Pulmonary Complications of Rheumatologic Diseases

Sjögren's syndrome:

Keratoconjunctivitis sicca, xerostomia, polyarthritis

90% female

speckled ANA + 80%

rheumatoid factor + 95%

Pulmonary disease:

1. airways disease:

bronchiectasis

inspisated secretions

obstructive PFTs

2. interstitial lung disease

UIP

Cellular interstitial pneumonitis

BOOP

LIP

pseudolymphoma

 

Systemic lupus erythematosus

1. acute lupus pneumonitis

2. diffuse alveolar hemorrhage

3. pleurisy

4. pulmonary fibrosis

5. pulmonary vascular disease

6. bronchiolitis

7. respiratory muscle dysfunction

Rheumatoid arthritis

1. pleurisy

2. pulmonary vascular disease

3. rheumatoid nodules

4. upper airway obstruction

5. BO and BOOP

6. Interstitial lung disease

 

Scleroderma

1. pleural disease

2. interstitial lung disease

3. pulmonary vascular disease

4. aspiration pneumonia

Polymyositis

1. aspiration pneumonia

2. interstitial lung disease

3. respiratory muscle dysfunction

 

Mixed connective tissue disease

1. pleural disease

2. pulmonary vascular disease

3. aspiration pneumonia

4. respiratory muscle dysfunction

5. interstitial lung disease

Lymphocytic interstitial disease

1. Sarcoidosis

2. Hypersensitivity pneumonitis

3. Lymphoma

4. Lymphocytic interstitial pneumonitis

5. Diffuse lymphoid hyperplasia

6. Angioimmunoblastic lymphadenopathy

7. Pseudolymphoma

8. Castleman's syndrome

9. Angiocentric immunoproliferative lesiona (lymphomatoid granulomatosis)

 

Pulmonary infiltrates in connective tissue disease:

1. cellular interstitial pneumonitis: RA, PM, MCTD

2. LIP: Sjögren's syndrome

3. UIP: all connective tissue disease

4. BOOP: RA, MCTD

5. Diffuse alveolar hemorrhage: SLE, Sjögren's syndrome, PM, MCTD, RA

6. Bronchiolitis: RA, Sjögren's syndrome

7. Nodules: RA, Sjögren's syndrome

 

Autoimmune serology

SSA (anti-Ro): Sjögren's syndrome (70-80%), SLE (40%)

Associated with pulmonary disease

Anti-double stranded DNA: SLE (50%), very specific

Anti-Sm:: SLE (30%): very specific

Anti-RNP: SLE (30%), MCTD (>95%)

SSB (anti-La): SLE (15%), Sjögren's syndrome (50-70%)

Anti-Scl: PSS (40-70%), CREST (10-20%)

Anti-centromere: CREST (80%), PSS (30%)

Anti-Jo1: polymyositis (18-25%)

Anti-mitochondrial: primary biliary cirrhosis (90-95%)

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