Pulmonary

• "P-I-P-E"
• Pulmonary fibrosis
• ILD
• Emphysema
• Pulmonary hypertension

• "A-AP"
• Polycythemia
• Alveolar hemorrhage
• Asthma

• Rifampin
• Dilantin
• Phenobarbital
• Carbamazepine
• Smoking

• "CAM-Flouro-PAZ"
• Acyclovir
• Cimetidine
• Macrolides
• Fluoroquinolones
• Propranolol
• Allopurinol
• Zafirlukast

• Medical instability-- respiratory or cardiac arrest, severe respiratory acidosis (pH <7.1), hemodynamic instbality, cardiogenic shock, UGI bleed.
• Unable to protect airway -- excessive secretions, severe bulbar dysfunction, excessive somnolence or encephalopathy.
• Mechanical issues -- recent facial trauma or surgery, upper airway obstruction.

• Pulmonary fibrosis (reticular opacities) concentrated at the bases of the lungs.
• Shaggy heart border
• May have associated pleural thickening or plaques, calcification of plaques and/or diaphragm
• "AsBasetosis" because the infiltrates are in the bases ( vs. at the upperlobes for silicosis)

"Diaphragmatic calcification" is pathognomonic.

• chest pain and dyspnea
• painful exudative effusion
• pain is a dull ache

• upper lobe distribution of fibrosis
• egg-shell calcification in hila (10%)

• From exposure to coal dust
• Melanoptysis
• Focal centrilobular emphysema
• 1 of 3 ILDs with obstructive pattern on PFT

• alloy in metals or alone, seen in nuclear weapons, electronics, aerospace, ceramics, metal recycling, dental prostheses, alloy machining, defense industries, automotive.
• CXR is difficult to differentiate from sarcoidosis other than by history

a cell-mediated immune response that can occur and persist from even a trivial exposure with a latency of 1-10 years.

2-6%

The berylliom lymphocyte-proliferation test is a blood test that can detect beryllium sensitivity

corticosteroids

• Diffuse pulmonary fibrosis in 80%
• Pulmonary involvement more severe in diffuse systemic sclerosis and less so in the CREST variant
• Pulmonary hypertension*

• A subset of patients with SSc develop rapidly progressive ILD during the first 2 years of their disease
• Spirometry and diffusion testing should be considered mandatory for all patients at the time of diagnosis.
• Patients with SSc should have annual lung function testing early in disease.
• If DLCO <50% of predicted, need TTE to monitor for pulmonary HTN.
• If pulmonary HTN present, consider warfarin therapy.

• If none of the following is present:
• Pleural/serum protein > 0.5
• Pleural/serum LDH > 0.6
• Absolute LDH value > 2/3 normal value for serum, usually > 200 IU.

**If any one of the criteria above is met, the fluid is exudative.

• CHF (500,000/year)
• Cirrhosis (50,000/year)
• Nephrosis
• Hypoalbuminemia

• Check two additional tests:
• Pleural cholesterol less than 45
• Pleural fluid/serum albumin gradient 1.2
• *if two above are met, then it is transudative.

LDH and protein

• Description of the fluid
• Glucose level
• Amylase level
• Cell count with differential
• Cytology and microbiology
• Pleural fluid pH

>7.6

above 7.2

• It is undetermined status when pH >7 but <7.2. Consider drainage if:
• glucose <50 mg
• LDH >1000 IU

Tumor, Trauma, Thromboembolism

• Generally large, >50% hemithorax
• Quick to reaccumulate
• Generally lymphocytic WBC's
• Often bloody

• Three separate taps will improve yield to 90%.
• If malignancy is highly suspected, VATS procedure is next step.

*Yield is not significantly increased by blind needle biopsy so these are not done anymore.

This is guided by the fluid pH, which reflects the metabolic activity of the tumor burden.

>7.2 -- tube thoracoscopy with chemical scleroses or thoracoscopy with pleural abrasion or talc insufflation.

<7.2 -- palliative care.

• AFB recovered in only 10%, but yield can be increased with up to 3 taps.
• Culture for AFB is positive in 65%.
• Pleural biopsy and culture together identify 85%.
• Blind pleural biopsy may be helpful.

