sulfur mustard); drug-induced; autoimmune (RA) | Affects 50-60% of lung transplant recipients by 5 years; rare otherwise; occupational forms well-documented | Any age depending on cause; post-transplant: 6 months to years after transplant | Progressive dyspnea, dry cough, wheezing unresponsive to bronchodilators; irreversible airflow obstruction; mid-inspiratory squeaks on auscultation | Concentric fibrosis and obliteration of membranous and respiratory bronchioles; mosaic attenuation on CT | PFTs showing fixed obstruction (FEV1 decline >20% from baseline); HRCT with mosaic attenuation and air trapping on expiration; surgical lung biopsy (definitive but rarely done); exclusion of other causes | Limited efficacy; azithromycin 250mg 3x/week; augmented immunosuppression post-transplant; re-transplantation for severe BOS; FAM protocol (fluticasone/azithromycin/montelukast) | Poor prognosis once established; median survival 3-5 years after BOS diagnosis; progressive decline; re-transplant only curative option; overall 5-year survival post-lung transplant 55% | J44.81 |
| 8 | 7 | Chronic Bronchitis | Obstructive Airway Diseases | Chronic inflammation of bronchial mucosa; smoking primary cause (>90%); air pollution, occupational dusts; defined clinically by chronic productive cough | Affects 3-7% of adults; higher in smokers (15-25%); often coexists with emphysema in COPD | >40 years typically; insidious onset in chronic smokers | Productive cough with sputum on most days for ≥3 months in ≥2 consecutive years; dyspnea; wheezing; frequent acute exacerbations; cyanosis in advanced ('blue bloater') | Mucous gland hypertrophy and hyperplasia; goblet cell metaplasia; Reid index >0.5; airway wall thickening and narrowing; mucociliary clearance impairment | Clinical diagnosis (cough criteria); spirometry may show obstruction; chest X-ray: increased bronchovascular markings; HRCT: bronchial wall thickening; sputum culture for exacerbations | Smoking cessation (most important); bronchodilators (LABA/LAMA); ICS for frequent exacerbators; mucolytics (N-acetylcysteine, erdosteine); antibiotics for acute exacerbations; pulmonary rehabilitation | Progressive if smoking continues; FEV1 decline accelerated; smoking cessation slows progression to near-normal rates; 10-year mortality 40-50% if moderate-severe obstruction | J42 |
| 9 | 8 | Chronic Obstructive Pulmonary Disease (COPD) | Obstructive Airway Diseases | Tobacco smoking (85-90%); alpha-1 antitrypsin deficiency; biomass fuel exposure; occupational dusts | ~390 million globally (GBD 2019); 3rd leading cause of death worldwide; prevalence 10-12% in adults >40 | Usually >40 years; progressive onset over decades | Progressive dyspnea, chronic productive cough, wheezing, exercise intolerance, frequent exacerbations; barrel chest, pursed-lip breathing in advanced disease | Airways and lung parenchyma; chronic bronchitis (airways) and emphysema (alveoli); airflow limitation | Spirometry: post-BD FEV1/FVC <0.70 (GOLD criteria); chest X-ray/CT; alpha-1 antitrypsin level; ABG in advanced disease | Smoking cessation; bronchodilators (LABA/LAMA: tiotropium, salmeterol); ICS for frequent exacerbators; pulmonary rehab; LTOT if PaO2 <55mmHg; lung volume reduction surgery | Progressive decline; FEV1 loss 30-60ml/year; 5-year survival 40-70% depending on GOLD stage; exacerbations accelerate decline | J44.9 |
