Idiopathic Pulmonary Fibrosis (IPF; Cryptogenic Fibrosing Alveolitis, Usual Interstitial Pneumonitis)


Familial Idiopathic Pulmonary Fibrosis


MUC5B Promoter Polymorphism (rs35705950)


Pathologic patterns (vasculitis, granulomas, signs of pneumoconioses suggest diagnoses other than IPF/inflammatory or obliterative airway narrowing, obstructive pneumonitis are usually mild-moderate and are seen in <25% of cases):
1) Organizing pneumonia:
2) Usual interstitial pneumonitis (UIP): probably represents intermediate stage of IPF between DIP and honecombing
-Broadened alveolar inter-stitium with fibrosis and fibroblastic proliferation/ type II pneumocyte hyper-plasia
3) Desquamative interstitial pneumonia (DIP): probably is earliest stage of IPF
-Alveolitis: alveoli are filled with alveolar macro-phages (previously thought to be sloughed epithelial cells) and lymphocytes/intact alveolar walls/probably represents the early cellular phase of UIP (and can occur in many lung diseases)/ more steroid-responsive
4) Giant Cell Interstitial Pneumonitis: variant of DIP with multinucleated giant cells also in alveoli (may be idiopathic or associated with hard metal pneumoconiosis)
5) Cellular interstitial pneumonia (CIP):
6) Honeycombing: end-stage, irre-versible IPF
-Disruption of lung architecture with cysts lined by metaplastic cuboidal epi-thelium (has potential for malignant tranformation into adeno or alveolar cell carcinoma) and filled with mucous-containing acute inflammatory cells/ proliferation of interstitial smooth muscle/ intimal smooth muscle proliferation and medial hypertrophy in vessels (indicate long-standing hypoxic pulmonary vasoconstriction)


Complete Blood Count (CBC) (see Complete Blood Count, [[Complete Blood Count]])

Erythrocyte Sedimentation Rate (ESR) (see Erythrocyte Sedimentation Rate, [[Erythrocyte Sedimentation Rate]])

Arterial Blood Gas (ABG) (see Arterial Blood Gas, [[Arterial Blood Gas]])

CXR/Chest CT Patterns (see Chest X-Ray, [[Chest X-Ray]] and Chest Computed Tomography, [[Chest Computed Tomography]])

High-Resolution Chest CT (HRCT) (see High-Resolution Chest Computed Tomography, [[High-Resolution Chest Computed Tomography]])

The features that best differentiated NSIP were relative subpleural sparing, absence of lobular areas with decreased attenuation, and lack of honeycombing
The features that best differentiated IPF were basal predominance of honeycombing, absence of relative subpleural sparing, and absence centrilobular nodules

Gallium Scan (see Gallium Scan, [[Gallium Scan]])

Ventilation/Perfusion (V/Q) Scan (see Ventilation-Perfusion Scan, [[Ventilation-Perfusion Scan]])

6-Minute Walk Test (see 6-Minute Walk Test, [[6-Minute Walk Test]])

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests, [[Pulmonary Function Tests]])

Bronchoscopy (see Bronchoscopy, [[Bronchoscopy]])

Thoracoscopic/Open Lung Biopsy

Other Testing

Clinical Manifestations

General Comments

Pulmonary Manifestations

Rheumatologic Manifestations

Other Manifestations




General Measures

Pirfenidone (Esbriet) (see Pirfenidone, [[Pirfenidone]])

Nintedanib (Ofev) (see Nintedanib, [[Nintedanib]])

Treatments with No Demonstrated Clinical Benefit

Azathioprine (Imuran) (see Azathioprine, [[Azathioprine]])

Bosentan (see Bosentan, [[Bosentan]])

Chlorambucil (see Chlorambucil, [[Chlorambucil]])

Colchicine (see Colchicine, [[Colchicine]])

Corticosteroids (see Corticosteroids, [[Corticosteroids]])

Coumadin (see Coumadin, [[Coumadin]])

Cyclophosphamide (Cytoxan) (see Cyclophosphamide, [[Cyclophosphamide]])

Etanercept (Enbrel) (see Anti-TNF Therapy, [[Anti-TNF Therapy]])

Imatinib (Gleevec) (see xxxx, [[xxxx]])

Interferon Gamma-1b (Actimune) (see Interferon Gamma-1b, [[Interferon Gamma-1b]])

N-Acetylcysteine (Mucomyst) (see N-Acetylcysteine, [[N-Acetylcysteine]])

Penicillamine (see Penicillamine, [[Penicillamine]])

Vincristine (see Vincristine, [[Vincristine]])

Experimental Treatments

Thalidomide (see Thalidomide, [[Thalidomide]])

Lung Transplant (see Lung Transplant, [[Lung Transplant]])