• Three biopsy samples will increase yield to 80%
• shows caseating granuloma
• if sent for TB, yield increases.

• Adenosine deaminase levels, > 50 U/L is 94% sensitive and 90% specific.
• Levels < 45 U\L 100% sensitive and specific for nontuberculous etiology.
• Mesothelial cells are rarely present.

• Follow carefully
• CXR every 6 months
• Approximately 80% of these will resolve, however, 20% will turn out to be cancer.

• pneumonia
• pancreatitis
• PE
• peritonitis

TB or malignancy

Rheumatoid arthritis

Cancer or empyema

TB

• loud 2nd heart sound
• tricuspid regard
• RV heave

Normal spirometry and volumes but with decreased DLCO.

schistosomiasis

Echo followed by right heart cath.

• MAP -- >25 mmHg at rest, but increases to >30 mmHg during exercise.
• Symptomatic when >45 mmHg

Useful to rule out a secondary cause of pulmonary hypertension, and PCWP has to be normal.

• Try CCB in cath lab (important to do it there due to risk of CV collapse). Only 1 in 4 will respond. If they respond, continue CCB.
• Prostacyclin by IV infusion pump
• Bosentan
• Sildenafil
• Heart-lung transplant

Complications and tachyphylaxis

Hepatic insufficiency. Need to monitor LFTs monthly.

• Anticoagulants
• Diuretics
• Oxygen

Secondary pulmonary hypertension, probably due to ASD.

• Homocysteinmia
• Prothrombin gene mutation
• Factor V Leident (heterozygous)
• Factor V Ledient (homozgoust)
• Increased factor VIII

What shoal you suspect?

• Signs of RV overload
• T wave inversion V1-V4
• S1Q3T3
• Transient RBBB
• Atrial fibrillation
• S1S2S3

thrombolytics unless contraindicated.

4%

• If symptomatic or if >25-40%, then chest tube
• If chest tube is needed, then also do pleurodesis since recurrence rate is 40-50%.
• If chest tube not needed for first PTX, most will have pleurodesis after the next recurrence.
• No pleurodesis = no air travel, scuba

PCP pneumonia

• Common in COPD, ILD, CF
• Tx is Chest tube and pleurodesis

• This suggests bronchopleural fistula.
• Requires surgical consultation for stapling and pleurodesis to prevent recurrences.

Cessation of airflow for at least 10 seconds

> 10 apneic spells an hour during sleep study

Obstructive - continued respiratory effort during the absence of airflow

Central - absence of effort and airflow.

More than 15-20/hr requires treatment

• Weight loss for mild cases, approx. 10% of weight
• Nasal CPAP or BiPAP for moderate/severe (if unable to lose weight.
• Avoid sedatives, ETOH
• If severe -- need tracheostomy

• acidification with acetazolamide
• supplemental O2
• CPAP

• Severe daytime hypersomnolence
• Cataplexy
• Sleep paralysis
• Hallucinations when drowsy

Polysomnography and Multiple Sleep Latency Test (MSLT) checks hypersomnolence and frequency of REM

Stimulants and REM suppression by tricyclic antidepressants and SSRI

dopaminergic agonists and narcotics

Periodic limb movement disorder -- contraction of the anterior tibialis muscle during sleep.

• Cough -- most frequent
• Dyspnea
• Hemoptysis

35%

• Can watch only if:
• there is no growth in 2 years when compared to an old film
• there is patterned calcification in the lesion.

• Tumor is the rumor
• Tissue is the issue
• Heal with steel
• When in doubt, cut it out

• CT scan assures that lesion is solitary and helps define if calcium is present.
• FOB not helpful if lesion is < 3 cm in size.
• PET scan may predict cancer probability, but it's role is not yet determined.
• Surgery (don't bother with FNA)

Stage IIIa and under

• T4 = direct invasion of mediastinum/vasculature
• N3 = contralateral mediastinum or hilar lymph nodes or and scalene or supraclavicular nodes
• M1 = distal mets

• Subclass of adenocarcinoma
• 1/3 never smokers
• 2:1 ratio of women to men
• represents only 2-3% of all NSCLCs
• may mimic pneumonia in appearance
• -persistent pneumonia
• Bronchorrhea is typically seen
• -frothy sputum
• -salty taste (due to high K+)
• Inclined to stay within the lung and is likely to spread to other organs.
• Has a better overall survival when matched stage for stage with other NSCLCs.