| 10 | 9 | Cystic Fibrosis | Obstructive Airway Diseases | Autosomal recessive; CFTR gene mutations (>2000 known); F508del most common (70%); defective chloride channel causing thick secretions | 1 in 2,500-3,500 Caucasian births; carrier frequency 1 in 25; less common in other ethnicities | Diagnosed in infancy/childhood via newborn screening; median diagnosis age <1 year in screened populations | Progressive bronchiectasis, chronic Pseudomonas infection, pancreatic insufficiency, malnutrition, CF-related diabetes, male infertility, digital clubbing, nasal polyps | Lungs: thick mucus, chronic infection, progressive bronchiectasis; pancreas, liver, intestines, sweat glands all affected | Newborn screening (IRT); sweat chloride >60 mmol/L (diagnostic); CFTR genotyping; pancreatic elastase; sputum cultures; CT chest; spirometry | CFTR modulators: elexacaftor/tezacaftor/ivacaftor (Trikafta) for F508del (90% of patients); airway clearance; inhaled dornase alfa, hypertonic saline; azithromycin; inhaled tobramycin; pancreatic enzymes; lung transplant for end-stage | Dramatically improved with CFTR modulators; median survival now >50 years (was 30 in 2000); Trikafta improves FEV1 by 14% points; lung transplant 5-year survival 50-60% | E84.0 |
| 11 | 10 | Emphysema (Centrilobular) | Obstructive Airway Diseases | Smoking-related destruction of respiratory bronchioles and proximal alveoli; protease-antiprotease imbalance; oxidative stress; predominantly upper lobe distribution | Most common subtype of emphysema; found in >50% of chronic heavy smokers; part of COPD spectrum | >50 years typically; correlates with smoking pack-years (>20 pack-years) | Progressive exertional dyspnea, minimal sputum ('pink puffer'), weight loss, hyperinflation, accessory muscle use, pursed-lip breathing, decreased breath sounds | Destruction of respiratory bronchioles centrally within lobule; upper lobe predominance; progressive loss of elastic recoil and gas exchange surface | CT chest: upper lobe centrilobular lucencies; PFTs: reduced DLCO, increased TLC/RV, obstructive pattern; chest X-ray: hyperinflation, flattened diaphragms | Smoking cessation; bronchodilators (LABA/LAMA); pulmonary rehab; LTOT if PaO2 <55mmHg; LVRS for upper-lobe predominant with low exercise capacity; endobronchial valves; lung transplant for advanced | Progressive decline; BODE index predicts mortality; 5-year survival ranges from 80% (GOLD I) to 30% (GOLD IV); LVRS can improve survival in select patients | J43.9 |
| 12 | 11 | Emphysema (Panlobular) | Obstructive Airway Diseases | Alpha-1 antitrypsin deficiency primarily; diffuse destruction of entire acinus; lower lobe predominance; smoking accelerates progression dramatically | Much less common than centrilobular; associated with AAT deficiency (1 in 2,500-5,000 Europeans) | 30-50 years (earlier than centrilobular); younger if homozygous ZZ and smoker | Progressive dyspnea, reduced exercise capacity; lower lobe predominant; may have concurrent liver disease; earlier onset than typical COPD | Uniform destruction of entire secondary pulmonary lobule; lower lobe predominance; loss of entire alveolar-capillary surface area | CT: diffuse lower lobe emphysema; AAT serum level; genotyping; PFTs: severely reduced DLCO; spirometry with fixed obstruction | AAT augmentation therapy (60mg/kg IV weekly); standard COPD therapies; lung transplant for severe disease; avoid smoking absolutely | Worse prognosis than centrilobular if smoking; AAT augmentation slows decline; median survival varies by FEV1 at diagnosis; lung transplant provides significant benefit | J43.1 |
| 13 | 12 | Exercise-Induced Bronchoconstriction | Obstructive Airway Diseases | Airway water and heat loss during exercise triggering mast cell mediator release; osmotic theory; occurs in 40-90% of asthmatics and 10-15% of general population | 10-15% of general population; up to 90% of asthmatics; higher prevalence in elite athletes (30-70% in winter sports) | Any age; common in children and young adults; athletes at particular risk | Cough, wheezing, dyspnea, chest tightness 5-15 minutes after exercise onset; peaks at 5-10 min post-exercise; spontaneous resolution in 30-60 min; refractory period of 1-3 hours | Transient bronchospasm triggered by airway dehydration and cooling during