Module 4.3 - Restrictive (Inflammatory) Lung Diseases Flashcards

1
Q

Define restrictive lung disease

A

Restrictive lung diseases are characterized by reduced lung volumes. Unlike obstructive lung diseases which show a normal or increased total lung capacity (TLC), restrictive diseases are associated with a decreased TLC.

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2
Q

What causes restrictive lung disease?

A

There are many disorders that cause reduction or restriction of lung volumes.

  1. The first is intrinsic lung diseases or diseases of the lung parenchyma.
    • The diseases cause scarring of the lung tissue (interstitial lung disease) or result in filling of air spaces with exudate and debris (pneumonitis)
  2. The second is extrinsic disorders or extra pulmonary disorders – specifically disorders of the chest wall, pleura and respiratory muscles (neuromuscular disorders).
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3
Q

What is pneumonia?

A
  • Acute febrile inflammatory disorder of the lung(s) associated with cough and exertional dyspnea.
  • An infiltrate is seen on CXR, sometimes 24-48 hrs. after symptoms begin.
  • Leukocytosis may be present.
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4
Q

Describe the etiology/incidence associated with pneumonia

A
  • Most common of all serious lung conditions, frequent cause of acute care hospitalizations and mortality
  • Elderly at risk due to poor immune systems, debilitated state, fragility
  • Over treatment with antibiotics of mild URI has contributed to antimicrobial drug resistance, especially S. pneumoniae
  • These comorbidities cause a higher mortality in patients with pneumonia: COPD, HF, DM, chronic liver and kidney disease.
  • Very young and very old patients are also at high risk for death despite adequate treatment with antibiotics.
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5
Q

What are the typical symptoms associated with pneumonia?

A

fever, chills, leukocytosis, cough, and sputum production increased fremitus. CXR may show single infiltrate

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6
Q

What are the atypical symptoms associated with pneumonia?

A

fever may be high, +/- leukocytosis. A left shift may be present, dry cough, HA, sore throat, excessive sweating, chest soreness,

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7
Q

What are the common organisms associated with Community acquired pneumonia (CAP)?

A
  • Most common bacteria – Streptococcus pneumoniae (most common), Haemophilus influenza, Klebsiella pneumoniae, gram negative organisms.
  • Atypical pathogens – Chlamydia pneumoniae, Mycoplasma pneumoniae, Mycobacterium tuberculosis.
  • Viruses – respiratory syncytial virus, adenovirus, rhinovirus
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8
Q

What is and what are the common organisms associated with Hospital acquired pneumonia (HAP)?

A

It is a Nosocomial infection that occurs 48 hrs. or more after admission. The most common organisms include:

  1. Staphylococcus aureus
  2. Streptococcus pneumoniae
  3. Haemophilus influenzae
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9
Q

What is and what causes Ventilator acquired pneumonia (VA)?

A
  • Pneumonia that occurs 48-72 hrs. after intubation.
  • Most common culprit: Pseudomonas
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10
Q

How would you evaluate a patient with suspected pneumonia?

A

Conduct a physical exam and look out for – tachycardia, tachypnea, fever, rales or crackles present over the affected lung, mental status changes, or confusion

Obtain a CXR – assess for infiltrates, may repeat if no infiltrate seen initially but pneumonia is still suspected

Check – CBC including white count, blood cultures, gram stain and culture of sputum, ABG and spirometry, procalcitonin levels if available.

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11
Q

How do you treat outpatient pneumonia?

A
  • Treat initially with a macrolide (azithromycin, clarithromycin, Erythromycin) or doxycycline for 5 days
  • Therapy should be revised as culture results are reported, if indicated, as initial treatment is empiric.
  • Remember to assess patient clinically for ongoing improvement or worsening of symptoms. Use the narrowest spectrum antibiotic. Cover anaerobic organisms incases of known or suspected pneumonia. Not all patients need hospitalization for treatment
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12
Q

What is the criteria for ICU admission for patients with pneumonia?

A
  • Need mechanical ventilation
  • Have hypotension (systolic BP ≤ 90 mm Hg) that is unresponsive to volume resuscitation
  • Other criteria that mandate consideration of ICU admission include
  • Respiratory rate >30/min
  • Pao2/fraction of inspired oxygen (Fio2) < 250
  • Multilobar pneumonia
  • Diastolic BP < 60 mm Hg
  • Confusion
  • BUN > 19.6 mg/dL
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13
Q

What does the CURB-65 scoring system stand for and what is it used for?

