Interstitial lung-disease

Interstitial lung-disease

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  INTERSTITIAL LUNGINTERSTITIAL LUNG DISEASESDISEASES Dr.QURA_TUL_AIN
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  CONTENTS DEFINITION CLASSIFICATIN SYMPTOMS & SIGNS COMPLICATIONS INVESTIGATIONS MANAGEMENT
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  DEFINITION The term interstitiallung 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.
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  CLASSIFICATION
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  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.
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  SIGNS PULMONARY SIGNS With advanceddisease, patients may have tachypnea and tachycardia, even at rest.  Bilateral, basilar, Velcro-like rales  Signs of pulmonary hypertension
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  EXTRAPULMONARY SIGNS  Clubbing(e.g. IPF)  Skin abnormalities, peripheral lymphadenopathy, hepatosplenomegaly (SARCOIDOSIS)  Subcutaneous nodules (RHEUMATOID ARTHRITIS)  Muscle tenderness and proximal weakness (POLYMYOSITIS)
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  D/D BASED ONONSET: 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
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  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
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  HIGH-RESOLUTION CT SCAN
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  INVESYIGATIONS PULMONARY FUNCTION TESTS ARTERIALBLOOD GAS ANALYSIS BRONCHOSCOPIC STUDIES  LUNG BIOPSY
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  MANEGEMENT PRINCIPAL AIMS: (1) toremove exposure to injurious agents, (2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma (3) to palliate the manifestations of these diseases.
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  MANEGEMENT General – Oxygen therapy •Forpatients with documented hypoxia – SpO2 < 89% – PaO2 < 55 mmHg •Improves exercise tolerance
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  CORTICOSTERIODS  Prednisone, 1mg/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
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  IMMUNO SUPRISSIVE AGENTS Cytotoxicagents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
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  Pirfenidone (antifibrotic) –Reduces acuteexacerbations 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