exercise; mast cell activation; more pronounced in cold, dry air | Exercise challenge test: FEV1 drop >10% post-exercise (diagnostic); eucapnic voluntary hyperventilation (EVH) test for athletes; mannitol challenge; baseline spirometry often normal | Pre-exercise SABA (salbutamol 200μg 15 min before); daily ICS if frequent; LTRA (montelukast 10mg daily); warm-up exercises; face mask in cold weather; avoid exercising in cold dry air | Excellent prognosis; does not limit athletic performance with proper management; does not progress to chronic asthma if isolated; well-controlled in >90% with pre-treatment | J45.990 |
| 14 | 13 | Occupational Asthma (Sensitizer-Induced) | Obstructive Airway Diseases | Workplace sensitizers: isocyanates (most common), flour dust, wood dust, latex, laboratory animals, metal salts; IgE-mediated or T-cell mediated; latency period before onset | 10-25% of adult-onset asthma; most common occupational lung disease in developed countries; incidence 20-175 per million workers/year | Working age adults 20-60; onset after latency period of months to years of exposure | Work-related wheeze, cough, dyspnea, chest tightness; symptoms improve on weekends/holidays; dual-phase response (early + late); rhinitis often precedes asthma; progressive worsening with continued exposure | Airway sensitization leading to eosinophilic inflammation and bronchial hyperresponsiveness; IgE-mediated (high-molecular-weight agents) or non-IgE (low-molecular-weight agents like isocyanates) | Serial PEF monitoring (at work vs away >2 weeks); specific inhalation challenge (gold standard); methacholine challenge showing work-related changes; specific IgE to occupational allergens; sputum eosinophils | Complete removal from exposure (most important); early removal improves prognosis; ICS/LABA for symptom control; standard asthma stepwise therapy; workers' compensation claim; workplace modification if removal impossible | Complete recovery in 30% if early removal; persistent asthma in 70% even after removal; continued exposure leads to severe irreversible asthma; better prognosis if diagnosed within 1 year of symptom onset | J45.20 |
| 15 | 14 | Severe Eosinophilic Asthma | Obstructive Airway Diseases | T2-high inflammation with persistent eosinophilia; IL-5/IL-13 driven; often steroid-dependent; genetic susceptibility (TSLP, IL33 polymorphisms) | 5-10% of all asthma; affects ~2 million in US; disproportionate healthcare burden (50% of asthma costs) | Variable onset; often adult-onset (>25 years); progressive severity | Frequent severe exacerbations despite high-dose ICS/LABA; oral steroid dependence; chronic dyspnea; mucus plugging; nasal polyps in 40-60%; anosmia | Persistent eosinophilic airway inflammation; mucus plugging with eosinophil-rich plugs; subepithelial fibrosis and airway remodeling | Blood eosinophils >300 cells/μL; sputum eosinophils >3%; FeNO >25ppb; serum periostin elevated; CT showing mucus plugging and air trapping | Biologic therapies: mepolizumab 100mg SC monthly, benralizumab 30mg SC q8wk, dupilumab 200mg SC q2wk; reduce OCS; bronchial thermoplasty in refractory cases | Significant improvement with biologics (50% reduction in exacerbations); OCS reduction achievable in 50-70%; long-term airway remodeling risk; mortality 2-3x higher than mild asthma | J45.50 |
