Updated: 10/11/2018

Other Restrictive Etiologies

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Collagen Vascular Disease
  • Systemic lupus erythematosus
    • pleuritis + pleural effusions
  • Rheumatoid arthritis (rheumatoid lung disease)  
    • pleuritis + pleural effusions
    • also see pulmonary fibrosis (restrictive pattern)
    • bilateral, diffuse appearance on chest radiograph
      • progress to honeycomb lung in severe disease
    • presentation
      • gradual onset dyspnea
      • end-inspiratory rales at lung base
      • lung biopsy shows patchy interstitial lymphoid infiltrate into walls of alveolar units
    • NOT associated with rheumatoid nodules in the lung
      • this finding is associated with Caplan's syndrome (see Pneumoconioses )
  • Systemic sclerosis (scleroderma)
    • may also present with CREST syndrome (a variant of scleroderma)
    • interstitial fibrosis due to deposition of collagen
      • triggered by increased TGF-beta secreting T-cells accumulating in lungs 
      • mainly involve capillaries and small arterioles
    • may lead to pulmonary hypertension and cor pulmonale
Iatrogenic Causes
  • Drug-associated 
    • anti-cancer agents
      • bleomycin/busulfan, methotrexate, nitrosourea, and cyclophosphamide
    • anti-arrhythmics
      • amiodarone
  • Radiation-induced
    • post-treatment pneumonitis (1-6 months following)
    • associated with fever, dyspnea, and pleural effusions
Occupational Causes
  • Silo filler's disease
    • hypersensitivity pneumonitis to nitrogen oxide gases released by plant matter
  • Byssinosis
    • hypersensitivity penumonitis to textile dusts (including cotton, hemp, and linen)
  • Farmer's lung 
    • hypersensitivity pneumonitis to Saccharopholyspora rectivirgula (thermophilic actinomyes)
      • found in moldy hay
      • type III hypersensitivity reaction with antigen-antibody complex depositing in lung
      • can become type IV hypersensitivity reaction with chronic exposure
Idiopathic Pulmonary Fibrosis
  • Introduction
    • most common group of idiopathic interstitial pneumonia
    • chronic alveolitis with no known cause
    • persistent inflammation results in fibrosis (due to fibroblast proliferation) and cyst formation that is most prominent in subpleural regions (lower lung predominantly) 
    • typically seen in men 40-70 years old
    • requires transplant at most advanced stages
  • Presentation 
    • dyspnea on exertion most common symptom
    • gradual onset of dry cough
      • subpleural cystic enlargement = "honeycomb" lung
    • diagnosis can be made by HRCT if underlying causes excluded
      • surgical lung biopsy shows usual interstitial pneumonia (UIP)
Pulmonary Alveolar Proteinosis
  • Idiopathic pathology where proteinaceous material fills the avleoli
    • can be caused by primary (idiopathic) or secondary (infectious/malignancies) causes
  • Pathophysiology
    • defect in clearance (macrophages) or production of surfactant 
      • macrophage defect and atypical infections sometimes seenin this pathology
 

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Questions (8)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.PL.52) A 61-year-old male presents to your office with fever and dyspnea on exertion. He has been suffering from chronic, non-productive cough for 1 year. You note late inspiratory crackles on auscultation. Pulmonary function tests reveal an FEV1/FVC ratio of 90% and an FVC that is 50% of the predicted value. Which of the following would you most likely see on a biopsy of this patient's lung? Review Topic

QID: 100855
1

Subpleural cystic enlargement

21%

(16/78)

2

Charcot-Leyden crystals

22%

(17/78)

3

Hyaline membranes

38%

(30/78)

4

Arteriovenous malformations

3%

(2/78)

5

Anti-GBM antibodies

14%

(11/78)

M1

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PREFERRED RESPONSE 1

(M1.PL.141) A 61-year-old male visits his primary care physician because of dyspnea that has worsened over several months. His other medical problems include essential hypertension and rheumatoid arthritis, but he refuses to take medication for either. He denies using alcohol, tobacco, or other drugs. Physical examination reveals bilateral end-inspiratory rales at the lung bases. Chest radiograph is shown in Figure A. Which of the following is the most likely explanation for this patient’s symptoms? Review Topic

QID: 100944
FIGURES:
1

Chronic bronchitis

0%

(0/2)

2

Tension pneumothorax

0%

(0/2)

3

Pulmonary embolism

0%

(0/2)

4

Pulmonary fibrosis

100%

(2/2)

5

Mitral stenosis

0%

(0/2)

M1

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PREFERRED RESPONSE 4
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