• Presents with shoulder pain (a shoulder film may be shown)
• May have Horner syndrome
• Most commonly squamous cell
• Usually operable
• Use MRI to evaluate brachial plexus and plan surgery

• Caused mostly by primary lung tumors
• Paraneoplastic syndrome does not indicate metastatic spread
• Paraneoplastic syndrome does not indicate unresectability

Squamous cell

Hyperprolactinemia

• HPO/Clubbing
• Marantic endocarditis

• Hypercalcemia
• Cerebellar ataxia

• Diffuse alveolar hemorrhage
• "ELKS" involvement -- ENT, Lung, Kidney, Sinusitis
• Sinusitis
• Cavitary lung lesions
• Glomerulonephritis
• Arthritis and cutaneous leukoytoclastic vasculitis

• cANCA present 96% of the time (PR3-ANCA)
• ANCA is 90% sensitivity and specificity

Open lung bx or bx nasal membrane -- not kidneys

Open lung or bx nasal membrane -- not kidneys.

Early mortality is 37%

focal segmental necrotizing GN

ESR, ANCA, serial DLCOs, urine sediment

linear deposition of anti-glomerular basement membrane antibodies in lung and kidney (Antibody against alpha-3 chain of Type IV collagen.

lung and kidney only, no ENT (as opposed to Wegener's)

Wegener and Goodpasture's

Necrotizing granulomatous vasculitis

60-80%

5-10%

• High-dose corticosteroids effective for Diffuse Alveolar Hemorrhage
• However, not effective for nephritis, so combined with cyclophosphamide and plasma exchange (usually for 2 weeks).

Diffuse alveolar hemorrhage (DAH)

50%

<30% glomeruli affected, and worst with >70%

• Eosinophilic pneumonia
• Eosinophils in blood and sputum
• Migratory peripheral infiltrates on CXR
• Minimal respiratory symptoms
• Often idiopathic
• Generally benign and self-limiting

Drugs and parasites, i.e. Strongyloides

• Most common PIE in the U.S.
• Occurs in middle-aged females
• Very high ESR
• Eos in blood and sputum
• Subacute illness: cough, wheezing, fever, night sweats, weight loss

Radiographic negative of pulmonary edema

Chronic Eosinophilic Pneumonia

• Histiocytosis X
• Eosinophilic granulomatosis
• Langerhans cell histiocytosis

Lymphangiomatosis

• Young male, smoker
• Proliferation of Langerhans cells from the bone marrow and mature eosinophils
• Cystic spaces in the upper lung fields
• 50% develop PTX in course of illness

Clue is recurrent pneumothorax and diabetes insipidus (because the Langerhans cells go to the hypothalamus)

• Diagnosis by FOB/BAL
• Langerhans cells on lung biopsy or BAL (contains X-body or Birbeck granule)

• smoking cessation
• supportive/trial steroids

• Premenopausal women
• There is immature smooth muscle proliferation - lympatics, bronchial, alveolar, vascular
• Honeycomb throughout
• Cysts lead to PTX
• Chylous pleural effusion

• Hormonal manipulation
• Exposure to estrogen is not recommended. This includes the use of contraceptive pills containing estrogen and hormone replacement therapy (HRT)
• Rapamycin is known to inhibit the proliferation of smooth muscle cells
• Lung transplantation

• Crescent sign -- cavity in the lung after inhaling aspergillus. Pt not asthmatic. "Ball of yarn" moves when change in position.
• Massive hemoptysis is usual presentation

• Bronchial arterial embolization (BAE) temporarily controls the bleeding
• Does not provide definitive treatment
• Stabilizes the patient and decreases the need for emergency surgical resection.

