Diffuse Parenchymal Lung Disease

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  1. What are the known causes of DPLF?
    • Connective tissue disease-associated (i.e., RA, polymyositis, scleroderma)
    • Hypersensitivity pneumonitis
    • Pneumoconioses (i.e., asbestosis, silicosis, coal worker's pneumoconiosis)
    • Drug-induced
    • Smoking-related
    • Pulmonary Langerhans cell histiocytosis
    • Respiratory bronchiolitis interstitial lung diseasse
    • Desquamative interstitial pneumonia
    • Radiation-induced
    • Toxic inhalation-induced
  2. What are the important components in the diagnostic approach to pts with DPLF?
    • Clinical context
    • Temporal pattern
    • Radiographic abnormalities
  3. If tissue is needed for the diagnosis of DPLF, which one has the highest yield?
    The diagnostic yield of surgical lung biopsy is approximately 90%.
  4. What patients are high risk for video-assisted thoracoscopic surgery (VATS)?
    VATS lung biopsy has a reported mortality rate of 2% and a complication rate of between 5% and 10%. High-risk patients with little potential benefit from surgical lung biopsy include those with suspected idiopathic pulmonary fibrosis who have a DLCO less than 20% of predicted or an accelerated clinical decline.
  5. What may be beneficial to treat hypersensitivity-type pulmonary reaction to drugs?
  6. What is the time of occurrence for Radiation pneumonitis?
    Nonspecific symptoms begin 1 to 6 months after radiation therapy has been completed and include fever, weight loss, dry cough, and dyspnea. Physical examination of the chest usually shows only skin changes from radiation exposure and the occasional presence of a pleural rub. The disease can be severe, with progression over days to weeks leading to acute respiratory failure. The clinical and radiographic worsening over days to weeks is a helpful clue to distinguish radiation pneumonitis from recurrent local or metastatic cancer. The radiographic features typically consist of alveolar opacification of the irradiated portion of the lung with occasional air bronchograms. As confluence progresses, a nonanatomic “edge” may be seen on the chest radiograph, which reflects the border of the radiation port. Radiation pneumonitis can occur even outside the radiation field and rarely affects the contralateral lung.
  7. What is Radiation fibrosis (DPLD)?
    Radiation fibrosis is clinically distinct from radiation pneumonitis, although the diseases may have overlapping clinical presentations. Radiation fibrosis can occur in patients with or without a history of radiation pneumonitis. Radiation fibrosis occurs 6 to 24 months after radiation therapy and represents a long-term fibrotic sequela of lung damage, most often within the radiation field. Symptoms are uncommon, but patients with previous marginal lung function may have worsening dyspnea. The fibrotic process is irreversible.
  8. What is the PFT pattern in pts with hypersensitivity pneumonitis?
    Pulmonary function tests in patients with hypersensitivity pneumonitis may show an obstructive or a restrictive pattern with a low DLCO.
  9. What are the key histopathologic features of acute hypersensitivity pneumonitis?
    The key histopathologic features of acute hypersensitivity pneumonitis include poorly formed granulomas with bronchiolitis and lymphoplasmacytic interstitial infiltration.
  10. Treatment for hypersensitivity pneumonitis?
    • Avoidance of exposure to the allergen
    • Prednisone
  11. What interstitial lung diseases are associated with smoking?
    Interstitial lung diseases associated with smoking include pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis–associated interstitial lung disease, and desquamative interstitial pneumonia. Smoking may also be a risk factor for the development of idiopathic pulmonary fibrosis.
  12. Describe Pulmonary Langerhans cell histiocytosis.
    • A lung disease associated with smoking.
    • Pulmonary Langerhans cell histiocytosis (formerly called histiocytosis X or eosinophilic granuloma) typically presents in a young (less than 40 years of age) current smoker. Pneumothorax occurs in 25% of patients with the disorder.
    • The temporal pattern of the disease tends to be subacute, with gradual worsening of cough and dyspnea.
    • Pulmonary function studies typically reveal an obstructive pattern with low DLCO in those with more severe parenchymal involvement, and those with mild disease may have obstructive, restrictive, or normal lung function.
    • HRCT showing a pattern of nodules accompanied by thin-walled cysts in an upper and middle lung-field distribution is virtually pathognomonic.
  13. What is the initial treatment for Pulmonary Langerhans cell histiocytosis?
    The initial treatment is smoking cessation. The disease may stabilize in patients who stop smoking, but most patients show evidence of gradual progression. In patients with the related systemic disease of Langerhans cell histiocytosis, chemotherapy with novel agents such as cladribine (2-chlorodeoxyadenosine [2-CdA]) appears to be effective, but such therapy has not been evaluated for treatment of smoking-related pulmonary Langerhans cell histiocytosis without systemic disease.
  14. What is Respiratory bronchiolitis–associated interstitial lung disease (RB-ILD) ?
    • a form of bronchiolitis that occurs in most smokers and is occasionally severe enough to cause clinical symptoms and characteristic radiographic abnormalities. Affected patients present with the gradual onset of persistent cough and dyspnea with impaired gas exchange
    • Pulmonary function tests show an obstructive or mixed obstructive/restrictive pattern.
