Interstitial lung-disease
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Transcript of Interstitial lung-disease
DEFINITIONThe 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.
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.
SIGNS
PULMONARY SIGNS
With advanced disease, patients may have
tachypnea and tachycardia, even at rest.
Bilateral, basilar, Velcro-like rales
Signs of pulmonary hypertension
EXTRAPULMONARY SIGNS
Clubbing (e.g. IPF) Skin abnormalities, peripheral lymphadenopathy, hepatosplenomegaly (SARCOIDOSIS) Subcutaneous nodules (RHEUMATOID
ARTHRITIS) Muscle tenderness and proximal weakness
(POLYMYOSITIS)
D/D BASED ON ONSET:
ACUTE ONSET: DAYS TO WEEKS Acute interstitial pneumonia Acute pneumonitis from collagen vascular
disease(especially SLE) Diffuse alveolar hemorrhage Hypersensitivity pneumonitis
CHRONIC: MONTHS TO YEARS Idiopathic pulmonary fibrosis Chronic hypersensitivity pneumonitis Collagen vascular disease–associated ILD
INVESTIGATIONSCHEST RADIOGRAPHY Nodules, linear (reticular) infiltrates, or a
combination of the two (reticulonodular infiltrates)
Diffuse ground glass pattern– EARLY Cystic areas (honeycomb pattern)-Late
INVESYIGATIONSPULMONARY FUNCTION TESTS
ARTERIAL BLOOD GAS ANALYSIS BRONCHOSCOPIC STUDIES
LUNG BIOPSY
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.
MANEGEMENT
General– Oxygen therapy
•For patients with documentedhypoxia
– SpO2< 89%– PaO2< 55 mmHg
•Improves exercise tolerance
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
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
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