• treat as outpatient
• Preferred antibiotics are:
• Macrolides
• Fluoquinolones
• Doxycyline

IV beta-lactam (cefotaxime or claforan) or ceftriaxone plus a macrolide level, or a fluoroquinolone alone.

If no response to antibiotics in 72 hours. Approximately 1-15% develop treatment failure. Almost 6% manifest rapidly progressive and life-threatening pneumonia.

• age >65 years
• previous Beta-lactam rx in last 3 months
• previous fluoroquinone therapy
• alcoholism
• co-morbidities
• immunosuppressive illness or therapy

Mycoplasma

Pneumococcus

Staphylococcus

Klebsiella

Pneumoccus/Staph

PCP

Nocardia

CMV

Histoplasmosis

coccidiomycosis

Blastomycosis

Cryptococcosis

Sporothrichosis

Mucormycosis

Actinomysis.

• age >65 years
• previous B-lactam rx in last 3 monks
• previous fluorquinolone therapy
• alcoholism
• co-morbidities
• immunosuppressive illness or therapy

Histoplasmosis

Coccidiomycosis

Blastomycosis

Cryptococcosis

Sporotrichosis

Mucormycosis

Actinomycosis

• It has a thick refractory cell wall, broad based budding yeast that makes it look like a bowling pin, and can be associated with a skin rash (dermatitis), likes to go to the bone and the brain.
• It's favorite reservoir is the hunting dog, i.e. the beagle.

• BAL
• If not successful, think: CMV if post-transplant, or Aspergillus if granulocytopenic (<1000 PMNs)

Think TB

Think PCP

Think MAI

• HIV infection
• Household contact with a person who has active TB
• NH patients with fibrotic changes on chest radiograph c/w old TB
• Organ transplants, immunosuppressed (greater than 15 mg/day prednisone for at least 1 month)

• recent (within 5 years) immigrants from high prevalence countries
• IV drug users
• residents or employees of high-risk settings such as jails, nursing homes, shelters, and hospitals
• those who have conditions associated with a high risk of disease after infection
• Persons with immunosuppressive medical conditions other than HIV infection, including diabetes, renal insufficiency, gastrectomy, silicosis, corticosteroid therapy (>15mg/day of prednisone for 2 weeks), and other immunosuppressive therapy and conditions.

• 9-month regimen (300 mg/day) considered optimal regardless of HIV status
• Contact with INH-resistant TB: 4-months daily rifampin

• A positive AFB smear suggests the disease
• Only a positive culture for M. tuberculosis confirms active disease.

• R-I-P-E
• Rifampin
• Isoniazid
• Pyrazinamide
• Ethambutol

Twice-weekly doses of isoniazid and rifampin

Consult a subspecialist

• Hypoxemia with hypocapnia
• Hypoxemia (V/Q mismatch) responds to low-flow oxygen
• Increased A-a gradient
• Respiratory alkalosis is expected
• ABGs that look normal during an acute asthma attack signal respiratory muscle fatigue.

• Low tidal volumes
• Enough PEEP to keep PaO2 > 60 mmHg with FiO2 < 60%.

Keep FiO2 < 60%

• Keep Peak pressures < 45 cm
• Keep Plateau pressures < 30 cm

Use tidal volume 6 cc/kg

Protective ventilation -- lower tidal volumes allow us to minimize the contribution of mechanical ventilation to ongoing lung injury and decrease relative mortality by 22-24% (from 40 to 32%).

low, low, high

High, low, high

Normal, High, Low

18

>2.2

Severe mitral regurgitation, and in the setting of a recent MI, may indicate papillary muscle dysfunction.

Equalization of the diastolic.

Increase of O₂ saturation from RA to RV.

CVP > PCWP

• Hypercarbia with hypoxemia
• V/Q mismatch physiology
• Acute exacerbation of COPD

• NIF < -20 cm
• Vital capacity < 1 liter (have them blow out)

This indicates inadequate respiratory muscle strength.

Two hours, and if no improvement, intubate.

It is not always needed in Type II respiratory failure. It is only needed to improve their oxygenation to a safer FiO₂.

Change PaO₂ by adjusting FiO₂ or PEEP

Change PaCO₂ by adjusting rate and tidal volume

The pressure needed to overcome airway resistance and lung stiffness.