A
  • Confusion
  • Uremia (BUN ≥19 mg/dL)
  • Respiratory rate > 30 breaths/min
  • Systolic BP < 90 mm Hg or diastolic BP ≤ 60 mm Hg
  • Age ≥ 65 yr

1 point is given for each risk factor and when added up determines the need for hospitalization.

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14
Q

Describe the point system used in the CURB-65 system.

A

0 or 1 points: Risk of death is < 3%. Outpatient therapy is usually appropriate.

2 points: Risk of death is 9%. Hospitalization should be considered.

≥ 3 points: Risk of death is 15 to 40%. Hospitalization is indicated, and, particularly with 4 or 5 points, ICU admission should be considered.

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15
Q

What 2 vaccinations are available to prevent pneumonia?

A
  1. PCV 13: pneumococcal conjugate vaccine for infants, children and adults older than 19 years at high risk for disease
  2. PPSV23: pneumococcal polysaccharide vaccine for adults older than 65 years of age and those older than 2 years of age at high risk.
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16
Q

What causes tuberculosis?

A
  • Mycobacterium tuberculosis.
  • The lungs are the major site for primary infection and disease. Manifestations include primary TB and reactivation TB.
17
Q

What are the symptoms associated with tuberculosis?

A

Many patients are asymptomatic.

Typical symptoms include:

  • Fever is generally gradual, begins with low grade, can be as high as 390 C and last for 14-21 days
  • Cough
  • Productive, purulent sputum that may contain blood
  • Weight loss
  • Night sweats severe enough that may require changing bed linens
  • Dyspnea can occur in the setting of extensive parenchymal involvement, if pleural effusions develop or if a pneumothorax occurs.
18
Q

What are the physical exam findings found in someone with tuberculosis?

A

Physical exam findings:

  • febrile
  • cachexia may be noted (consumption)
  • rales over the affected areas
  • atypical posttussive rales (heard after a short cough).
  • Dullness with decreased fremitus may indicate pleural thickening.
  • Distant hollow breath sounds may be heard.

In the absence of treatment:

  • patients may present with painful ulcers of the mouth, tongue, larynx or GI tract due to chronic expectoration and swallowing highly infectious secretions. These findings are rare in the setting of treatment. In general, patients in the ambulatory setting will present with milder symptoms than those diagnosed in the hospital.
19
Q

What lab changes are found in patients with TB (CBC, Chemistries, Cortisol, Sputum)?

A
  • CBC – frequently normal initially, normocytic anemia, leukocytosis may be seen in later disease
  • Chemistries – frequently normal initially. Hyponatremia, hypoalbuminemia and hypergammaglobulinemia are late findings.
  • Cortisol – level may be low if disseminate disease to the adrenal glands and has destroyed the adrenal cortices
  • Sputum – acid-fast smears are often positive. Therapy may have to be started empirically if clinical signs are suggestive of TB but smear is negative. Cultures for M. tuberculosis are usually positive within 6 weeks
20
Q

What does a skin PPD test for, and when and how do you interpret its results?

A

intradermal purified protein derivative. This test indicates exposure only, not active disease. TB skin test has been used for years as an aid in diagnosing latent tuberculosis infection (LTBI). Its result is a measurement of a hypersensitivity response.

Findings after 48 hours should be interpreted as:

  • Less than 5 mm (indurated area only) is negative
  • 5 mm or more is positive in HIV infected patient, recent TB exposure, immunocompromised patient or one with chest x-ray typical for TB
  • 10 mm or more is positive in health care workers, HIV negative injection drug users, residents in nursing homes, homeless shelters or recent immigrants.

15 mm or more is positive in any situation/person

21
Q

How is the Quantiferon Gold Test used for TB testing?

A
  • It is a serum test for diagnosis of TB, either latent or active, used instead of PPD.
  • The results are less subjective to reader bias and error and is quickly replacing the traditional testing in some practices although the CDC discourages the use for those patients among populations at low risk.
22
Q

What and when are chest x-ray changes seen for TB?

A

Infiltrates can be present in any portion of the lungs but are usually seen in the upper lobes or in the superior segments of the lower lobes. Cavitation is seen.

23
Q

What are the treatment options for patients who have suspected TB disease, smear positive, or have sputum culture results pending?

