Interstitial Lung Disease (ILD)

Etiology

Infection

Viral

Bacterial

  • Aspiration Pneumonia (see Aspiration Pneumonia, [[Aspiration Pneumonia]])
  • Chlamydia Trachomatis (see Chlamydia Trachomatis, [[Chlamydia Trachomatis]])
  • Legionellosis (see Legionellosis, [[Legionellosis]])
    • Patchy Lobar Infiltrate, Which Progresses to Consolidation: most common pattern
    • Diffuse Interstitial Infiltrates: this pattern has also been reported
  • Miliary Tuberculosis (Disseminated Tuberculosis) (see Tuberculosis, [[Tuberculosis]])
  • Mycobacterium Kansasii (see Mycobacterium Kansasii, [[Mycobacterium Kansasii]])
  • Nocardiosis (see Nocardiosis, [[Nocardiosis]])
  • Post-Infectious Interstitial Lung Disease
  • Salmonella (see Salmonella, [[Salmonella]])
  • Staphylococcus Aureus (see Staphylococcus Aureus, [[Staphylococcus Aureus]])

Fungal

Parasitic

Neoplasm

Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]])

  • Vegetable Products
  • Animal Products
  • Insect products
  • Reactive Simple Chemicals
  • Drugs

Pneumoconiosis

  • Aluminum Dust Pneumoconiosis (see Aluminum, [[Aluminum]])
  • Asbestosis (see Asbestos, [[Asbestos]])
  • Berylliosis (see Beryllium, [[Beryllium]])
  • Coal Worker’s Pneumoconiosis (see Coal Workers Pneumoconiosis, [[Coal Workers Pneumoconiosis]])
  • Cobalt Pneumoconiosis (see Cobalt, [[Cobalt]]): combination of tungsten carbide + cobalt -> known as Hard Metal Pneumoconiosis
  • Nylon Flock Worker’s Lung (see Nylon Flock Workers Lung, [[Nylon Flock Workers Lung]])
  • Siderosis (see Siderosis, [[Siderosis]])
  • Silicosis (see Silicosis, [[Silicosis]])
  • Stannosis (see Stannosis, [[Stannosis]])
  • Talc Pneumoconiosis (see Talc, [[Talc]])
  • Titanium (see Titanium, [[Titanium]])

Rheumatologic, Connective Tissue, and Autoimmune Disease

Pulmonary Infiltrates with Eosinophilia (see Pulmonary Infiltrates with Eosinophilia, [[Pulmonary Infiltrates with Eosinophilia]])

  • Acute Lung Transplant Rejection (Acute Cellular Lung Transplant Rejection) (see Acute Lung Transplant Rejection, [[Acute Lung Transplant Rejection]]): peripheral eosinophilia may occur with/without pulmonary infiltrates (as acute rejection may be detected by surveillance bronchoscopy with transbronchial biopsy prior to the development of pulmonary infiltrates)
  • Chronic Eosinophilic Pneumonia (see Chronic Eosinophilic Pneumonia, [[Chronic Eosinophilic Pneumonia]])
  • Eosinophilia-Myalgia Syndrome (see Eosinophilia-Myalgia Syndrome, [[Eosinophilia-Myalgia Syndrome]])
  • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
  • Tropical Pulmonary Eosinophilia (Occult Filariasis) (see Tropical Pulmonary Eosinophilia, [[Tropical Pulmonary Eosinophilia (Occult Filariasis)]])

