Interstitial Lumg Diseases Flashcards

Question

What are the most common symptoms in ILD and what is important in history?

Answer 1

1- Age and Gender. 2-Nonproductive cough and dyspnea with (subacute to chronic course) are the most common presenting symptoms of a DPLD. 3-When DPLD is suspected, questions should focus on determining the onset of symptoms, system review (CTD related symptoms), past medical history, medications, exposures ( occupations, home environment, hobbies, and other activities) and smoking history.

Answer 2

The physical examination of patients with ILD is usually nonspecific. 1-In patients with connective tissue disorders, findings may include Raynaud phenomenon, skin thickening, sclerodactyly, inflammatory arthritis, or tenosynovitis. This is very important **** 2- Resting and exertional pulse oximetry. It is common for patients with DPLD to have normal resting pulse oximetry while > 4% drop in O2 saturation suggestive of ILD. 3- Lung examination: is frequently abnormal in ILD, but the findings are generally nonspecific. Crackles or "velcro rales" are present on chest examination in most forms of ILD. 4-Signs of right sided heart failure suggestive of long standing ILD.

Answer 3

Air within fluid ( the fluid is thick like sputum)

Answer 4

Make him cough by using auscultation.

Answer 5

1-The first and PFT. 2- HIGH RESOLUTION CT( HRCT) is the best: scan of the chest (slice thickness 1-2 mm) is the best imaging study to identify abnormalities that can help diagnose the underlying disease.

Answer 6

Reticulondular

Answer 7

The American Thoracic Society guidelines recommend screening all patients with DPLD with an antinuclear antibody (ANA), rheumatoid factor, and anti-cyclic citrullinated peptide antibody appropriate in the following: 1-Younger patients, in particular those younger than 40 years of age. 2-Patients with symptoms of an underlying rheumatologic disorder. 3-Patients with a family history of autoimmune or rheumatologic disease.

Answer 8

the physician must consider the risks and benefits of eithe: 1-bronchoscopic or 2- surgical lung biopsy, including the patient's general health and risk of intervention.

Answer 9

a small area of chronic inflammation. Granulomas form when immune cells clump together and create tiny nodules at the site of the infection or inflammation. A granuloma is the body's way: to contain an area of bacterial, viral or fungal infection so it can try to keep it from spreading; or. to isolate irritants or foreign objects.

Answer 10

multisystem granulomatous disorder. 1- It commonly affects young adults. 2-It is a common disease of unknown etiology that is often detected on routine chest X-ray. 3-Typically presents with bilateral hilar lymphadenopathy, pulmonary infiltration and skin or eye lesions. 4- Typical sarcoid granulomas consist of focal accumulations of epithelioid cells, macrophages and lymphocytes, mainly T cells.

Answer 11

due to increased production of 1,25-dihydroxy vitamin D by granuloma that increase the activation of inactive form of vitamin D 25-hydroxlase

Answer 12

Pulmonary involvement may be an i ncidental finding, cough, exertional SOB and vague chest discomfort are common presentations.

Answer 13

1-Stage I: BHL (usually symmetrical); paratracheal nodes often enlarged: Often asymptomatic but may be associated with erythema nodosum and arthralgia. The majority of cases resolve spontaneously within 1 year 2-Stage II: BHL and parenchymal infiltrates : Patients may present with breathlessness or cough. The majority of cases resolve spontaneously. 3- Stage III: parenchymal infiltrates without BHL: Disease less likely to resolve spontaneously ( require treatment). 4- Stage IV: pulmonary fibrosis: Can cause progression to ventilatory failure, pulmonary hypertension and cor pulmonale.( end stage) (BHL = bilateral hilar lymphadenopathy)

Answer 14

It is a triad and a typical combination of: 1-bilateral hilar lymphadenopathy, 2-erythema nodosum, 3-arthralgia( ankle joint) R- and fever.  Has excellent prognosis as usually resolves spontaneously without the need for treatment.

Answer 15

Reassurance

Answer 16

 Imaging. Chest X-ray is the initial modality for staging, followed by HRCT for assessment of parenchymal involvement.  CBC. There may be cytopenia , mild normochromic, normocytic anemia with raised ESR.  Biochemistry. Calcium Level as activated macrophages in lung and lymph nodes are able to hydroxylate vitamin D leading to increased intestinal absorption of dietary calcium.  Serum ACE level. Less specific, so used for disease monitoring. Lung function tests. Showed restrictive pattern as disease progress.  Biopsy. Non-caseating granulomas.