A

A four drug therapy is recommended for these patients:

  1. Isoniazid 300 mg PO daily – maximum 300 mg daily, with pyridoxine 50 mg PO, to prevent INH induced peripheral neuropathy.
  2. Rifampin (Rifadin/Rimactane) 600 mg PO daily (or 10 mg/kg daily, maximum 600 mg/dose).
  3. Ethambutol (Myambutol) 15-25 mg/kg PO each day (maximum 2.5 grams/dose) preceded by screening of color vision. Note: Ethambutol (Myambutol) may cause red/green color blindness as an adverse effect as well as changes in visual activity.
  4. Pyrazinamide 15-30 mg/kg PO in three divided doses (maximum 2 grams/dose)

Modification of regimen may be necessary if drug susceptibility studies demonstrate resistance to first line drugs. If isolate proves to be fully susceptible to INH and RIF then ethambutol may be dropped. Therapy is continued for 6-9 months. 6 months is generally sufficient for most patients, 9 months is indicated for HIV and other immunocompromised patients.

24
Q

What are the treatment options for non-adherent patients with TB?

A

Directly observed treatment may be initiated at 3 times per week, usually after 2 weeks of observed therapy, and is often done in the hospital. May follow one of three regimens:

  1. INH + rifampin + pyrazinamide + ethambutol daily for 2 months followed by INH + rifampin 2-3 times weekly for an additional 4 months, especially if susceptibility to INH/rifampin is noted
  2. INH + rifampin + pyrazinamide + ethambutol daily for 2 weeks. followed by the same agents weekly for 6 weeks, then INH + rifampin 2 times weekly for 4 months, if susceptibility to both is noted
  3. Thrice weekly dosing of INH + rifampin + pyrazinamide + ethambutol for 24 doses (8 weeks) followed by thrice weekly dosing of INH + rifampin for 54 doses (18 weeks)

Patients with advanced HIV (CD4 counts less than 100/ul) should be treated with daily or three times weekly therapy in both the initial and continuous phases of treatment.

25
Q

When are patients given prophylactic TB medications and what is given?

A
  • Treatment is considered for patients with (+) PPD and normal CXR, patients exposed to active TB when their own PPD is (-), HIV infected patients, and patients undergoing immunosuppressive therapy for other reasons.
  • INH 300 mg PO daily for 6 months – 1 year is given. Clinicians generally choose 9 months as the length of treatment is controversial. Pyridoxine 50 mg PO daily may also be given to prevent neuropathy.
26
Q

What is Idiopathic pulmonary fibrosis (IPF)?

A

IPF is a progressive disease that is seen in elderly patients (60-70yrs old), occurs more often in men than women, and usually carries a poor prognosis, especially if the disease proves to be progressing rapidly. The pathogenesis is complex and likely involves cycles of epithelial cell injury and dysregulated repair.

It is strongly linked to cigarette smoking

27
Q

What conditions should be ruled out prior to treating idiopathic pulmonary fibrosis?

A

Prior to treatment, the following conditions need to be ruled out:

  • Inhalation exposure
  • Autoimmune disorders
  • Chronic lung infections including TB, fungal infections of Histoplasmosis coccidioidomycosis and spread of prior known cancers
28
Q

What are some symptoms associated with idiopathic pulmonary fibrosis

A
  • Progressive dyspnea, begins gradually and worsens over months
  • Fatigue, fever myalgia’s and arthralgia’s can occur but are uncommon
  • Nonproductive cough

Specific questions of the patient regarding their exposure to the following is essential to evaluate their symptoms:

  • Inorganic dusts – Silica, asbestos
  • Organic dusts – chlorine, sulfur dioxide
  • Drugs including chemotherapeutic agents (e.g. bleomycin), antibiotics (nitrofurantoin and sulfa), gold salts (may have been used during treatment for RA), a miodarone (for cardiac arrhythmias).
  • Radiation to lung parenchyma
  • Risk factors for Pneumocystis carinii pneumonia
  • Known heart failure
29
Q

What physical exam finding is seen with idiopathic pulmonary fibrosis?

A

Rales (Velcro crackles) may be heard on auscultation

30
Q

What laboratory tests/diagnostic tests are used to confirm a idiopathic pulmonary fibrosis diagnosis?

A
  • Routine lab is not helpful in diagnosis. PPD should be obtained as well as fungal cultures including Histoplasma capsulatum, Coccidioides immitis and Blastomycosis. Familiarize yourself with your patient’s environment and fungi pertinent to that region.
  • CXR and High Resolution Chest CT - show changes in the lung parenchyma especially in the lower lobes. Other findings may include interstitial infiltrates (may resemble HF), nodules and cystic or honeycombing changes. Bronchiectasis may also be seen.
  • PFTs - Complete PFTs including spirometry, lung volumes, diffusing capacity and resting and ambulatory pulse oximetry should be obtained. Restrictive defects will be noted along with mild hypoxemia on ABG if obtained, CO2 retention in the later stages. This study will be helpful during the course of the disease to help establish a pattern and assess for additional lung changes: e.g. obstructive
  • Fiber optic bronchoscopy with biopsy - is safe and should be done to confirm diagnosis. Broncho alveolar lavage (BAL) obtains specimens for culture. If inadequate tissue is obtained with this procedure, patients will need to undergo open lung biopsy by cardiothoracic surgery, if they are stable enough.
31
Q

What are the treatment options for patients with idiopathic pulmonary fibrosis?