Unclassified Interstitial Lung Disease

Drug/Toxin


Diagnosis

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

  • Leukopenia (see Leukopenia, [[Leukopenia]])
    • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • Connective Tissue Disease
    • Lymphoma
    • Drug-induced ILD
  • Leukocytosis (see xxxx, [[xxxx]])
    • Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]])
    • Lymphoma
    • Drug-induced ILD
    • Systemic Vasculitis
  • Eosinophilia (see Peripheral Eosinophilia, [[Peripheral Eosinophilia]])
    • Pulmonary Infiltrates with Eosinophilia (PIE) (see xxxx, [[xxxx]])
  • Thrombocytopenia (see Thrombocytopenia, [[Thrombocytopenia]])
    • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • Connective Tissue Disease
    • Drug-induced Interstitial Lung Disease
    • Gaucher’s Disease
    • Idiopathic Pulmonary Fibrosis (IPF)
  • Normocytic Anemia (see Anemia, [[Anemia]])
    • Diffuse Alveolar Hemorrhage
    • Connective Tissue Disease
    • Pulmonary Metastases-Lymphangitic Carcinomatosis
  • Hemolytic Anemia (see Hemolytic Anemia, [[Hemolytic Anemia]])
    • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • Connective Tissue Disease
    • Lymphoma
    • Drug-Induced Interstitial Lung Disease
    • Idiopathic Pulmonary Fibrosis (IPF) (see xxxx, [[xxxx]])

Urinalysis (see Urinalysis, [[Urinalysis]])

  • Abnormal Urinary Sediment
    • Connective tissue disease
    • Systemic Vasculitis
    • Drug-Induced Interstitial Lung Disease

Immunoglobulin Levels

  • Hypogammaglobulinemia
    • Lymphocytic Interstitial Pneumonia (LIP) (see xxxx, [[xxxx]])
  • Hypergammaglobulinemia
    • *Lymphocytic Interstitial Pneumonia]]
    • Connective Tissue Disease
    • Systemic Vasculitis
    • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • Idiopathic Pulmonary Fibrosis]
    • Asbestosis
    • Silicosis
    • Lymphoma

Serum Angiotensin Converting Enzyme (ACE) Level (see Serum Angiotensin Converting Enzyme, [[Serum Angiotensin Converting Enzyme]])

  • Elevated Angiotensin Converting Enzyme (ACE) Level

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

General Radiographic Features

Normal Chest X-Ray (CXR) (see Chest X-Ray, [[Chest X-Ray]])

  • General Comments: normal CXR is seen in 10% of all ILD cases, but these usually have abnormal HRCT
  • Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]]): may be normal in some cases discovered in population studies, rarely normal in isolated chronic HP cases
  • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
  • Bronchiolitis Obliterans (BO) (see xxxx, [[xxxx]])
  • Connective Tissue Disease
  • Idiopathic Pulmonary Fibrosis (IPF): early in course
  • Asbestosis
  • Lymphangioleiomyomatosis (LAM) (see Lymphangioleiomyomatosis, [[Lymphangioleiomyomatosis]])

Upper Lung Zone-Predominance

Alveolar Filling Pattern

  • [[Bronchioloalveolar Cell Carcinoma]]: alveoli filled with malignant cells and mucinous material
  • [[Diffuse Alveolar Hemorrhage]]: alveoli filled with blood or hemosiderin-laden macrophages
  • [[Acute Eosinophilic Pneumonia]]:
  • [[Chronic Eosinophilic Pneumonia]]
  • [[Cryptogenic Organizing Pneumonia]]
  • [[Pulmonary Alveolar Proteinosis]]: alveoli filled with proteinaceous material
  • [[Lymphoma]]: alveoli filled with malignant lymphocytes
  • [[Lymphocytic Interstitial Pneumonia]]: alveoli filled with lymphoplasmacytic cells
  • [[Sarcoidosis]]: alveoli filled with granulomas or lymphycte-macrophage alveolitis
  • [[Desquamative Interstitial Pneumonia]]: alveoli filled with macrophages
  • [[Pulmonary Infiltrates with Eosinophilia]]: alveoli filled with eosinophils, macrophages, and lymphocytes
  • [[Pulmonary Alveolar Microlithiasis]]: alveoli filled with calcium-phosphate microliths
  • [[Cryptogenic Organizing Pneumonia]]: alveoli filled with collagen
  • [[Lipoid Pneumonia]]: alveoli filled with lipid-filled macrophages
  • [[Hypersensitivity Pneumonitis]]: alveoli filled with lymphoplasmacytic cells