The amount of pressure to keep the lung inflated. No air flow

the difference between peak pressure and plateau pressure.

• It means that Airway Resistance has increased and the patient needs a bronchodilator, suctioning, evaluate for a mucus plug, or a kink in the ET tube.
• The differential diagnoses are: mucus plugging, bronchospasm, airway secretions, narrow ET-tube.

• If the difference (Airway Resistance) is the same as before the change occurred, the patient does not need a bronchodilator but may need lasix, or check for abdominal distention.
• Differential diagnoses are: tension pneumo, atelectasis, multilobar pneumonia, pulmonary edema, bronchial intubation

• Weaning Standard Criteria:
• Able to oxygenate in FiO2 of 40%
• Resting minute ventilation < 10 liters
• NIF > -25 cm H₂O
• Maximal tidal volume > 10 cc/kg

These criteria are specific but not sensitive. Failure to meet one or more does not preclude success.

It is the new criteria for weaning. You put the patient on CPAP mode, convert tidal volume to liters and do the math: f/VT

f/VT <100 breaths/L then 80% chance of weaning success

f/VT > 100 breaths/L then 95% chance of weaning failure

• Effective hand washing
• Use of protective gowns and gloves
• Semirecumbent positioning
• Avoidance of large gastric volumes
• Oral (non-nasal) intubation
• Continuous subglottic suctioning
• Postural changes
• Proper maintenance of ventilator circuits
• Sedation holidat

Critical Illness Neuromuscular disease. This is a complication of sepsis and multiorgan failure, occurs in approximately 25% of patients in ICU longer than 7 days. EMG will show decreased muscle and nerve action potentials. Check the DTRs. If those are absent, your diagnosis is confirmed.

• Glucocorticoids
• Malnutrition
• Nondepolarizing muscle agents
• Poor glucose control

• Acute hemolytic reaction (10%)
• Febrile reactions (44%)
• Allergic reactions (45%)

RBC

WBC

antigens in the plasma

Those who are IgA deficient.

6-8 hours

• Drotrecogin alfa (activated) administered in accordance with a standardized hospital policy
• Low-dose steroids administered for septic shock in accordance with a standardized hospital policy
• Glucose control maintained greater than the lower limit of normal, but less than 150 mg/dl
• Inspiratory plateau pressures maintained less than 30 cm H₂O

• Symptoms -- < 2 days/week
• Nighttime awakenings -- < 2 X /month
• SABA use for symptom control -- <2 days/week
• interference with normal activity -- none
• lung function -- Normal FEV1 between exacerbation, FEV1 >80% of predicted, FEV1/FVC normal

>2 days/week but not daily

Daily

throughout the day

<2x / month

3-4 x / month

> 1 x / week

often 7 x / week

>2 days/week but not more than 1 x / day

daily

several times a day

• normal FEV₁ between exacerbations FEV₁ > 80% of predicted
• FEV₁/FVC normal

• FEV₁ >80% of predicted
• FEV₁/FVC normal

• FEV₁ > 60% but < 80% of predicted
• FEV₁/FVC reduced 5%

• FEV₁ < 60% of predicted
• FEV₁/FVC reduced > 5%

SABA PRN

low-dose ICS

• Lung volume reduction surgery involves resecting parts of the lung to reduce hyperinflation. This type of surgery makes respiratory muscles more effective by improving their mechanical efficiency and improves expiratory flow rates by increasing the elastic recoil pressure of the lung. Because this procedure is expensive and high-risk, it is recommended only in carefully selected patients. The surgery is most beneficial in patients with severe, predominantly upper lobe disease and a large amount of mildly emphysematous or normal middle and lower lung.
• Patients with severe COPD who have had maximal medical treatment, including pulmonary rehabilitation, and have FEV1 greater than 20% of predicted, DLCO greater than 20% of predicted, and predominant upper lobe emphysema are likely to benefit from lung volume reduction surgery.

• loud P2
• fixed split S2
• pulmonic flow murmur
• tricuspid regurgitation

Chest radiographs are usually normal in early disease but may show enlarged pulmonary arteries, right atrium, and right ventricle. Electrocardiographs in these patients may show right ventricular strain or hypertrophy.

