Presentation on "Diagnosis of ILD" | Jindal Chest Clinic

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Diagnosis of ILD
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Diagnosis of ILDs Three-step approach

• Objective Examples
  Tests required
• 1. Establishing the LVF, COPD,
  Clinical CXR,
• ILD diagnosis TB, others
  ECG, PFT, sputum
• ECHO,CT
• 2. Finding the cause
• -Secondary CTDs, Sarcoidosis, HRCT,
  PFT, BAL
• 
  pneumoconioses, TBLB, Others
• Drugs, Infections
• -Primary (IIP)
• 3. Type of IIP UIP, NSIP, COP HRCT, LB
• DIP, AIP, LIP,
• RBILD

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Evaluation of suspected IIP

• History, Exam., PFT, CXR
• Suspected ILD
• HRCT
• (Septal thickening, traction
• Yes bronchiectasis,
  honey combing)
• 
  No
• UIP/NSIP
  Secondary causes Yes
  (CTDs
  etc.)
• Secondary causes (CTDs)
  No Non-diagnostic Bl tests, TBLB
• Surgical Lung biopsy Diagnostic
  
• No Yes.
  
  Treat appropriately
• (Negative tests) Treat appropriately
  
• IPF
  Diagnostic Non-diagnostic
• 
  Treat as required Multidisciplinary
  review

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Diagnosing ILD

• Importance of clinical history in differential
  diagnosis

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Clinical algorithm
Raghu G. Am J Respir Crit Care Med 1995 151909
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Clinical algorithm
Raghu G. Am J Respir Crit Care Med 1995 151909
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Age
King, TE, Jr. Pulmonary and Respiratory Therapy
Secrets, Philadelphia, Handley Belfus, Inc. 1997.
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Gender

• Women - Lymphangioleiomyomatosis and pulmonary
  involvement in tuberous sclerosis in
  premenopausal women
• Lymphocytic interstitial pneumonitis, ILD in
  Hermansky-Pudlak syndrome, and the connective
  tissue diseases have less pronounced female
  preponderance
• Men - Rheumatoid arthritis associated ILDs, IPF

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H/O onset of symptoms
Acute (days to weeks)
Acute idiopathic interstitial pneumonia (AIP, Hamman-Rich syndrome)
Eosinophilic pneumonia
Hypersensitivity pneumonitis
Cryptogenic organizing pneumonia
Subacute (weeks to months)
Sarcoidosis
Some drug-induced ILDs
Alveolar hemorrhage syndromes
Cryptogenic organizing pneumonia
Connective tissue disease (systemic lupus erythematosus or polymyositis)
Chronic (months to years)
Idiopathic pulmonary fibrosis
Sarcoidosis
Pulmonary Langerhans cell histiocytosis
Chronic hypersensitivity pneumonitis
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Smoking history

• Current or former smokers
• Pulmonary Langerhans cell histiocytosis\
• Desquamative interstitial pneumonitis
• Respiratory bronchiolitis-interstitial lung
  disease
• Idiopathic pulmonary fibrosis)
• Never or former smokers
• Sarcoidosis
• Hypersensitivity pneumonitis

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Family history 

• IPF and NSIP may occur within a single family
• Autosomal dominant pattern of inheritance in IPF,
  tuberous sclerosis, and neurofibromatosis 
• Autosomal recessive pattern of inheritance in
  Niemann-Pick disease, Gaucher's disease, and
  Hermansky-Pudlak syndrome 

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H/O prior medication
List of drugs reported to cause pulmonary
toxicity is available at http//www.pneumotox.com
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Exposure history

• Inhaled inorganic dust e.g. silicosis,
  asbestosis, berylliosis, coal worker's
  pneumoconiosis and siderosis
• Inhaled organic dusts (hypersensitivity
  pneumonitis), thermophilic fungi, bacteria and
  animal proteins (eg, bird fancier's disease)