A

Prednisone 1 mg/kg/day for 12 weeks

  • Used for acute exacerbations
  • Patients report subjective relief – objective improvement on CXR on PFTs is generally not seen
  • Caution with the elderly due to side effects

Alternative treatments:

  • Cyclophosphamide (Cytoxan) 1 mg/kg/day OR
  • Azathioprine (Imuran) 3 mg/kg/day
  • Noninvasive use of positive airway pressure by mask may improve symptoms in a selected patient group
  • Patients with rapidly progressing disease or end stage disease should be referred to Palliative Care for counseling and end of life discussions
32
Q

What is Sarcoidosis?

A
  • The disease is characterized pathologically by the presence of non caseating granulomas and interstitial lung disease.
  • The systemic manifestations of the disease include lymphadenopathy, cardiac involvement, iritis, cutaneous lesions, arthritis, gastrointestinal involvement and potentially other organs.
  • Etiology is unknown and affects individuals worldwide.
33
Q

What are the symptoms associated with sarcoidosis?

A

Onset of symptoms is generally between the ages of 20-40 years of age:

  • Progressive dyspnea
  • slow or rapid onset
  • Nonproductive cough
  • Patients can also complain of chest discomfort
  • fatigue/malaise
  • weight loss

All vague symptoms. In approximately half of the cases, the disease is incidentally found by radiographic abnormalities.

34
Q

What are the physical exam findings seen in sarcoid

A
  • Physical exam may show normal lung exam or rales (Velcro crackles) if there is fibrosis present.
  • Wheezing may be present if there is endobronchial involvement or scarring of the lungs.
  • Digital clubbing is rare and typically associated with extensive lung sarcoidosis with pulmonary fibrosis.
  • A full skin exam should be done and patient asked to identify any new skin lesions (particularly around tattoos or scars).
35
Q

What are and describe the stages of sarcoidosis

A
36
Q

What lab findings/diagnostic findings are used to diagnose sarcoidosis?

A
  • PFTs - typically demonstrate a restrictive pattern with diffusing capacity of the lung for carbon monoxide reduced. ABG are generally normal initially may show hypoxemia, especially in the later stages of the disease. Carbon dioxide retention indicates severe disease.
  • PPD testing as well as fugal titers should be done
  • Bronchoscopy with biopsy and BAL – see pulmonary fibrosis
  • If other organs are involved, biopsy of these sites/organs should also be obtained – assess other organs with lab tests to include CBC, Calcium (sarcoidosis is associated with hypercalcemia) Liver function, kidney function, urinalysis. Patients should also be evaluated with EKG and opthalmologic exam.
  • CBC – anemia is uncommon initially, leukopenia, eosinophilia and thrombocytopenia can be seen.
  • Hypercalcuria is more common than hypercalcemia
  • Alkaline phosphatase – moderate elevation suggests diffuse granulomatous hepatic involvement
37
Q

What is the first line treatment for sarcoidosis?

A

Corticosteroids

  • Initial dose of prednisone: 0.3-0.6 mg/kg of ideal body weight (usually 20-40 mg/day) for 6 weeks
  • Maintenance: Prednisone 0.25-0.5 mg/kg (usually 10-20 mg) per day for at least 6-8 months. Total treatment will equal 1 year.
  • Some patients report improvement in less time – length of treatment in these patients is unknown. Far fewer show improvement in CXR or PFT findings
  • As usual, caution is advised in the elderly due to side effects
38
Q

What are some alternative therapies for sarcoidosis?

A

Alternative treatments-may be considered in selected patients when they are refractory to first line treatment with corticosteroids.

  • Cyclophosphamide (Cytoxan) 25-50 mg/day (not to exceed 150 mg/day) Has potential for toxicity so should be reserved as a third choice or third line drug. OR
  • Azathioprine (Imuran) 2 mg/kg or 50 m daily
  • Methotrexate 7.5 mg PO daily – can be increased by 2.5 mg every two weeks with a maximum dose of 10-15 mg per week – and chloroquine 250-750 mg PO daily or every other day also have been used as alternative treatment for sarcoidosis