Answer 17

Pulmonary Sarcoidosis has good prognosis, spontaneous remission within 6 months.  Systemic treatment is indicated for hypercalcemia and extrathoracic major organ involvement, particularly neurological, cardiac, progressive pulmonary or ocular disease resistant to topical therapy.  1-First-line treatment is with Prednisone 0.5 mg/ kg for 4–6 weeks, gradually tapering to a maintenance dose for at least 12 months.  2-Alternative immunosuppressants, including methotrexate. 3-  Lung transplantation should be considered for suitable patients with stage IV disease and respiratory failure.

Answer 18

Hypersensitivity pneumonitis (HP) is caused by an allergic reaction affecting the small airways and alveoli in response to an inhaled antigen. ***Cigarette smokers have a lower risk of developing HP due to decreased antibody reaction to the antigen, but once established, smoking may lead to a more chronic or severe disease course.

Answer 19

End-inspiratory wheezing

Answer 20

HP can be categorized according to the time course of symptoms, as determined by duration and intensity of exposure. 1- Symptoms include weight loss, malaise, dyspnea and cough. 2- Auscultation reveals inspiratory squeaks due to bronchiolitis, and bilateral fine crackles. Levels of symptoms: 1-Acute: symptom onset 4–6h following exposure. Fever is common and patients may be mistakenly diagnosed with a chest infection. Resolution occurs 24–48h following removal from the inciting antigen. 2-Subacute: usually occurs with intermittent or lower-level exposure. Improvement is seen in weeks to months following removal from exposure. 3-Chronic: usually no history of preceding acute symptoms. Insidious onset of respiratory and constitutional symptoms is typical. Finger clubbing may be present. Progression to irreversible fibrosis is associated with increased mortality.

Answer 21

Mosaic pattern

Answer 22

The key to successful treatment is avoidance of exposure to the inciting antigen (if known) and this may be achieved by changes in work practice.  Pigeon fancier’s lung is more difficult to control, as affected individuals remain strongly attached to their hobby.  Prednisone should be initiated in patients whose symptoms persist despite withdrawal from the causative antigen, and in severe disease.  Established fibrosis will not resolve and, in some patients, the disease may progress inexorably to respiratory failure.

Answer 23

Only If the exposure known cause known or the antigen known

Answer 24

IPF is the most common of the idiopathic interstitial pneumonias. It is a progressive and ultimately fatal disease of unknown cause. Affecting old ages >50 years. It is thought that repetitive injury to the alveolar epithelium, caused by currently unidentified environmental stimuli, leads to the activation of several pathways responsible for repair of the damaged tissue.

Answer 25

insidious onset of progressive dyspnea that may be accompanied by cough, with or without sputum production.

Answer 26

1- chest shows bi-basal end inspiratory crackles (velcro rales) . 2- finger clubbing. 3. Acute form (fulminant) called Hamman-Rich Syndrome.

Answer 27

1-PFT usually show a restrictive pattern (FEV1/FVC ratio >70%) with reduced total lung volumes and capacity. However, spirometry may be normal in early disease. 2- Serological tests, Bronchoalveolar lavage and possible lung biopsy >>> To Rule out alternative diagnosis (as it is disease of exclusion). 3-Chest X-ray shows small-volume lungs with increased reticular shadowing at the bases but may be normal in early disease.

Answer 28

HRCT is the imaging modality of choice. A confident diagnosis of IPF may be made in patients with: 1-Basal distribution. 2-Subpleural reticulation: reticulation is most evident in the lung peripheries. 3-Traction bronchiectasis: the fibrotic process distorts the normal lung architecture, pulling the airways open and causing bronchiectasis. 4-Honeycombing: there are basal layers of small, cystic air-spaces with irregularly thickened walls composed of fibrous tissue.

Answer 29

2-5 years And Mortality is increased following acute exacerbations.

Answer 30

1-Immunosuppression is generally avoided in IPF and 2-steroids are no longer recommended in confirmed disease.

Answer 31

1- Pirfenidone, an antifibrotic agent, has been shown to slow the rate of FVC decline, with the most common side-effects being a reversible photosensitive rash and gastrointestinal disturbance. 2- Other treatments include Nintedanib, an intracellular inhibitor of tyrosine kinases.

Interstitial Lumg Diseases Flashcards

(60 cards)