| 16 | 15 | Status Asthmaticus | Obstructive Airway Diseases | Severe acute asthma exacerbation unresponsive to initial bronchodilator therapy; triggers: viral infection, allergen exposure, medication non-compliance, NSAID/beta-blocker use | 5-10% of asthma ED visits require ICU; 2-20% of ICU admissions for acute asthma; mortality 1-3% of hospitalized cases | Any age; bimodal peaks in children (2-5 years) and adults (20-40 years) | Severe dyspnea at rest, inability to speak in sentences, accessory muscle use, diaphoresis, silent chest (ominous), tachycardia >120, pulsus paradoxus >25mmHg, altered consciousness | Severe bronchospasm, mucous plugging, airway edema; progressive air trapping; dynamic hyperinflation; V/Q mismatch leading to hypoxemia then hypercapnia | PEF <25% predicted; ABG: initial respiratory alkalosis then acidosis; chest X-ray to exclude pneumothorax; continuous pulse oximetry; clinical assessment (inability to speak, silent chest) | Continuous nebulized salbutamol; IV magnesium sulfate 2g; systemic corticosteroids (methylprednisolone 125mg IV); ipratropium bromide; IV aminophylline; non-invasive ventilation; intubation if failing (ketamine induction preferred) | With aggressive treatment: mortality <2%; near-fatal episodes increase future risk 10-fold; ICU stay 1-5 days; full recovery expected in most; 10-25% relapse within 2 weeks | J46 |
| 17 | 16 | Tracheobronchomalacia | Obstructive Airway Diseases | Weakness of tracheal/bronchial cartilage; acquired: post-intubation, COPD, relapsing polychondritis; congenital: Williams-Campbell syndrome, Mounier-Kuhn | Found in 4-13% of patients undergoing bronchoscopy; likely underdiagnosed; M:F 2:1 | Congenital: infancy; Acquired: typically >40 years in COPD patients | Expiratory stridor, barking cough, dyspnea worsening with exertion, difficulty clearing secretions, recurrent infections, exercise intolerance | Trachea and/or main bronchi with >50% collapse during expiration due to cartilage weakness or posterior membrane redundancy | Dynamic bronchoscopy showing >50% airway collapse on expiration (gold standard); dynamic CT with paired inspiratory/expiratory images; PFTs may show variable intrathoracic obstruction | CPAP/BiPAP for symptom relief; airway stenting (silicone or metallic) for severe cases; tracheobronchoplasty (surgical posterior membrane stabilization); treat underlying cause | Variable; mild cases managed conservatively; stenting provides symptomatic improvement in 80%; surgical tracheobronchoplasty successful in 85%; chronic management often needed | J39.8 |
| 18 | ▸ INTERSTITIAL LUNG DISEASES (16 disorders) | |||||||||||
| 19 | 17 | Acute Respiratory Distress Syndrome (ARDS) | Interstitial Lung Diseases | Diffuse alveolar damage from pulmonary (pneumonia, aspiration) or extrapulmonary (sepsis, pancreatitis, trauma) causes; neutrophilic inflammation destroying alveolar-capillary barrier | Incidence 10-86 per 100,000; accounts for 10% of ICU admissions; 3 million cases annually worldwide | Any age; most common in adults 50-70; neonatal form (IRDS) in prematures | Acute onset bilateral hypoxemia, severe dyspnea, tachypnea, diffuse bilateral crackles; PaO2/FiO2 ratio defines severity: mild 200-300, moderate 100-200, severe <100; Berlin criteria | Diffuse alveolar damage: exudative (0-7 days), proliferative (7-21 days), fibrotic (>21 days); hyaline membranes; alveolar flooding; impaired gas exchange | Berlin criteria: acute onset within 1 week, bilateral opacities on imaging, PaO2/FiO2 ≤300 on PEEP ≥5, not fully explained by cardiac failure; echocardiography to exclude cardiogenic edema | Lung-protective ventilation (6ml/kg IBW, plateau pressure <30cmH2O); prone positioning for moderate-severe (>16 hrs/day); conservative fluid management; neuromuscular blockade early; dexamethasone; ECMO for refractory hypoxemia | Overall mortality 35-45%; mild 27%, moderate 32%, severe 45%; survivors: 25% have persistent PFT abnormalities at 5 years; significant long-term cognitive/psychological morbidity; improving outcomes with better ventilator management | J80 |