Pulmonary function studies in patients with PAH usually reveal an isolated decreased DLCO in the setting of normal airflow and lung volumes (excluding restrictive lung disease).

Lung transplantation should be considered in patients hospitalized with COPD exacerbation complicated by hypercapnia (PCO2 greater than 50 mm Hg) and patients with FEV₁ not exceeding 20% of predicted and either homogeneous disease on high-resolution CT scan or DLCO less than 20% of predicted who are at high risk of death after lung volume reduction surgery.

• objective measure
• can be used to assess the presence and severity of excessive daytime sleepiness.
• A multiple sleep latency test can be performed if daytime sleepiness persists in a patient who is consistently able to use CPAP set at an optimal pressure. It will objectively confirm her complaints of sleepiness but will not aid in identifying its cause.

A carcinoid tumor is the most likely tumor in a young person who has never smoked and who has evidence of endobronchial obstruction. Bronchial carcinoid is a slow growing tumor that originally was classified as an adenoma but has been reclassified as a malignant neoplasm because of its ability to metastasize. Most bronchial carcinoid tumors are located in proximal airways and cause symptoms by either obstructing an airway or bleeding. Common presenting symptoms include cough or wheeze, hemoptysis, and recurrent pneumonia in the same pulmonary lobe. The carcinoid syndrome is caused by systemic release of vasoactive substances such as serotonin, and the most typical features include cutaneous flushing and diarrhea. Bronchial carcinoids are not commonly associated with the carcinoid syndrome because of their relatively small amount of serotonin production.

Diagnosis is suggested by the presence of airway hyperresponsiveness and confirmed when cough resolves with a trial of inhaled albuterol.

• 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) is generally done after CT of the chest.
• Lung cancers are metabolically active and take up FDG avidly, whereas benign pulmonary nodules do not.
• FDG-PET is most helpful in patients with a nodule 1 cm or larger and an intermediate probability of malignancy.

• Paraneoplastic neurologic disorders are associated with systemic malignancy but are not related to direct tumor involvement or to other toxic or metabolic, infectious, or vascular complications of cancer.
• Paraneoplastic sensory neuropathy is commonly associated with lung carcinoma (particularly small cell carcinoma) but may develop in patients with other malignancies.
• The neurologic syndrome precedes the diagnosis of cancer in most patients.
• Paraneoplastic sensory neuropathy manifests with progressive paresthesias, profound sensory ataxia, and multimodality sensory loss.
• The ANNA-1 antibody is the most common antibody present in a sensory neuropathy associated with small cell carcinoma.

should be considered if patients have persistent hypoxemia and/or hypercapnia with a pH less than 7.35 and a PCO2 greater than 45 mm Hg and a respiration rate greater than 25/min despite maximal medical therapy.

• The development of severe, anterior chest pain during therapeutic thoracentesis is virtually diagnostic of an unexpandable lung with the development of significant negative intrapleural pressure. The anterior chest pain is quickly relieved by allowing air entry into the pleural space through the thoracentesis needle or catheter. In this situation, the patient is best managed with an indwelling catheter. The patient and his family are instructed to drain the pleural fluid when breathlessness ensues and to discontinue drainage immediately when anterior chest pain develops.
• Oxygen supplementation is indicated if the estimated in-flight PO2 will be less than 55 mm Hg

• The data suggest that there is a moderate increase in risk for postoperative pulmonary complications among current smokers.
• There is also evidence suggesting that at least 2 months of smoking cessation reduces postoperative pulmonary risk.
• A brief period of abstinence does not improve perioperative pulmonary outcomes, and smokers who tried to decrease cigarette use shortly before surgery are more likely to develop a postoperative pulmonary complication than those who continued smoking.

Bosentan, an endothelin receptor antagonist, is effective in improving symptoms in patients with PAH; however, it is teratogenic and is contraindicated for use during pregnancy.

Medication overuse headache is generally defined as a headache for more than 15 days per month and the use of acute headache medication on more than 10 days per month.

