calassification,symptomsand other details

2. INTERSTITIAL LUNGINTERSTITIAL LUNG
DISEASESDISEASES
Dr.QURA_TUL_AIN

3. CONTENTS
DEFINITION
CLASSIFICATIN
SYMPTOMS & SIGNS
COMPLICATIONS
INVESTIGATIONS
MANAGEMENT

4. DEFINITION
The term interstitial lung disease (ILD) refers to a
broad category of lung diseases rather than a specific
disease entity.1,2 It includes a variety of illnesses with
diverse causes, treatments, and prognoses. These
disorders are grouped together because of similarities in
their clinical presentations, plain chest radiographic
appearance, and physiologic features.

5. CLASSIFICATION

6. SYMPTOMS
Breathlessness (most common): Initially,
dyspnea on exertion→ later at rest
 Nonproductive cough
 Fatigue
 Pleuritic chest pain
 Hemoptysis-- infrequent
 A family history of ILDs should be
sought.

7. SIGNS
PULMONARY SIGNS
With advanced disease, patients may have
tachypnea and tachycardia, even at rest.
 Bilateral, basilar, Velcro-like rales
 Signs of pulmonary hypertension

8. EXTRAPULMONARY SIGNS
 Clubbing (e.g. IPF)
 Skin abnormalities, peripheral
lymphadenopathy, hepatosplenomegaly
(SARCOIDOSIS)
 Subcutaneous nodules (RHEUMATOID
ARTHRITIS)
 Muscle tenderness and proximal weakness
(POLYMYOSITIS)

9. D/D BASED ON ONSET:
ACUTE ONSET: DAYS TOWEEKS
 Acute interstitial pneumonia
 Acute pneumonitis from collagen vascular
disease(especially SLE)
 Diffuse alveolar hemorrhage
 Hypersensitivity pneumonitis
CHRONIC: MONTHS TOYEARS
 Idiopathic pulmonary fibrosis
 Chronic hypersensitivity pneumonitis
 Collagen vascular disease–associated ILD

10. INVESTIGATIONS
CHEST RADIOGRAPHY
Nodules, linear (reticular) infiltrates, or a
combination of the two (reticulonodular
infiltrates)
Diffuse ground glass pattern– EARLY
Cystic areas (honeycomb pattern)-Late

12. HIGH-RESOLUTION CT SCAN

13. INVESYIGATIONS
PULMONARY FUNCTION TESTS
ARTERIAL BLOOD GAS ANALYSIS
BRONCHOSCOPIC STUDIES
 LUNG BIOPSY

14. MANEGEMENT
PRINCIPAL AIMS:
(1) to remove exposure to injurious agents,
(2) to suppress inflammation to prevent further destruction
of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases.

15. MANEGEMENT
General
– Oxygen therapy
•For patients with documented
hypoxia
– SpO2 < 89%
– PaO2 < 55 mmHg
•Improves exercise tolerance

16. CORTICOSTERIODS
 Prednisone, 1 mg/kg for 1 month, followed by 40 mg/day
given for 2 months
 Gradually tapered (5 mg/week) over several months to a
maintenance dose of 15 to 20 mg/day
 Corticosteroids are continued until pulmonary function is
stable for 1 year
 Immunizations
(pneumococcal, influenza)
 Pulmonary Rehabitation
 Treatment of PHT

17. IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or
immunosuppressive agents (Azathioprine) may
be used in patients who do not improve on
steroid therapy or who cannot tolerate
corticosteroids

18. Pirfenidone (antifibrotic)
–Reduces acute exacerbations and reduction in FVC
N-acetyl cystein (antioxydant)
N Engl J Med 2005;353:2229-42
–NAC 600 mg PO tid added to prednisone and azathioprine, preserves
vital capacity and FVC and DLCO