Preserved or Increased Lung Volumes

  • Respiratory Bronchiolitis Interstitial Lung Disease
  • Langerhans Cell Histiocytosis (see Langerhans Cell Histiocytosis, [[Langerhans Cell Histiocytosis]])
  • Lymphangioleiomyomatosis (LAM) (see Lymphangioleiomyomatosis, [[Lymphangioleiomyomatosis]])
  • Tuberous Sclerosis
  • Sarcoidosis
  • Neurofibromatosis
  • Chronic Hypersensitivity Pneumonitis

Honeycombing

  • [[Idiopathic Pulmonary Fibrosis]]
  • Connective Tissue Disease-Associated ILD
  • [[Asbestosis]]
  • Drug-Induced ILD
  • [[Lymphocytic Interstitial Pneumonia]]
  • Chronic [[Aspiration Pneumonia]]
  • [[Idiopathic Pulmonary Hemosiderosis]]
  • [[Hermansky-Pudlak Syndrome]]
  • [[Pulmonary Alveolar Proteinosis]]

Pneumothorax

Pleural Involvement

  • [[Lung Metastases-Lymphangitic Carcinomatosis]]: pleural effusion
  • Connective Tissue Disease-Associated ILD ([[SLE]], [[Rheumatoid Arthritis]]): pleural effusion
  • [[Nitrofurantoin]]: pleural effusion
  • [[Sarcoidosis]]: pleural effusion
  • Radiation Pneumonitis (see [[Radiation Pneumonitis and Fibrosis]]): pleural effusion
  • [[Lymphangioleiomyomatosis]]: chylothorax
  • [[Asbestosis]]: pleural calcifications

Hilar/Mediastinal Adenopathy

  • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
  • [[Lymphoma]]
  • [[Lung Metastases-Lymphangitic Carcinomatosis]]
  • [[Lymphocytic Interstitial Pneumonia]]
  • [[Berylliosis]]
  • [[Amyloidosis]]
  • [Gauchers Disease]]

Eggshell Calcification of Lymph Nodes

Kerley B Lines

  • Lung Metastases-Lymphangitic Carcinomatosis
  • Lymphangioleiomyomatosis
  • Pulmonary Venous Hypertension
    • Mitral Stenosis
    • Pulmonary Veno-Occlusive Disease
  • Lymphoma
  • Amyloidosis

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

High-Resolution Chest CT Patterns

  • Normal HRCT
  • Interlobular Septal Thickening
    • Lymphangitic Spread
  • Crazy Paving Pattern: fine reticular pattern superimposed on ground glass opacification, due to thickening of intralobular interstitium -> creates geometric shapes) -> this pattern is fairly non-specific
    • PAP: classic association
    • UIP
    • Infection: PCP
    • Pulmonary edema
    • ARDS
    • BOOP
    • DAH
    • XRT
    • SARS
    • Bronchoalveolar Carcinoma
    • Sarcoidosis
    • Lipoid Pneumonia
  • Ground Glass
    • Mycoplasma: central pattern of ground-glass opacification and consolidation
    • Chlamydia: central pattern of ground-glass opacification and consolidation
    • Influenza: central pattern of ground-glass opacification and consolidation
    • SARS: peripheral, lower-lobe predominance
    • PCP: central ground-glass or mosaic pattern
    • AIP:
    • RB-ILD/DIP
    • NSIP
    • UIP/IPF
    • COP/BOOP
    • LIP
  • Centrilobular Nodules with Peripheral Tree-In-Bud: indicates bronchiolar disease

BRONCHIOLAR TREE IN BUD

BRONCHIOLAR CENTR NODULES

High-Resolution Chest CT Patterns by Disease (see High-Resolution Chest Computed Tomography, [[High-Resolution Chest Computed Tomography]])

