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Questions and Answers
Which of the following is NOT a common cause of death in patients with interstitial lung diseases?
What is the most prevalent symptom in patients with Idiopathic Pulmonary Fibrosis (IPF)?
Which type of interstitial lung disease is more common in males than females?
What is a specific clinical feature indicative of Desquamative Interstitial Pneumonia (DIP)?
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Which interstitial lung disease has a specific association with smoking?
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What is a distinguishing characteristic of Nonspecific Interstitial Pneumonia (NSIP) compared to other interstitial lung diseases?
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In what proportion do male and female patients typically present with Usual Interstitial Pneumonia?
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What is the primary cause of death for patients with Idiopathic Pulmonary Fibrosis, besides respiratory failure?
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Which of the following clinical presentations is most likely in a patient with Eosinophilic Granuloma (histiocytosis X)?
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Which treatment is indicated for interstitial lung diseases with inflammatory changes shown on HRCT?
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What is the significant difference in histopathology between Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP)?
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Which interstitial lung disease is associated with a predilection for lytic bone lesions and pituitary involvement?
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What is a characteristic feature seen on HRCT in patients with Lymphangioleiomyomatosis?
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Which treatment strategy is recommended for Langerhans cell granulomatosis (Eosinophilic Granuloma)?
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What is a defining feature of Asbestosis on histopathology?
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Which interstitial lung disease presents with inspiratory crackles, clubbing, and ground-glass opacities on HRCT?
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What is the definitive treatment option for deteriorating interstitial lung diseases?
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What is the main distinguishing feature between Desquamative Interstitial Pneumonia (DIP) and Usual Interstitial Pneumonia (UIP) on HRCT?
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What is the primary cause of respiratory failure in patients with interstitial lung diseases?
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Study Notes
Interstitial Lung Diseases (ILD)
- ILD is a group of disorders characterized by inflammation and scarring in the lungs
- Symptoms: loss, fatigue, anorexia, and physical findings of inspiratory crackles and clubbing
Diagnosis
- Diagnosis is based on H&P, Labs, PFT, CXR, HRCT-Chest, BAL, and Biopsy
- Labs: R/O secondary causes of ILD like CT-Diseases
- PFT: restrictive, decreased DLCO
- ABG: hypoxia, normal/low PaCo2
- CXR: ILD, changes more prominent in lower lobes and periphery (subpleural)
- HRCT-Chest: ground glass then honeycombing changes (worse prognosis)
- BAL: increased Neutrophils (NL=1%)
- Biopsy: open lung, confirms the diagnosis
- Histopathology:
- Usual interstitial pneumonia (UIP): a histopathological term for IPF
- Desquamative interstitial pneumonia (DIP): more inflammation and better response to steroid
- Nonspecific interstitial pneumonia (NSIP): more diffuse and without heterogeneity, reversible and better prognosis
Treatment
- Indication: deteriorating or significant symptoms, inflammatory changes (ground-glass opacities) on HRCT
- Treatment options:
- Steroids and oxygenation
- Immunosuppressive or cytotoxic agents: azathioprine, cyclophosphamide
- Antifibrotic agents: colchicine, D-Penicillamine, γ-interferon
- Lung transplantation (definitive)
Prognosis
- Poor prognosis
- Causes of death: Cor pulmonale, Respiratory failure (3-8 years after onset of Sx.), Malignancy (Adenocarcinoma) is more common
Eosinophilic Granuloma (Histiocytosis X, Langerhans' cell histiocytosis)
- Smokers, M>F
- Presentation: lytic bone lesion, DI (pituitary), Pneumothorax (up to 50%)
- HRCT chest: diffuse centrilobular nodules and cysts formation
- Treatment: stop smoking + steroids
Lymphangioleiomatosis
- Exclusively affects premenopausal women
- Immature SM proliferation in lymphatic, vascular, and alveolar wall
- HRCT-Chest: diffuse thin-walled cystic lesion
- Presentation: recurrent pneumothorax, hemoptysis, chylothorax
- Treatment: No Estrogen, advice against pregnancy, Anti-Estrogen (progesterone), oophorectomy
Desquamative Interstitial Pneumonia
- Smoking-related interstitial disease
- Histopathology: interstitial inflammation and macrophages in alveolar spaces
- HRCT: bilateral ground-glass opacities located in the basal parts of the lung (seen in 92% of patients)
- Responds to steroids
Asbestosis
- Diffuse interstitial fibrosis of the lung caused by inhalation of asbestos fibers
- Predilection for lower lobes
- Increased risk of bronchogenic carcinoma and malignant mesothelioma
Sarcoidosis
- Unknown etiology
- Symptoms:
- Asymptomatic (CXR)
- Acute: fever, LÖfgren’s syndrome (triad of skin lesion, polyarthritis, and bilateral hilar lymphadenopathy)
- Chronic: pulmonary, extrapulmonary
- Presentation:
- Pulmonary: nonproductive cough, progressive SOB
- Extrapulmonary:
- ENT: hoarseness, xerostomia, hearing loss
- Eye: anterior uveitis (75%), enlarged lacrimal gland (characteristic)
- Heart: blocks, arrhythmias, CMP
- Skin: EN, Lupus Pernio
- Nervous System: Bell’s palsy, meningeal, peripheral neuropathy
- Diagnosis:
- H&P
- Labs: ACE (not specific)
- PFT: usually restrictive changes, decreased DLCO
- CXR:
- Stage I: B/L Hilar LN
- Stage II: B/L Hilar LN + ILD
- Stage III: ILD (No LN)
- Stage IV: fibrotic changes (honeycombing), shrinkage the lung
- Gallium scan
- Bronchoalveolar lavage: increase T-lymphocyte (CD4+)
- Biopsy (confirm the diagnosis): transbronchial (more peripheral), mediastinoscopy (for central areas like LN)
- Prognosis:
- Good prognosis
- Most are self-limiting
- Benign course with remission (85%) within 2 years
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Description
Learn about the diagnosis of Interstitial Lung Disease (ILD) through history and physical examination, lab tests, pulmonary function tests, arterial blood gas analysis, imaging studies, bronchoalveolar lavage, and lung biopsy. Understand common findings such as inspiratory crackles, clubbing, and characteristic radiological changes like ground glass and honeycombing patterns.