| 20 | 18 | Asbestosis | Interstitial Lung Diseases | Chronic inhalation of asbestos fibers (chrysotile, crocidolite, amosite); latency 15-40 years; fibers penetrate alveoli causing chronic inflammation and fibrosis; dose-response relationship | Declining in developed nations due to asbestos bans; still prevalent in developing countries; occupational: construction, shipyard, mining workers | 50-70 years (due to long latency); insidious onset decades after exposure | Progressive dyspnea, dry cough, bibasilar crackles, clubbing (40-80%); associated with pleural plaques (>80%); increased risk of mesothelioma and lung cancer | Lower lobe predominant diffuse interstitial fibrosis; asbestos bodies (ferruginous bodies) in tissue; fibrosis similar to UIP pattern but with asbestos bodies | HRCT: subpleural lines, parenchymal bands, honeycombing (lower lobes); pleural plaques (confirms exposure); PFTs: restrictive with reduced DLCO; asbestos bodies in BAL or biopsy; occupational history essential | No specific treatment; supportive care; supplemental oxygen; smoking cessation (synergistic cancer risk); pulmonary rehabilitation; surveillance for mesothelioma and lung cancer; lung transplant rarely | Progressive fibrosis even after exposure cessation; mortality depends on severity; 5-year survival 80-90% for mild; advanced disease similar to IPF; mesothelioma risk 7-10% in heavily exposed | J61 |
| 21 | 19 | Cryptogenic Organizing Pneumonia (COP) | Interstitial Lung Diseases | Idiopathic organizing pneumonia; intraluminal buds of granulation tissue in distal airways and alveoli; secondary causes: infections, drugs, CTD, radiation | 6-7 per 100,000; equal gender distribution; non-smokers predominantly affected | 50-60 years; subacute onset over weeks to months mimicking pneumonia | Persistent cough, dyspnea, malaise, low-grade fever, weight loss; flu-like prodrome common; does not respond to antibiotics; migratory infiltrates | Granulation tissue plugs (Masson bodies) within alveolar ducts and alveoli; peribronchiolar distribution; preserved lung architecture | CT: bilateral patchy consolidation, often peripheral and migratory; ground-glass opacities; reversed halo sign (atoll sign); BAL: mixed cellularity; biopsy showing organizing pneumonia pattern | Prednisone 0.75-1mg/kg for 4-8 weeks then slow taper over 6-12 months; relapse common with rapid taper (50%); macrolides for mild cases; mycophenolate for refractory/relapsing | Excellent prognosis; 80% respond to steroids within days to weeks; complete recovery in 65-80%; relapse rate 30-50% during taper; mortality <5%; rarely progressive | J84.116 |
| 22 | 20 | Desquamative Interstitial Pneumonia (DIP) | Interstitial Lung Diseases | Strongly associated with smoking (>90%); accumulation of pigmented macrophages in alveoli; related to RB-ILD but more diffuse; rare non-smoking causes (CTD, drugs) | Very rare; 3% of ILD biopsies; M:F 2:1; almost exclusively smokers | 30-50 years; insidious onset over weeks to months | Gradually progressive dyspnea, persistent dry cough; clubbing (50%); bibasilar crackles; often less symptomatic than imaging suggests | Diffuse accumulation of macrophages within alveolar spaces; mild alveolar septal thickening; uniform temporal appearance (unlike UIP); preserved lung architecture | HRCT: diffuse ground-glass opacities, lower zone predominant; PFTs: restrictive with reduced DLCO; BAL: pigmented macrophages, smoker's macrophages; surgical biopsy for definitive diagnosis | Smoking cessation (primary treatment; improves 75%); corticosteroids (prednisone 40-60mg) for persistent/progressive disease; macrolides; supportive care | Good prognosis; 10-year survival 70-100%; smoking cessation alone leads to improvement in 75%; steroid response good; rarely progressive; recurrence if smoking resumes | J84.117 |