  • HRCT in Mycoplasma/Chlamydia/Influenza: central pattern of ground-glass (due to airspace filling) and consolidation: may progress to centrilobular, acinar shadows and air-space consolidation with a lobular distribution
  • High-Resolution Chest CT in Hypersensitivity Pneumonitis (see xxxx, [[xxxx]])
    • Location of Infiltrates: predominantly in the middle lung zone, sparing the lower lobes
    • Ground-glass (due to airspace filling) with consolidation:
    • Alveolar inflammation:
    • Centrilobular nodules (due to bronchiolocentric distribution of disease): may be present
  • High-Resolution Chest CT in Chronic Eosinophilic Pneumonia (see xxxx, [[xxxx]])
    • Location of Infiltrates: predominantly in middle and upper lobes
    • Ground glass (due to airspace filling) with/without consolidation: consolidation present in 74% of cases
  • High-Resolution Chest CT in Cryptogenic Organizing Pneumonia (see xxxx, [[xxxx]])
    • Location of Infiltrates: predominantly in periphery, lower lobes
    • Ground glass (due to airspace filling) with/without consolidation: consolidation (usually patchy) present in 87% of cases
    • “Crazy Paving” Pattern: ground-glass with thickening of interlobular and intralobular septa, producing polygonal shapes
    • Bronchiectasis: may be seen
    • Nodules (32% of cases)”
    • Lymphadenopathy (13% of cases):
    • Pleural effusion (20% of cases):
  • High-Resolution Chest CT in SARS
    • Location of Infiltrates: predominantly in periphery, lower lobes (appears similar to [[Cryptogenic Organizing Pneumonia]])
    • Ground glass (due to airspace filling) with/without consolidation: common feature
    • “Crazy Paving” Pattern: ground-glass with thickening of interlobular and intralobular septa, producing polygonal shapes
    • Bronchiectasis: may be seen
  • High-Resolution Chest CT in PAP
    • “Crazy Paving” Pattern: ground-glass with thickening of interlobular and intralobular septa, producing polygonal shapes
    • Predominantly central alveolar opacities in mid-lower lung zones, sparing subpleural areas and areas adjacent to diaphragm and heart
    • Mosaic Pattern:
  • High-Resolution Chest CT in Acute Interstitial Pneumonia (AIP)
    • Patchy or Diffuse Ground Glass Infiltrates (Due to Airspace Filling)
  • High-Resolution Chest CT in DIP
    • Ground glass (Due to Airspace Filling) without Polygonal Lines
  • High-Resolution Chest CT in Lymphangitic Mets
    • Diffuse reticulonodular infiltrates, often with linear markings radiating out from enlarged hilar or mediastinal nodes
    • “Beaded chain” or “string of pearls” thickening of interlobular septa (mainly at periphery of lung and extending to the pleural surface) due to pulmonary lymphatic obstruction -> producing polygonal shapes
    • Intevervening parenchyma between the interlobular septa is typically normal (unlike in IPF, where it is usually distorted or honeycombed)
    • Presence of lymphangitic pattern in patient with known malignancy: 70-80% probability of metastases being etiologic
  • High-Resolution Chest CT in Interstitial Pulm Edema
    • Smoothly thickened interlobular septa -> producing polygonal shapes
  • High-Resolution Chest CT in Diffuse Alveolar Hemorhage (DAH)
    • Ground glass (due to airspace filling)

Bronchoscopy (see Bronchoscopy, [[Bronchoscopy]])

Bronchoalveolar Lavage (BAL)

ildhrctbal

Recommendations for Bronchoalveolar Lavage (BAL) in the Setting of Interstitial Lung Disease (Am J Respir Crit Care Med, 2012) [MEDLINE]
  • For Patients with Suspected Interstitial Lung Disease in Whom a Bronchoalveolar Lavage Can Be Tolerated, BAL Target Site Be Chosen on the Basis of an HRCT Performed Before the Procedure, Rather than Choosing a Traditional Bronchoalveolar Lavage Site (Such as the Right Middle Lobe or Lingula)
    • HRCT Should Be Performed within 6 wks of the BAL
  • For Patients with Suspected Interstitial Lung Disease Who Undergo Bronchoalveolar Lavage, a Differential Cell Count Should Be Performed on the Bronchoalveolar Lavage Fluid
    • Including Lymphocyte, Neutrophil, Eosinophil, and Mast Cell Counts
    • Remaining Sample Should Be Used for Microbiological, Virological, and/or Malignant Cell Cytology Laboratory Testing, if Indicated
  • For Patients with Suspected Interstitial Lung Disease Who Undergo Bronchoalveolar Lavage, Lymphocyte Subset Analysis Should Not Be a Routine Component of Bronchoalveolar Lavage Cellular Analysis

