Pulmonary Manifestation of Systemic Disease Flashcards
Differential Dx mneumonic
- VINDICATE
- Vascular
- Infection
- Neoplasm
- Drugs
- Inflammatory/idiopathic/iatrogenic
- Congenital
- Autoimmune
- Trauma
- Endocrine/metabolic
Pulmonary anatomic differential includes:
•Airways •Alveoli •Interstitium •Vascular •Pleura •Chest wall •Extrathoracic
ALS + pulmonary manifestations
- progessibve neurogenerative disease –> weakness
- •Chronic aspiration due to uncoordinated pharyngeal muscle movement (“bulbar dysfunction”)
- •Recurrent pneumonia
- •Respiratory muscle weakness
- –Inadequate ventilation
- –Nocturnal hypoventilation
- –Weak cough
Dx of pulmonary disease associated w/ALS
- CXR: right middle lobe infiltrates from chronic aspiration + low lung volumes
- PFTs:
- Restrictive pattern: airflow symmetrically reduced; reduced TLC
- •Reduced maximal inspiratory pressure (MIP) and reduced maximal expiratory pressure (MEP)
- •Reduced maximum voluntary ventilation (MVV)
- •Generally normal DLCO initially; prolonged hypoventilation results in atelectasis and shunt.
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Neuromuscular diseases (acute, relapsing, chronic)
- Acute: Guillain-Barre (GBS)
- Relapsing: Multiple sclerosis (MS), myasthenia gravis (MG)
- Chronic, progressive: ALS
Neuromuscular diseases anatomical locations
- Spinal cord: MS, infarct, trauma, tetanus, transverse myelitis, tumors
- Motor nerves –ALS, polio, GBS, Phrenic nerve injury
- NM junction –Botulism, MG, Lambert Eaton, organophosphates
- Muscles –HypoK, hypophos, muscular dystrophy, polymyositis, thyroid disease
RA + pulmonary manifestation
- RA = systemic autoimmune disease w/symmetric inflammatory arthritis
- •Pleuritis
- •Pleural Effusion
- •Airway obstructions
- •Interstitial Lung Disease (UIP > NSIP)
- •Pulmonary Hypertension
- •Drug reactions (esp methotrexate, sulfasalazine)
- •Pulmonary infections due to immunosuppression
Connective tissue disease w/pulmonary manifestation
- SLE
- RA
- •Systemic sclerosis (scleroderma)
- •Sjogren’s disease (autoimmune destruction of exocrine glands)
- •Dermatomyositis/polymyositis
- •Mixed connective tissue disease
- •Ankylosing spondylitis
Systemic diseases that lead to diffuse alveolar hemorrhange + renal disease
- Granulomatosis with polyangiitis (formerly Wegener’s)
- Microscopic polyangiitis
- Churg-Strauss Syndrome
- Goodpasture’s Syndrome (Anti-GBM disease)
- Systemic Lupus Erythematosus
- Systemic Sclerosis (Scleroderma)
- Henoch-Schonlein Purpura
- Cryoglobulinemia
Pulmonary manifestations of IBD
- •Tracheobronchitis
- •Bronchiectasis ==> obstructive
- •Bronchiolitis ==> obstrutive
- •Pleural Effusion ==> restrictive
- •Interstitial Lung Disease ==> restrictive
- •Pulmonary embolism
- •Drug complications
- •Infections
Common types of Obstructive disease
–Tracheobronchitis
–Bronchiectasis
–Bronchiolitis
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Common Types of Restrictive Disease
–Pleural Effusion
–Interstitial Lung Disease
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Pulmonary Manifestation of Sickle Cell Disease
- •Infection
- •Embolic phenomena due to bone marrow infarction and fat emboli
- •Infarction caused by in-situ thrombosis
- •Hypoventilation due to rib and sternal infarctions
- •Pulmonary Edema due to excessive hydration
- •Pulmonary hypertension
- •Chronic lung disease
Pulmonary complications in HIV (infectious)
- Bacterial pneumonia
- Tuberculosis
- Pneumocystis jirovecii (PCP)
- Fungal pneumonia
- Viral pneumonia
Pulmonary complications in HIV (noninfectious)
- Kaposi’s sarcoma
- Non-Hodgkin’s lymphoma
- Lung cancer
- Emphysema
- ILD
- Pulmonary HTN
- Effusions –Parapneumonic –TB –Malignant; esp lymphoma