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Extrapulmonary Symptoms in History indicating
Specific Diagnosis
Recurrent oral ulcerations
Lupus, overlap syndromes
Ocular or oral dryness
Sjogrens syndrome, overlap syndromes
Facial or skin tightness, nail bed changes
Systemic Sclerosis
Rashes, photosensitivity
SLE, SSc, Overlap
Small joint arthritis
RA, MCTD
Arthralgias
SLE, SSc, Overlap, Sarcoidosis
Skin thickening over hands, forehead
Dermatomyositis, SSc, Overlap
Myalgias/ proximal muscle weakness
Dermatomyositis, Polymyositis
Facial lesions like angiofibroma
LAM (TS), BHD
Renal involvement
Pulmonary Renal Syndromes SLE
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Investigations to differentiate cause of ILD
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Laboratory investigations
Laboratory tests to order in the majority of patients with interstitial lung disease
Complete blood count and differential
Urinalysis
Alkaline phosphatase
Alanine aminotransferase (ALT, SGPT) and aspartate aminotransferase (AST, SGOT)
Blood urea nitrogen (BUN)
Creatinine
Tests for possible rheumatic disease Antinuclear antibody (ANA) Rheumatoid factor (RF)
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Laboratory investigations
Laboratory tests to order in selected patients with interstitial lung disease
Additional possible tests for systemic rheumatic disease Anti-cyclic citrullinated peptide (Anti-CCP) Creatine kinase (CK), aldolase Anti-Jo-1 antibody Anti-neutrophil cytoplasmic antibody (ANCA) Anti-topoisomerase (Scl-70) antibody, anti-PM-1 (PM-Scl) antibody Anti-double stranded (ds) DNA antibodies
Sicca features or positive anti-extractable nuclear antigen (ENA) Check anti-RO (SS-A), anti-La (SS-B), anti-ribonucleoprotein (RNP), serum protein electrophoresis, serum IgG4
Sclerodactyly, prominent GERD Check anti-centromere, anti-topoisomerase I (anti-Scl-70)
Mechanics hands Antisynthetase antibodies (in addition to anti-Jo-1)
Suspicion of heart failure or pulmonary hypertension Brain natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
Anemia and/or hemoptysis Coagulation studies, anti-glomerular basement membrane (GBM) antibodies, antiphospholipid antibodies, serum IgA endomysial or tissue transglutaminase antibodies in patients who may have idiopathic pulmonary hemosiderosis
Mediastinal lymphadenopathy serum protein electrophoresis
Beryllium exposure Peripheral blood beryllium lymphocyte proliferation test
Risk factors for HIV HIV test
Testing for hypersensitivity pneumonitis antibodies based on patient exposures
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Investigations to screen for hypersensitivity
pneumonitis

• The utility of screening panels for
  hypersensitivity pneumonitis is unclear due to
  problems with specificity
• One can reserve serologic testing for HP for
  patients with a historical risk factor (eg,
  occupational or environmental exposures) or
  features that are atypical for IPF (eg, younger
  age, centrilobular nodules on HRCT imaging).

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Importance of Pulmonary function tests
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Pulmonary function testing

• Spirometry, lung volumes, diffusing capacity and
  resting and exercise pulse oximetry should be
  obtained in virtually all patients with suspected
  interstitial lung disease
• Lung function is most helpful for assessing the
  severity of lung involvement and the pattern,
  whether obstructive, restrictive, or mixed.
• The pattern is useful in narrowing the number of
  possible diagnoses

Bradley B et al. Thorax. 2008 
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Spirometry and lung volumes

• Most of the interstitial disorders have a
  restrictive defect with reductions in total lung
  capacity (TLC), functional residual capacity
  (FRC), and residual volume (RV)
• FEV1/FVC ratio is usually normal or increased

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Spirometry and lung volumes

• Interstitial pattern on chest radiograph
  accompanied by obstructive airflow limitation is
  suggestive of any of the following
• Sarcoidosis
• Lymphangioleiomyomatosis
• Hypersensitivity pneumonitis
• Pulmonary Langerhans cell histiocytosis
• Tuberous sclerosis and pulmonary
  lymphangioleiomyomatosis
• Combined chronic obstructive pulmonary disease
  (COPD) and ILD
• Constrictive bronchiolitis

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Diffusing capacity

• A reduction in DLCO is a common, but nonspecific
  finding in ILD
• Moderate to severe reduction of DLCO in the
  presence of normal lung volumes suggests
• Combined emphysema and ILD
• Combined ILD and pulmonary vascular disease
• Pulmonary Langerhans cell histiocytosis
• Pulmonary lymphangioleiomyomatosis

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Diffusing capacity

• The severity of the DLCO reduction
  does not correlate well with disease prognosis,
  unless the DLCO is less than 35 percent of
  predicted.
• Longitudinal changes in DLCO have been used to
  assess disease progression or regression.
• Due to difficulties with reproducibility in
  measuring DLCO, a change of 15 percent is needed
  to identify a true change in disease severity

Latsi PI et al. Am J Respir Crit Care Med.
2003 Bradley B et al. Thorax. 2008
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Gas exchange at rest and on exertion

• Resting arterial blood gases
• Exercise testing 
• Cardiopulmonary exercise testing
• Six-minute walk test (6MWT)

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Investigations to exclude other disease in D/D
Bronchiectasis, COPD, CHF, PTE (as suspected)
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Cardiac evaluation

• ECG to evaluate cardiac disease
• Serum brain natriuretic peptide or
  N-terminal-proBNP levels if heart failure
• Echocardiography in patients with an abnormal
  electrocardiogram, suspected heart failure, rapid
  onset of radiographic findings, or a moderate to
  severe reduction in diffusing capacity (DLCO)

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Pulmonary hypertension

• ECG
• Serum brain natriuretic peptide or
  N-terminal-proBNP
• Doppler echocardiography
• Right heart catheterization in patients with
  normal echocardiogram but high clinical suspicion
  for pulmonary hypertension 

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