Open Lung Biopsy-Pathologic Patterns

Granulomas (Arch Pathol Lab Med, 2010) [MEDLINE]

  • General Comments
    • Definition of Granuloma: compact aggregate of histiocytes (macrophages)
      • Histiocytes in Granulomas are Often Described as Epithelioid: epithelioid histiocytes have indistinct cell borders and elongated, sole-shaped nuclei (in contrast to the well-defined cell borders and round, oval, or kidney bean–shaped nuclei of ordinary histiocytes
      • Aggregation of Histiocytes is the Minimum Requirement for the Diagnosis of a Granuloma: regardless of whether the lesion also contains necrosis, lymphocytes, plasma cells, or multinucleated giant cells
  • Infection
  • Non-Infectious

Diffuse Alveolar Damage (DAD)

Organizing Pneumonia

  • Cryptogenic Organizing Pneumonia (see [[Cryptogenic Organizing Pneumonia]])
  • Organizing Stage of Diffuse Alveolar Damage
  • [[Diffuse Alveolar Hemorrhage]]
  • Drugs: [[Amiodarone]], Cocaine
  • Infections
  • Connective Tissue Disease
  • Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]])
  • Eosinophilic Pneumonia
  • [[Wegeners Granulomatosis]]

Desquamative Interstitial Pneumonia

  • Tobacco Abuse
  • Idiopathic Desquamative Interstitial Pneumonia
  • Connective Tissue Disease
  • Eosinophilic Granuloma
  • Asbestosis
  • Hard Metal Pneumoconiosis (Cobalt)
  • Gauchers Disease
  • Niemann-Pick Disease
  • Hermansky-Pudlak Syndrome
  • Drugs: amiodarone, nitrofurantoin

Non-Specific Interstitial Pneumonitis

  • Connective Tissue Disease
  • Hypersensitivity Pneumonitis (see [[Hypersensitivity Pneumonitis]])
  • Diffuse Alveolar Damage
  • Infection
  • Lymphocytic Interstitial Pneumonia
  • HIV Disease (see [[HIV Disease]])
  • Infection
  • Chronic Eosinophilic Pneumonia
  • Idiopathic Pulmonary Hemosiderosis
  • Pulmonary Alveolar Proteinosis
  • Idiopathic Non-Specific Interstitial Pneumonitis

Usual Interstitial Pneumonia

  • Connective Tissue Disease: uncommon
  • Asbestosis
  • Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]])
  • Chronic Aspiration Pneumonia
  • Radiation Pneumonitis
  • Hermansky-Pudlak Syndrome
  • Neurofibromatosis
  • Idiopathic Pulmonary Fibrosis (IPF) (see Idiopathic Pulmonary Fibrosis, [[Idiopathic Pulmonary Fibrosis]])

Lymphocytic Interstitial Pneumonia (LIP) (see Lymphocytic Interstitial Pneumonia, [[Lymphocytic Interstitial Pneumonia]])

  • Dysproteinemia
    • Hypogammaglobulinemia
    • Monoclonal or Polyclonal Gammopathy
  • Autoimmune
    • [[Sjogrens Syndrome]]
    • Chronic Active Hepatitis
    • [[Myasthenia Gravis]]
    • [[Primary Biliary Cirrhosis]]
    • [[Hashimotos Thyroiditis]]
    • [[Pernicious Anemia]]
    • Autoimmune [[Hemolytic Anemia]]
    • [[SLE]]
  • Allogeneic Bone Marrow Transplant
  • Infection
    • [[HIV Disease]]
    • EBV
    • HTLV-1
    • [[Tuberculosis]]
    • Following [[Legionellosis]]
  • Celiac Sprue
  • [[Dilantin]]
  • Surfactant Protein C Deficiency
  • Idiopathic Lymphocytic Interstitial Pneumonia

