Interstitial lung disease

Dr CHHORN Sotheary
Pulmonologist
Deputy director of Techo Santepheap national hospital
 What we will learn
• Definition
• Etiology
• Pathophysiology
• Clinical manifestation
• Diagnostic evaluation
• Management
 Definition

Is a diffuse parenchymal lung disease with progressive scarring of the

lung, generally irreversible
Etiology
Commonly used drugs leading to ILD
- NSAID
- Nitrofurantoin
- Phenytoin sodium
- Carbamazepine
- Antiarrhythmic drugs
- Hydralazine
Etiology
- D-Penicillamine
- Amiodarone
- Cyclophosphamide, Methotrexate

Infectious: Tuberculosis ++,

viral +, bacterial, ..
 Etiology
Acute Subacute to chronic
- Connective tissue disease associated ILD
- Cryptogenic organizing pneumonia
- Idiopathic pulmonary fibrosis (IPF)
(COP)
- Sarcoidosis
- Acute eosinophilic pneumonia - Chronic hypersensitivity pneumonitis (CHP)
(AEP) - Occupational lung disease
- Acute hypersensitivity pneumonitis - Nonspecific interstitial pneumonia (NSIP)
- Diffuse alveolar hemorrhage - Desquamative interstitial pneumonitis (DIP)
- Respiratory bronchiolitis interstitial lung
- Acute interstitial pneumonia (AIP) disease (RB-ILD)
- Acute exacerbation of idiopathic - Lymphocytic interstitial pneumonia (LIP)
pulmonary fibrosis or other ILDs - Chronic eosinophilic pneumonia
Pathophysiology
 Clinical manifestation

- Nonspecific, normally progressive

- > 60 years
- Dyspnea on exertion or cough
- Clubbing of fingers
- Connective tissue disease clinical signs
- Night sweats, fever, fatigue or weight loss suggest an underlying inflammatory
condition
- Gastrointestinal symptoms: esophageal motility problems (sclerosis, polymyositis),
chronic intermittent aspiration (lung fibrosis), bloating and diarrhea…
- Musculoskeletal symptoms: arthralgias/morning stiffness/joint swelling/ erythema
(connective tissue disease), swollen fingers (sclerosis, polymyositis), Raynaud’s
phenomenon (scleroderma, SLE and antisynthetase syndrome) …
- Ophthalmologic symptoms: dry eyes (Sjogren syndrome), uveitis (SLE, sarcoidosis)
- Edema, syncopal events, exertional chest discomfort
- Pleuritic chest pain
 Investigation and diagnosis
1. History taking
- Occupational history: inorganic exposure, organic exposure
- Medication history: Nitrofurantoin, Amiodarone, NSAIDs,
chemotherapy, immune-modulating drug use
- Connective tissue disease finding
- HIV, Lymphocytic interstitial pneumonia (LIP) are common.
2. Blood test
- Standard test, kidney disease, liver disease
- Serology, antibody, auto-antibody…
3. Imageries finding:
- X-ray, CT scan
 Laboratory testing

- Elevated liver enzymes or hypercalcemia

in sarcoidosis
- Serologies and antibody
- Renal insufficiency or pulmonary-renal
syndromes
- Peripheral eosinophilia (eosinophilic
pneumonia, Churg-strauss syndrome,
drug reaction).
 Radiologic of ILD
Upper lung zone Lower lung zone

Sarcoidosis Usual interstitial pneumonia (UIP/IPF)

Silicosis Nonspecific interstitial pneumonia

Coal worker’s pneumoconiosis Connective tissue disease associated ILD

Hypersensitivity pneumonitis Asbestosis

Langerhan cell histiocytosis Desquamative interstitial pneumonia (DIP)

Berylliosis
Chronic eosinophilic pneumonia
 Pattern of ILD
Nodular
Peripheral reticular
- Sarcoidosis
- Idiopathic pulmonary fibrosis
- Berylliosis
- Non specific interstitial pneumonia
- Hypersensitivity pneumonitis
Ground glass - Langerhans cell histiocytosis
- NSIP - Silicosis, Talcosis
- Cryptogenic organizing pneumonia - Metastasis
- Eosinophilic pneumonia - Granulomatous polyangiitis (Wegener’s granulomatosis)
- Infection (opportunistic or viral) - Bronchiolitis
- Alveolar hemorrhage Cystic
- Hypersensitivity pneumonitis - Laymphangioleiomyomatosis
- Desquamative interstitial pneumonia - Langerhan cell histiocytosis

