Diffuse Parenchymal Lung Diseases With a Known Cause
Pulmonary and Critical Care Medicine · Diffuse Parenchymal Lung Disease 🎯 Practice these cards
The pathologic signature of smoking-related DPLD is accumulation of ____ macrophages, patchy and confined to the respiratory bronchioles in ____ but diffusely filling the alveolar spaces in DIP.
The signature of smoking-related DPLD is accumulation of pigmented ('smoker's') macrophages — patchy and confined to the respiratory bronchioles in RB-ILD, diffuse with pneumocyte hyperplasia and interstitial thickening in DIP; RB-ILD and DIP are best understood as a severity spectrum of the same smoking-driven macrophage injury.
PLCH on HRCT shows nodular lesions and ____ in the ____ lung zones, and its cystic architecture predisposes to recurrent ____.
PLCH on HRCT shows nodular lesions and thin-walled cysts in the upper and mid lung zones; its cystic architecture predisposes to recurrent pneumothoraces, and diagnosis is confirmed by Langerhans cells staining positive for S100 or CD1a on BAL or biopsy.
The primary therapy for every smoking-related DPLD (RB-ILD, DIP, PLCH, SRIF) is ____; ____ are reserved for severe disease or refractory symptoms.
Smoking cessation is the primary therapy for all four smoking-related DPLDs (RB-ILD, DIP, PLCH, SRIF); glucocorticoids are reserved for severe disease or refractory symptoms, and no drug therapy has convincingly improved outcomes beyond cessation itself.
In the 2025 ERS/ATS classification update, desquamative interstitial pneumonia (DIP) was renamed ____ pneumonia, a new ____ interstitial pneumonia (BIP) pattern was added, and acute interstitial pneumonia was replaced by idiopathic diffuse alveolar damage.
The 2025 ERS/ATS update — the first revision of the interstitial pneumonia classification since 2013 — renamed DIP as alveolar macrophage pneumonia (AMP), added a new bronchiolocentric interstitial pneumonia (BIP) pattern, replaced acute interstitial pneumonia with idiopathic diffuse alveolar damage, and unified idiopathic and secondary interstitial pneumonias in a single framework with secondary causes listed first.
Across connective tissue diseases as a group, the most common ILD pattern is ____; the exception is ____, where UIP predominates and carries a survival disadvantage comparable to IPF.
Across connective tissue diseases as a group, NSIP is more common than UIP; rheumatoid arthritis is the exception, where UIP predominates and carries a survival disadvantage comparable to IPF.
In systemic sclerosis the predominant ILD pattern is ____, and the 2025 ERS/EULAR guideline recommends screening all SSc patients with ____ rather than PFTs or lung ultrasound.
In systemic sclerosis, ILD is the most common pulmonary manifestation and NSIP predominates; the first joint ERS/EULAR CTD-ILD guideline (2025/2026) recommends HRCT screening for all SSc patients (strong recommendation) rather than PFTs or lung ultrasound, mycophenolate offers efficacy comparable to cyclophosphamide with better tolerability, and nintedanib is added for progressive fibrosis.
Antisynthetase syndrome is defined by inflammatory myopathy with ILD and anti-aminoacyl-tRNA synthetase antibodies (prototype ____); its rapidly progressive ILD form is underlain by a ____ pattern.
Antisynthetase syndrome — polymyositis or dermatomyositis with radiographic ILD plus anti-aminoacyl-tRNA synthetase antibodies (anti-Jo-1 prototype) — can present as severe, rapidly progressive ILD without overt systemic disease; a diffuse alveolar damage pattern underlies the rapidly progressive form, and myositis-specific antibodies should be checked in any unexplained ILD, particularly in younger patients.
In acute hypersensitivity pneumonitis, symptoms develop within ____ hours of a large antigen exposure and typically resolve within about ____ hours of removal from exposure; recurrence on ____ is the clinical hallmark.
Acute hypersensitivity pneumonitis produces fever, cough, and fatigue within 12 hours of a large antigen exposure, typically resolves within about 48 hours of removal from exposure, and recurrence on rechallenge is the clinical hallmark of the disease.