Eosinophilic Pneumonia

Alveolar Proteinosis

Amyloid Deposition

Diffuse Alveolar Hemorrhage (DAH)


Summary of Features of the Idiopathic Interstitial Pneumonias (American Thoracic Society/European Respiratory Society, 2013) (Am J Respir Crit Care Med, 2013) [MEDLINE]

idio-int-pneum


Clinical Manifestations

Historical Elements

  • Age
    • Peak age group for connective-tissue associated ILD/LAM/EG/Familial [[Idiopathic Pulmonary Fibrosis]]/Hermansky-Pudlak syndrome/Gaucher’s disease: between age 20-40 y/o
    • Peak age group for [[Sarcoidosis]]: 21-30 y/o (almost all cases occur in <60 y/o group)
    • Peak age group for [[Eosinophilic Granuloma]]: 20-40 y/o (almost all cases occur in <60 y/o group)
    • Peak age group for [[Idiopathic Pulmonary Fibrosis]]: 61-70 y/o (most cases are >50 y/o)
  • Gender
    • LAM/TS-associated ILD occur exclusively in premenopausal women
    • Female-predominance: LIP/Hermansky-Pudlak syndrome/connective tissue-associated ILD (except RA-associated ILD)
    • Male predominance: RA-associated ILD/pneumoconioses (due to increased occupational exposure in men)
  • Smoking History
    • Higher frequency in current or former smokers: EG/DIP/IPF/RB-ILD
    • Smoking increases risk of of DAH in Goodpasture’s (smoking exposes BM to Ab)
    • Higher frequency in former or never smokers: sarcoidosis/HP
  • Duration of Illness
    • Acute (days-wks): AIP/AEP/HP/BOOP
    • Subacute (wks-months): sarcoidosis/some drug-induced ILD/DAH/idiopathic BOOP/SLE or polymyositis-associated ILD
    • Chronic (months-yrs): IPF/sarcoidosis/EG
  • Prior Medication Use
    • See etiologies
  • Family History
    • Autosomal-dominant: IPF/sarcoidosis/TS/neurofibromatosis
    • Autosomal-recessive: Niemann-Pick/Gaucher’s/Hermansky-Pudlak
  • Occupational History
    • Especially relation of symptoms to presence in workplace
  • Environmental Exposures
    • Exposures to pets (especially birds)/air conditioners/humidifiers/hot tubs/evaporative cooling systems (swamp coolers)/areas of water damage
    • Familial passive exposure: can occur with asbestos or berylium dust from worker’s clothing
  • Spontaneous Pneumothorax (see xxxx, [[xxxx]])
    • Langerhans Cell Histiocytosis (see Langerhans Cell Histiocytosis, [[Langerhans Cell Histiocytosis]])
    • Lymphangioleiomyomatosis (LAM) (see xxxx, [[xxxx]])
    • Neurofibromatosis (see xxxx, [[xxxx]])
    • Tuberous Sclerosis (see xxxx, [[xxxx]])
  • Cough: usually dry
  • Hemoptysis
    • LAM
    • TS
    • Pulmonary VOD
    • DAH syndromes
    • Long-standing mitral valve disease
  • Wheezing: unusual
    • Sarcoidosis (see Sarcoidosis, [[Sarcoidosis]])
    • LAM
    • Langerhans Cell Histiocytosis (see Langerhans Cell Histiocytosis, [[Langerhans Cell Histiocytosis]])
    • Toluene Diisocyanate (TDI)
    • Lymphangitic Carcinomatosis
    • Chronic Eosinophilic Pneumonia
    • Churg-Strauss
    • RB-ILD
  • Chest Pain
    • Sarcoidosis (substernal pain is common)
    • SLE-associated ILD (pleuritic)
    • RA-associated ILD (pleuritic)
    • MCTD-associated ILD (pleuritic)
  • Symptoms Preceding Onset of a Connective Tissue Disease
    • ILD may precede other manifestations of disease by months-yrs in SLE/RA/polymyositis