| 23 | 21 | Drug-Induced Interstitial Lung Disease | Interstitial Lung Diseases | Medication toxicity: methotrexate, amiodarone, nitrofurantoin, bleomycin, checkpoint inhibitors, biologics; mechanisms include direct toxicity, immune-mediated, oxidative stress | Variable by drug; amiodarone ILD: 5-7%; bleomycin pulmonary toxicity: 6-10%; checkpoint inhibitor pneumonitis: 3-5%; methotrexate pneumonitis: 1-2% | Any age depending on drug; onset days (acute) to years (chronic) after exposure | Dyspnea, dry cough, fever; pattern depends on drug: amiodarone (insidious), bleomycin (dose-related), checkpoint inhibitors (can be fulminant); may mimic infection | Various patterns: NSIP, OP, DAD, eosinophilic pneumonia, pulmonary fibrosis; depends on drug and mechanism | Temporal association with drug exposure; exclusion of infection and disease progression; HRCT pattern recognition; BAL to exclude infection; rarely biopsy needed; improvement after drug withdrawal supportive | Discontinue offending drug; corticosteroids (prednisone 1mg/kg) for moderate-severe; high-dose methylprednisolone for fulminant; supportive care; monitor for improvement over weeks | Highly variable; drug withdrawal often leads to improvement; amiodarone toxicity slower to resolve (long half-life); bleomycin fibrosis may be irreversible; checkpoint inhibitor pneumonitis: grade 3-4 mortality 10-15% | J70.4 |
| 24 | 22 | Hypersensitivity Pneumonitis (Chronic) | Interstitial Lung Diseases | Chronic exposure to inhaled organic antigens; bird proteins (pigeon breeder's lung), molds (farmer's lung), bacteria; combined type III and IV hypersensitivity; progresses to fibrosis | Varies by exposure; 0.3-0.9 per 100,000 in general population; 5-15% of exposed farmers; bird fanciers 6-20% | 40-65 years for chronic form; insidious onset over months to years of continued exposure | Progressive dyspnea, chronic cough, fatigue, weight loss; differs from acute HP (no fever/chills); bibasilar crackles; clubbing in advanced fibrotic disease | Upper/mid-lung predominant fibrosis; granulomatous interstitial pneumonia; airway-centered fibrosis; chronic antigen exposure drives progressive fibrosis | HRCT: mosaic attenuation, ground-glass, fibrosis (upper/mid lung); BAL lymphocytosis >30%; specific IgG precipitins to suspected antigen; exposure history critical; biopsy shows granulomas and fibrosis | Complete antigen avoidance (most important); prednisone 40-60mg for symptomatic; mycophenolate or azathioprine long-term; nintedanib for progressive fibrotic phenotype; lung transplant for advanced | Fibrotic chronic HP: 5-year survival 70-85%; worse if continued exposure; fibrotic phenotype similar to IPF prognosis; early diagnosis and antigen avoidance improve outcome significantly | J67.9 |
| 25 | 23 | Idiopathic Pulmonary Fibrosis (IPF) | Interstitial Lung Diseases | Unknown etiology; UIP pattern; risk factors: smoking, GERD, viral infections (EBV, HHV), genetic variants (TERT, TERC, MUC5B); aberrant wound healing in alveolar epithelium | 3-9 per 100,000; incidence increasing; M:F 1.5:1; predominantly in older adults | >50 years (median 66 years); insidious onset over months to years | Progressive exertional dyspnea, chronic dry cough (75%), bibasilar velcro crackles (>90%), digital clubbing (25-50%), late-stage cor pulmonale and respiratory failure | Usual interstitial pneumonia (UIP) pattern; peripheral, basal predominant fibrosis; honeycombing; fibroblast foci; temporal heterogeneity | HRCT showing UIP pattern (honeycombing, traction bronchiectasis, basal predominant); PFTs: restrictive with reduced DLCO; surgical lung biopsy if uncertain; ANA/RF to exclude CTD-ILD; BAL rarely needed | Antifibrotics: nintedanib 150mg BID or pirfenidone 801mg TID (slow decline by ~50%); pulmonary rehab; supplemental O2; lung transplant referral (only cure); palliative care for advanced disease | Poor prognosis; median survival 3-5 years from diagnosis; annual FVC decline 150-200ml; acute exacerbations carry 50% mortality; lung transplant median survival 4.5 years | J84.112 |
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