Patient will present as →a 55-year-old female who is a non-smoker presents with a 9-month history of respiratory symptoms, including dyspnea on exertion, thoracic pain, and dry cough, which were preceded by a pulmonary infection. On auscultation, you hear inspiratory crackles. Pulmonary function tests (PFTs) show a restrictive pattern with a decreasein total lung capacity,decreased forced vital capacity (FVC), and preserved FEV1/FVC ratio.Chest CT demonstrates diffuse patchy fibrosis with pleural-based honeycombing.
Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason
Over time, the scarring gets worse, and it becomes hard to take in a deep breath, and the lungs cannot take in enough oxygen
Etiology unknown; MC of all interstitial lung diseases
In order to be considered "idiopathic," you must rule out other common causes, such as drugs (amiodarone) and environmental or occupational exposures
risk factors: older age, male sex, smoking, genetics/family history (± GERD, viral infections); must rule out: connective tissue disease, hypersensitivity pneumonitis, occupational dusts (asbestos, silica), drugs, radiation
Physical exam: Inspiratory crackles
DX: HRCT is the key test (CXR may show basilar reticular opacities)
HRCT: usual interstitial pneumonia (UIP) pattern — basal, subpleural reticulation with honeycombing and traction bronchiectasis (definite UIP = no biopsy needed)
PFTs = restrictive (decreased lung volume, normal/increased FEV1/FVC ratio) with a reduced DLCO
PFTs = restrictive
Decreased total lung capacity (TLC)
Decreased forced vital capacity (FVC)
Decreased forced expiratory volume in the first second (FEV1)
Despite the reduction in FVC, the FEV1/FVC ratio is preserved or even increased because both FEV1 (forced expiratory volume in the first second) and forced vital capacity (FVC) are proportionally reduced
Tx: antifibrotics — nintedanib or pirfenidone (nerandomilast is a newer option) slow the decline in FVC
O2 for hypoxemia, pulmonary rehab, early lung transplant referral
No corticosteroids for stable IPF (they can be harmful); steroids only for acute exacerbations
Pulmonary fibrosis induced by amiodarone.
HRCT in IPF: usual interstitial pneumonia (UIP) pattern with basal, subpleural honeycombing and traction bronchiectasis. Image: Yale Rosen, CC BY-SA 2.0
Any fibrosis of the lung tissues with a known cause - usually from prolonged environmental or occupational contact
Patient with coal workers pneumoconiosis will present as → a 62-year-old male, a retired coal miner, who arrives at the clinic complaining of a persistent cough and shortness of breath that has gradually worsened over the past two years. He mentions he worked in coal mines for over 35 years. The cough is dry, and he denies any significant sputum production. He also reports a recent loss of appetite and mild weight loss. His medical history is significant for smoking a pack of cigarettes a day for 40 years, though he quit 5 years ago. On examination, he appears tachypneic with a respiratory rate of 22 breaths per minute. Auscultation of the lungs reveals bilateral rales but no wheezing. Chest X-ray shows small, rounded opacities throughout the lung fields and upper lobe predominance. Pulmonary function tests indicate a mild restrictive pattern.
Patient with silicosis will present as → a 57-year-old male working as a stone cutterwho comes to the clinic complaining of progressive breathlessness and a persistent dry cough over the last few months. He has been in the stone-cutting business for approximately 30 years and reports consistent exposure to dust without adequate respiratory protection. He denies smoking or any past significant medical history. On physical examination, he is noted to be short of breath while talking. Auscultation reveals fine bilateral end-inspiratory crackles, predominantly in the upper lobes. His oxygen saturation on room air is 94%. Chest X-ray demonstrates bilateral nodular opacities and hilar lymphadenopathy. High-resolution computed tomography (HRCT) of the chest confirms the presence of multiple small rounded nodules with upper lobe predominance and eggshell calcificationof the hilar nodes, supporting the diagnosis of silicosis.