- Sarcoidosis - Lymphocytic interstitial pneumonia

- Pulmonary alveolar proteinosis - PCP

CT scan
- Ground glass
- Reticular infiltrates
- Reticulonodular shadowing
- Honeycombing
- Cystic
- Patent of bronchioles
- Traction bronchiectasis
- Consolidation
RETICULAR INFILTRATES

HONEYCOMB LUNG
 Other procedures

Pulmonary function tests

Oximetry
Arterial blood gas
Bronchoscopy: BAL, biopsy
Mediastinoscopy
Lung biopsy by transthoracic,
video-assisted thoracoscopic
surgery
 Treatment
• Removal from exposures
• Stop smoking
• Immunosuppressive therapy: steroids, azathioprine, cyclophosphamide
• Antifibrotic drugs in progressive fibrotic lung diseases: Pirfenidone/Nintedanib
• Oxygen therapy
• Physiotherapy /rehabilitation
• Comorbidities
• Emotional support
• Nutrition support
• Palliative care
• Vaccination
• Lung transplantation
Wells AU et al. Lancet Respir Med 2020;8:453–60
 Complication
• Pulmonary hypertension
• Right sided heart failure
• Respiratory failure
• Kidney failure
• Muscle paralysis
 Case report
A 37-year-old female patient
- known complaints of Scleroderma associated with ILD, Cor pulmonale, and
hypothyroidism
Chief complain: She was presented to the hospital via OPD with complaints of SOB
Grade-IV and orthopnea, which was not accompanied by wheeze.
History of disease: she has cough and pain in the fingers of both hands, one year
back. Over the last 10 days, the patient developed breathlessness. Gradually patient
complained of loss of wrinkling over the face and limitation of movement at finger
joints due to skin tightening, accompanied by pain in the digits.
Medical history:
- The patient's drug history, allergy history, family history, and psychosocial history
were not significant.
Physical examination
- On inspection: diffuse skin pigmentation, with particularly salt and
pepper pigmentation found in the face and neck region, impending
digital gangrene of, the index fingers bilaterally and an ulcer noted on
the right foot.
Manifestation paraclinical
- The 2D echocardiography showed pulmonary hypertension with a right
atrium and ventricular dilation accompanied by severe tricuspid
regurgitation.
- Abdominal ultrasound: Hepatomegaly
- High Resolution Computed Tomography (HRCT) of the chest: decreased
volume of both lungs with diffuse ground attenuation and traction
bronchiectasis and bronchiectasis in bilateral lung fields. subpleural
interlobular and intralobular septal thickening with increased reticulation
was found, along with focal areas of early honeycombing
ILD-fibrotic non-specific interstitial pneumonia (NSIP) pattern.
Diagnosis
 Diffuse scleroderma with ILD-exacerbation.
Case report 2
A 65-year-old woman with a 35-year history of limited cutaneous SSc was admitted to our hospital for
progressive dyspnoea on exertion with onset at 3 months before admission.
Physical examination revealed
- scleroderma in both arms and legs, with puffy fingers,
- Raynaud phenomenon and ankyloglossia.
- Lung auscultation revealed fine crackles in both lung bases.
Laboratory test
- high levels of Krebs von den Lungen-6 (1223 U/mL),
- surfactant protein D (128 ng/mL),
- brain natriuretic peptide (BNP, 39.5 U/mL),
- antinuclear antibody titre (320-fold) and antiribonucleoprotein antibody titre (64-fold).
- Arterial blood gas analysis showed a pH of 7.41, arterial carbon dioxide tension of 41.5 mm Hg and
arterial oxygen tension of 80.6 mm Hg at room air.
The pulmonary function test
- restrictive impairment (forced vital capacity
(FVC) of 1.81 L and 77.7% of predicted) with
decreased diffusion capacity for carbon monoxide
(8.78 mL/min/mm Hg and 49.4% of predicted).
Chest high-resolution CT (HRCT) scan:
- Diffuse reticular and widespread ground-glass
opacity (GGO) shadows and interlobular septal
Diffuse reticular and widespread GGO shadows and interlobular
thickening in both middle and lower lobes, septal thickening in both middle and lower lobes, without
without honeycombing honeycombing, were evident on the chest CT scan. (A)
The lung biopsy specimens of the left lung Transverse section on chest HRCT (left lower lobe). (B) Coronal
segments, S8 and S10, obtained by video-assistedimages of chest CT. GGO, ground-glass opacity; HRCT, high-
thoracic surgery (VATS), resolution CT.
fibrocellular non-specific IP (NSIP)
accompanied by accumulation of lymph follicle
formation.
The patient was diagnosed with fibrocellular
NSIP associated with SSc.
Care your lung care your
breath