• HAPE is a form of nancardiogenic pulmonary edema due to leakage of fluid and hemorrhage into the alveolar spaces. The most effective preventive measure for HAPE is an appropriately gradual axcent to altitude (not greater than 300 to 500 m (984 to 1640 ft).
• Nifedipine is used to prevent and to treat HAPE.

• Acetazolamide is used as prophylaxis for periodic breathing related to high altitude and acute mountain sickness (AMS) but is not indicated for HAPE.
• Dexamethasone is used for the prevention and treatment of AMS; it is not generally considered as a prophylactic agent for HAPE.

• rhabdomylsis
• compartment syndromes
• DIC
• pulmonary edema
• ATN

In patients with severe hypothermia (temperature less than 30.0 °C [86.0 °F]), active internal rewarming measures using warmed intravenous fluids, warm and humid oxygen, peritoneal lavage, and extracorporeal rewarming should be considered.

• Diagnosis of RB-ILD requires the clinical context of a patient with a significant smoking history. Respiratory bronchiolitis, which is also called smoker’s bronchiolitis, is common among smokers and is thought to be responsible for the reduction in air flow in young, otherwise healthy smokers.
• Pulmonary function testing typically shows a mixed obstructive–restrictive pattern with a slightly reduced diffusing capacity for carbon monoxide but in some cases, testing may be normal or associated with only an increase in the residual volume.
• RB-ILD is causally linked to inhalation of tobacco smoke, whereas cryptogenic organizing pneumonia, idiopathic pulmonary fibrosis, and nonspecific interstitial pneumonia are not.

The propofol infusion syndrome is a rare and often fatal syndrome originally described in critically ill children undergoing long-term propofol infusion at high doses. The syndrome has recently been reported in adults, mostly in patients with acute neurologic illnesses or acute inflammatory diseases complicated by severe infections or even sepsis and who are receiving catecholamines and/or corticosteroids in addition to propofol. The main features of the syndrome consist of cardiac failure, rhabdomyolysis, severe metabolic acidosis, and renal failure associated with hyperkalemia. Central nervous system activation with production of catecholamines and corticosteroids, and systemic inflammation with cytokine production are priming factors for cardiac and peripheral muscle dysfunction. High-dose propofol, but also supportive treatments with catecholamines and corticosteroids act as triggering factors. At the subcellular level, propofol impairs free fatty acid utilization and mitochondrial activity. The syndrome can be lethal if not identified early, and caution should be exercised when using prolonged (more than 48 h) propofol sedation at doses greater than 75 µg/kg/min, particularly in patients with acute neurologic or inflammatory illnesses. In these cases, alternative sedative agents should be considered immediately, and monitoring of the plasma levels of troponin I, creatine kinase, and myoglobin should be undertaken.

Corticosteroids and cyclophosphamide are indicated to induce disease remission in patients with anti-GBM antibody disease. Because relapse rarely occurs, cyclophosphamide may be discontinued after 3 to 6 months. In addition, pulmonary hemorrhage is a medical emergency requiring immediate plasmapheresis to remove the causative antibody.

patients with severe Wegener granulomatosis who underwent plasma exchange in addition to immunosuppressive therapy with daily cyclophosphamide and high-dose oral methylprednisolone had a 24% risk reduction for progression to ESKD compared with those who received immunosuppressive therapy alone.

Diagnosis of Wegener granulomatosis or microscopic polyangiitis involves antibody assays and kidney biopsy. Most patients are ANCA positive. Antiproteinase-3 (anti-PR3) antibodies are usually detected in patients with Wegener granulomatosis, whereas antimyeloperoxidase (anti-MPO) antibodies are usually found in those with microscopic polyangiitis. Serial ANCA testing should not be used to monitor disease activity or to guide treatment decisions in patients with Wegener granulomatosis.

To establish a diagnosis of Wegener granulomatosis, microscopic polyangiitis, polyarteritis nodosa, or Churg-Strauss syndrome with kidney involvement, kidney biopsy must reveal the presence of a necrotizing crescentic glomerulonephritis or necrotizing vasculitis of microscopic vessels such as the small arteries, arterioles, capillaries, or venules.