Silicosis: mining, sandblasting, stone, quarry work, engineered-stone countertops; increased risk of TB (test for latent TB), lung cancer, and progression to massive fibrosis
CXR: small, rounded opacities throughout the lung, hilar lymph nodes may be calcified - "eggshell" calcifications
Patient with asbestosis will present as →a 53-year-old man presents to the office complaining of progressive dyspnea over the past few years. History reveals that he has worked in construction for the past 20 years, demolishing and refurbishing old buildings. He rarely uses any protective breathing equipment. Physical examination demonstrates an afebrile man in mild respiratory distress with inspiratory crackles. The chest x-ray reveals a reticular linear pattern with basilar predominance, opacities, and honeycombing.
Asbestos: insulation, demolition, shipbuilding, construction; complications = lung cancer (most common; risk multiplied by smoking) and mesothelioma (most specific; not linked to smoking)
CXR: interstitial fibrosis, thickened pleura, calcified plaques appear on diaphragms or lateral chest wall
Patient with Berylliosis will present as → a 52-year-old female who works in an aerospace manufacturing facility, presenting with a 6-month history of increasing shortness of breath, dry cough, and fatigue. She reports no smoking history or significant past medical issues. On examination, she appears to be in respiratory distress, with a respiratory rate of 26 breaths per minute. Auscultation of her lungs reveals diffuse crackles. Chest X-ray shows bilateral infiltrates and hilar lymphadenopathy. Her work history reveals prolonged exposure to beryllium. Pulmonary function tests indicate a restrictive pattern and a subsequent lung biopsy demonstrates noncaseating granulomas. A beryllium lymphocyte proliferation test (BeLPT) confirms the diagnosis of chronic berylliosis.
Berylliosis: high-tech field, nuclear power, ceramics, aerospace, electrical plants, foundries; remove from exposure + corticosteroids for symptomatic or progressive disease (often long-term); confirm with the beryllium lymphocyte proliferation test (BeLPT)
CXR: diffuse infiltrates and hilar adenopathy
S/SX: SOB + nonproductive cough + chronic hypoxia, cor pulmonale
DX: exposure history + CXR (interstitial fibrosis or nodules); HRCT is more sensitive
Patient will present as → a 38-year-old African-American woman who visits the clinic complaining of persistent dry cough, shortness of breath, and mild chest pain for the past two months. She also notes occasional fevers and unexplained weight loss. You note erythema nodosum on her lower legs. Her chest X-ray reveals bilateral hilar lymphadenopathy and reticular opacities. Pulmonary function tests show a restrictive lung disease pattern. Laboratory findings include elevated serum angiotensin-converting enzyme (ACE) levels. A biopsy of the lymph nodes obtained during bronchoscopy demonstrates noncaseating granulomas, consistent with a diagnosis of sarcoidosis.
Multisystem granulomatous disease of unknown cause (an exaggerated immune response) in which small nodules (granulomas) develop in lungs, lymph nodes, and other organs
Lupus pernio (chronic, violaceous, raised plaques and nodules commonly found on the nose, cheeks, ears, and lips) = pathognomonic for sarcoid and most specific physical exam finding
DX: Sarcoidosis is diagnosed by compatible clinical and radiographic findings with biopsy-proven noncaseating granulomas after excluding infection and malignancy
Biopsy → non-caseating granulomas; for hilar/mediastinal nodes, EBUS-guided needle aspiration is the preferred first procedure
No biopsy needed for classic Löfgren syndrome (erythema nodosum + bilateral hilar adenopathy + fever or ankle arthritis); treat with NSAIDs — it usually resolves
ACE is elevated in many patients but is not sensitive or specific enough for diagnosis; baseline ECG and eye exam for everyone
"Sarcoidosis causes elevated ACE and hypercalcemia because activated macrophages within granulomas produce ACE and convert inactive vitamin D into its active form, increasing intestinal calcium absorption."
TX: many patients need no treatment (asymptomatic disease often remits); symptomatic or organ-threatening disease → corticosteroids (prednisone) act fastest (classic first-line); severe/progressive disease needing long-term therapy → methotrexate often preferred (± prednisone if rapidly progressive)
Methotrexate = main steroid-sparing agent; TNF inhibitors (infliximab) for refractory disease
Serial PFTs to assess disease progression/guide treatment
Prognosis depends on disease severity; spontaneous improvement common
Pulmonary fibrosis = leading cause of death; cardiac sarcoidosis (heart block, ventricular arrhythmias) is next
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Osmosis
