Sarcoidosis
From IDWiki
Pathophysiology
- Thought to be related to abnormal T-cell activation
Differential Diagnosis
- Hilar lymphadenopathy
- Sarcoidosis
- Infection: TB, fungal, HIV, mycoplasma
- Malignancy: lymphoma
- Others
Scadding Classification
- Stage I: Bilateral hilar lymphadenopathy (70% resolve)
- Stage II: Above, with interstitial lung disease (50% resolve)
- Stage III: Interstitial lung disease alone (15% resolve)
- Stage IV: Fibrotic, "burnt out" lungs (0% resolve)
Risk Factors
- Women more than men (2:1)
- More common in African-Americans
Clinical Manifestations
Extrapulmonary disease
- Skin: Erythema nodosum and lupus pernio, and others
- Cardiac: 40% of patients, though only 5-10% are symptomatic
- CNS: 5-10%, multiple presentations
- Eyes: 10% of all uveitis cases, usually bilateral
- Hypercalcemia
- Nephrocalcinosis
Diagnosis
- Evidence of granulomatous inflammation (often on BAL or EBUS) without infection
- Either lung involvement or multiorgan involvement
Investigations
- Labs
- CBC, lytes, creatinine, calcium, liver panel
- Imaging
- High-res CT scan
- Other
- PFTs: most commonly restrictive with decreased DLCO, but can show combined restriction-obstruction, or rarely any other pattern. Often normal.
- EKG for cardiac involvement
- Eye exam for uveitis
Management
- Only treat if symptomatic, as many will resolve spontaneously
- Spontaneous remission depends on Scadding stage (I 70%, II 50%, III 15%, IV 0%)
- Prednisone 20-40mg daily for 8-12 weeks, then taper
- Add vitamin D and calcium if serum calcium is low
- Can still use bisphosphates for bone protection
- Second-line steroid-sparing agents include
- Methotrexate
- Azathioprine, leflonamide, MMF, hydroxychloroquine, thalidomide
- TNG-alpha inhibitors are last line
Management by organ system
| Organ | Clinical Features | Treatment |
|---|---|---|
| Lungs | Dyspnea w FEV1 or FVC <70% | Prednisone 24-40 mg/day |
| Lungs | Cough, wheeze | Inhaled corticosteroid |
| Eyes | Anterior uveitis | Topical corticosteroid |
| Eyes | Posterior uveitis | Prednisone 20-40 mg/day |
| Eyes | Optic neuritis | Prednisone 20-40 mg/day |
| Skin | Lupus pernio | Prednisone 20-40 mg/day Hydroxychloroquine 400 mg/day Thalidomide 100-150 mg/day Methotrexate 10-15 mg/week |
| Skin | Plaques or nodules | Prednisone 20-40 mg/day Hydroxychloroquine 400 mg/day |
| Skin | Erythema nodosum | NSAID |
| CNS | Cranial nerve palsies | Prednisone 20-40 mg/day |
| CNS | Intracerebral involvement | Prednisone 40 mg/day Azathioprine 150 mg/day Hydroxychloroquine 400 mg/day |
| Heart | Complete heart block | Pacemaker |
| Heart | Ventricular fibrillation or tachycardia | AICD |
| Heart | Decreased LVEF <35% | AICD and prednisone 30-40 mg/day |
| Liver | Cholestatic hepatitis with constitutional symptoms | Prednisone 20-40 mg/day Ursodiol 15 mg/kg per day |
| MSK | Arthralgias | NSAID |
| MSK | Granulomatous arthritis | Prednisone 20-40 mg/day |
| MSK | Myositis or myopathy | Prednisone 20-40 mg/day |
| Calcium | Kidney stones, fatigue | Prednisone 20-40 mg/day Hydroxychloroquine 400 mg/day |
from the [NEJM sarcoidosis review article][1]
Further Reading
- [1]: Iannuzzi MC, Rubicki BA, and Teirstein AS. Sarcoidosis. N Engl J Med. 2007 Nov 22;357(21):2153-65.
- Costabel U and Hunninghake GW. ATS/ERS/WASOG statement on sarcoidosis. Eur Respir J. 1999 Oct;14(4):735-7.