Pulmonary Exam Elements

  • Velcro Crackles: common
  • Late Inspiratory Squeaks: frequent in bronchiolitis

Extrapulmonary Findings

  • Clubbing
    • [[Asbestosis]] (common)
    • Idiopathic Pulmonary Fibrosis (IPF) (see Idiopathic Pulmonary Fibrosis, [[Idiopathic Pulmonary Fibrosis]]): common
    • [[Sarcoidosis]] (rare)
    • Hypersensitivity Pneumonitis (HP) (see Hypersensitivity Pneumonitis, [[Hypersensitivity Pneumonitis]]): rare
    • [[Eosinophilic Granuloma]] (rare)
  • Hypertension
    • Connective tissue diseases
    • Some DAH syndromes
    • Neurofibromatosis
  • Pulmonary Hypertension
    • Scleroderma
  • Lymphadenopathy (see xxxx, [[xxxx]])
    • Sarcoidosis
    • Lymphocytic Interstitial Pneumonia (LIP) (see Lymphocytic Interstitial Pneumonia, [[Lymphocytic Interstitial Pneumonia]])
    • Lymphoma
    • Lymphangitic carcinomatosis
  • Hepatosplenomegaly
    • Sarcoidosis
    • EG
    • Connective tissue diseases
    • Amyloidosis
    • Lymphocytic Interstitial Pneumonia (LIP) (see Lymphocytic Interstitial Pneumonia, [[Lymphocytic Interstitial Pneumonia]])
  • Pericarditis (see xxxx, [[xxxx]])
    • Radiation pneumonitis
    • Connective tissue diseases
  • Myositis
    • Polymyositis
    • Connective tissue diseases
    • L-tryptophan
  • Muscle Weakness
    • Connective tissue diseases
  • Uveitis
    • Sarcoidosis
    • Ankylosing spondylitis
    • Behcet’s
  • Scleritis
    • SLE
    • Scleroderma
    • Sarcoidosis
    • Systemic vasculitides
  • Keratoconjunctivitis Sicca (Sjogren’s)
    • Lymphocytic Interstitial Pneumonia (LIP) (see Lymphocytic Interstitial Pneumonia, [[Lymphocytic Interstitial Pneumonia]])/Pseudolymphoma
    • Primary Pulmonary Lymphoma (MALT)
  • Rash-Erythema Nodosum
    • Sarcoidosis
    • Behcet’s
    • Connective tissue diseases
    • Histo
    • Cocci
  • Rash-Maculopapular
    • Drug-induced
    • Amyloidosis
    • Lipoidosis
    • Connective tissue diseases
    • Gaucher’s
  • Rash-Heliotrope
    • Dermatomyositis
  • Rash-Telangiectasia
    • Scleroderma
  • Rash-Cutaneous Vasculitis
    • Systemic vasculitides
    • Connective tissue diseases
  • Raynaud’s
  • Cafe-au-lait Spots
    • [[Neurofibromatosis]]
  • Subcutaneous Nodules
    • Neurofibromatosis
    • [[Rheumatoid Arthritis]]
  • Albinism
    • Hermansky-Pudlak
  • Calcinosis
    • Dermatomyositis
    • Scleroderma
  • Salivary Gland Enlargement

References

  • Granulomatous lung disease: an approach to the differential diagnosis. Arch Pathol Lab Med. 2010 May;134(5):667-90. doi: 10.1043/1543-2165-134.5.667 [MEDLINE]

  • An Official American Thoracic Society Clinical Practice Guideline: The Clinical Utility of Bronchoalveolar Lavage Cellular Analysis in Interstitial Lung Disease. Am J Respir Crit Care Med. 2012 May 1;185(9):1004-14. doi: 10.1164/rccm.201202-0320ST [MEDLINE]

  • An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48. doi: 10.1164/rccm.201308-1483ST [MEDLINE]