Restrictive Lung Disease

Aka: Restrictive Lung Disease, Restrictive Pulmonary Disease, Restrictive Lung Function

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II. Causes: Chest Wall

  1. Ankylosing Spondylitis
  2. Kyphosis
  3. Morbid Obesity
  4. Scoliosis

III. Causes: Medications

  1. Amiodarone
    1. Pulmonary fibrosis
  2. Methotrexate
    1. Pulmonary fibrosis
    2. Hypersensitivity Pneumonitis
  3. Nitrofurantoin
    1. Acute Nitrofurantoin Pulmonary Toxicity

IV. Causes: Interstitial Lung Disease

  1. See Interstitial Lung Disease
  2. Asbestosis
  3. Eosinophilic Pneumonia
  4. Hypersensitivity pneumonitis
  5. Idiopathic Pulmonary Fibrosis
  6. Sarcoidosis
  7. Silicosis (late stage)

V. Causes: Neuromuscular

  1. Amyotrophic Lateral Sclerosis (ALS)
  2. Guillain-Barre Syndrome
  3. Muscular Dystrophy
  4. Myasthenia Gravis

VI. Diagnosis: Pulmonary Function Test

  1. See Pulmonary Function Test
  2. Criteria
    1. FEV1 to FVC Ratio >0.7 (or >LLN or for children>85% of predicted) AND
    2. FVC < LLN (or for age 5 to 18 years old, <80% of predicted)
  3. Assess Severity
    1. FVC over 80%: Normal
    2. FVC over 60%: Mild Restrictive Lung Disease
    3. FVC over 40-50%: Moderate Restrictive Lung Disease
    4. FVC under 50%: Severe Restrictive Lung Disease
  4. Additional testing
    1. Diffusing lung capacity for Carbon Monoxide (DLCO)
    2. DLCO to alveolar volume ratio
    3. Expiratory Reserve Volume
  5. Precaution
    1. Note that mixed obstructive-Restrictive Lung Disease can also occur

VII. References

  1. Johnson (2014) Am Fam Physician 89(5): 359-66 [PubMed]

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Ontology: Restrictive lung disease (C0085581)

Definition (NCI) Decreased lung volume and inadequate ventilation due to parenchymal lung disorders (e.g., interstitial pulmonary fibrosis) or extrapulmonary disorders (e.g., scoliosis). Patients present with shortness of breath and cough.
Concepts Disease or Syndrome (T047)
SnomedCT 36485005
English restrictive lung disease, restrictive lung disease (diagnosis), diseases lung restrictive, disease lung restrictive, Restrictive Lung Disease, Restrictive lung disease, Restrictive lung disease (disorder), Restrictive lung disease, NOS
Spanish enfermedad restrictiva de pulmón, neumopatía restrictiva (trastorno), neumopatía restrictiva
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Lung function restrictive (C0429676)

Concepts Finding (T033)
SnomedCT 142195009, 165022002
English Lung function restrictive (situation), Lung function restrictive, Lung function restrictive (finding)
Spanish función pulmonar restrictiva (hallazgo), función pulmonar restrictiva
Derived from the NIH UMLS (Unified Medical Language System)

Ontology: Restrictive pulmonary disease (C0877013)

Concepts Disease or Syndrome (T047)
Italian Malattia polmonare restrittiva
Japanese 拘束性肺疾患, ｺｳｿｸｾｲﾊｲｼｯｶﾝ
Czech Restrikční plicní onemocnění
English restrictive pulmonary disease, pulmonary restrictive disease, diseases pulmonary restrictive, Restrictive pulmonary disease
Hungarian Restrictív tüdőbetegség
Portuguese Doença pulmonar restritiva
Spanish Enfermedad pulmonar restrictiva
Dutch restrictieve longziekte
French Affection pulmonaire restrictive
German restriktive Lungenerkrankung
Derived from the NIH UMLS (Unified Medical Language System)

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