    • Chest radiograph may be normal but usually shows a pattern of bronchial wall thickening and alveolar infiltrates in a central and basilar distribution.
    • HRCT shows a pattern of centrilobular nodules with air-trapping and scattered ground-glass attenuation.
  15. What is Desquamative Interstitial Pneumonia?
    • DIP is a rare disorder that shares causal and clinical features and treatment with RB-ILD.
    • Treatment of RB-ILD and DIP includes smoking cessation and avoidance of all passive exposure to smoke; most patients who avoid smoke improve. Corticosteroids are commonly used in severe disease but are of uncertain efficacy. The disorders progress in a small subset of patients despite smoking cessation and corticosteroid therapy.
  16. What is the role of bronchoscopic lung biopsy in the diagnosis of Idiopathic Interstitial Pneumonia?
    • Bronchoscopic lung biopsy is
    • not helpful because recognition of usual interstitial
    • pneumonia requires a larger sample than can be obtained by this
    • procedure.
    • Surgical lung biopsy is required if HRCT features that suggest usual interstitial pneumonia, including reticular opacities and honeycombing with a peripheral and basilar predominance and minimal or no ground-glass opacifications, are not present.
  17. What is Nonspecific Interstitial Pneumonitis?
    a rare idiopathic disorder that shares many clinical and radiologic features with idiopathic pulmonary fibrosis. HRCT features of nonspecific interstitial pneumonitis include basilar or mid-lung reticular changes, rarely with honeycombing, and a dominant pattern of ground-glass opacification in the mid- and lower-lung zones
  18. How is Nonspecific Interstitial Pneumonitis diagnosed?
    A diagnosis of nonspecific interstitial pneumonitis requires open lung biopsy.
  19. What is the treatment for Nonspecific Interstitial Pneumonitis?
    Corticosteroids with or without cytotoxic agents
  20. What is Cryptogenic Organizing Pneumonia?
    • Cryptogenic organizing pneumonia (formerly called idiopathic bronchiolitis obliterans organizing pneumonia) is an idiopathic lung disease with the relatively nonspecific histopathologic correlate of organizing pneumonia.
    • Diagnosis requires organizing pneumonia as the primary histopathologic finding in conjunction with clinical exclusion of known associated conditions. Surgical lung biopsy is the preferred technique, although bronchoscopic lung biopsy occasionally provides sufficient tissue for diagnosis.
    • systemic corticosteroids result in rapid symptomatic improvement and gradual resolution of radiologic findings
  21. What is Sarcoidosis?
    a multisystem, granulomatous, inflammatory condition of unknown cause that occurs in young adults of both sexes. The temporal pattern of disease progression ranges from asymptomatic to acute systemic presentations with fever, erythema nodosum, polyarthralgia, and hilar lymphadenopathy (Löfgren syndrome).
  22. What is Lofgren Syndrome?
    • fever, erythema
    • nodosum, polyarthralgia, and hilar lymphadenopathy in a pt with sarcoidosis
  23. How is saorcoidosis diagnosed?
    The diagnosis of sarcoidosis requires histopathologic demonstration of organ involvement with noncaseating granulomatous inflammation in conjunction with the appropriate clinical and radiographic findings and the exclusion of other diseases. Diagnostic tissue can be obtained from any involved organ. Bronchoscopy has a diagnostic yield of up to 90% when multiple transbronchial lung biopsies are combined with endobronchial biopsies.
  24. What is the treatment for sarcoidosis in a pt with asymptomatic radiographic abnormalities
    • None if with asymptomatic radiologic changes
    • Prednisone if symptomatic
    • Patients who do not tolerate corticosteroids or who have resistant disease may be treated with methotrexate, hydroxychloroquine, or azathioprine.
  25. What is lymphangiomyomatosis?
    • a rare progressive cystic lung disease accounting for less than 1% of all interstitial lung disease and occurring almost exclusively in women in their third or fourth decade. The disorder occurs as either a sporadic isolated pulmonary disease or, in approximately 15% of patients, in conjunction with tuberous sclerosis.
    • most common symptom at presentation was breathlessness
  26. What is the usual presentation in Lymphangioleiomyomatosis?
    Nearly 90% of all patients diagnosed with lymphangioleiomyomatosis (with or without tuberous sclerosis) presented with respiratory complications; 36% presented for medical evaluation because of spontaneous pneumothorax, with an overall incidence of spontaneous pneumothorax of 55% in all patients.
  27. How is Lymphangioleiomyomatosis diagnosed?
    • The diagnosis is based on imaging studies in the appropriate clinical context. Many young women are initially diagnosed with emphysema. Spontaneous pneumothoraces, chylothorax, and young age indicate that lymphangioleiomyomatosis should be considered. Chest radiographic findings paradoxically show diffuse reticulonodular infiltrates associated with hyperinflation.
    • HRCT findings may be diagnostic when demonstrating a pattern of diffuse, thin-walled, small cysts that are distributed diffusely throughout the lung. Surgical lung biopsy is not required, but when obtained, shows proliferation of smooth-muscle cells and cysts. T
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Diffuse Parenchymal Lung Disease
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