The presence of ____ on HRCT (septal thickening, traction bronchiectasis, honeycombing) is the prognostic fork in HP: chronic fibrotic HP carries a substantially increased risk of progression and death, behaving more like a ____ than a reversible inflammatory disease.
Fibrosis on HRCT — septal thickening, traction bronchiectasis, honeycombing — is the prognostic fork in HP: chronic fibrotic HP carries a substantially increased risk of progression and death, behaving more like a fibrosing interstitial pneumonia than a reversible inflammatory one, and should be monitored like any progressive pulmonary fibrosis.
Radiation pneumonitis typically presents ____ weeks after thoracic radiation; the pathognomonic imaging finding is a sharply demarcated, nonanatomic boundary conforming to the ____, and the late fibrotic phase emerges ____ months after exposure.
Radiation pneumonitis typically presents 4 to 12 weeks after thoracic radiation with cough, dyspnea, and a new infiltrate; the pathognomonic imaging finding is a sharply demarcated, nonanatomic boundary conforming to the radiation port, and the late fibrotic phase (septal thickening, traction bronchiectasis, volume loss) emerges 6 to 12 months after exposure.
____ pneumonitis is re-inflammation of previously irradiated lung triggered by later exposure to checkpoint inhibitors or chemotherapeutic agents; a new infiltrate confined to ____ in a patient who recently started such an agent is this diagnosis until proven otherwise.
Previously irradiated lung can be re-inflamed ('recalled') by later exposure to immune checkpoint inhibitors or chemotherapeutic agents (doxorubicin, etoposide, gemcitabine, paclitaxel, pemetrexed); a new infiltrate confined to an old radiation port in a patient who recently started one of these agents is recall pneumonitis until proven otherwise.
Because of its very long half-life, amiodarone lung toxicity can progress despite ____, and ____ during glucocorticoid taper is common, so a slow taper is required.
Amiodarone's very long half-life means drug persists in lung parenchyma long after discontinuation: improvement with stopping alone is rare, toxicity can progress despite discontinuation, and relapse during glucocorticoid taper is common — plan a slow taper.
Per NCCN, grade 1 (asymptomatic) checkpoint-inhibitor pneumonitis is managed with ____, grade 2 with prednisone 1-2 mg/kg/day, and grade 3-4 with IV methylprednisolone plus hold or permanent discontinuation; if there is no improvement at 48-72 hours, escalate to IVIG, mycophenolate, or ____.
NCCN guidance grades management of checkpoint-inhibitor pneumonitis: grade 1 (asymptomatic) — observe and monitor; grade 2 — prednisone 1-2 mg/kg/day; grade 3-4 — IV methylprednisolone 1-2 mg/kg/day with hold (grade 3) or permanent discontinuation (grade 4); assess at 48-72 hours, taper over 4-8 weeks, and if no improvement escalate to IVIG, mycophenolate, or infliximab. Median onset is about 2-3 months and baseline lung fibrosis markedly raises risk.
Antibody-drug conjugate-associated ILD is a class effect driven by off-target uptake of the cytotoxic payload by ____; the best-characterized agent, trastuzumab deruxtecan (T-DXd), carries an incidence of roughly ____ with a fatal-event rate near 2.2%.
ADC-associated ILD is a class effect — reported with HER2-, TROP2-, and HER3-directed agents — driven by off-target uptake of the cytotoxic payload by alveolar macrophages; the best-characterized agent, trastuzumab deruxtecan (T-DXd), carries an incidence of roughly 10-15% with median onset at 5-6 months and a fatal-event rate near 2.2%, managed with baseline risk assessment, serial HRCT surveillance, prompt ADC interruption at grade 1-2, and corticosteroids.
The 2025 adult LCH guideline reframes PLCH as a neoplasm of the ____ pathway: somatic mutations drive it, with ____ found in roughly half of cases, and targeted dabrafenib plus trametinib has documented lung-function responses.
The 2025 adult Langerhans cell histiocytosis guideline reframes PLCH as a MAPK-pathway neoplasm: somatic MAPK-pathway mutations drive it, BRAF V600E is found in roughly half of cases with tobacco smoke as the key etiologic cofactor, and progressive PLCH despite smoking cessation can be treated with cladribine or targeted BRAF/MEK inhibition (dabrafenib plus trametinib) with documented lung-function responses.
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