Sarcoidosis: Difference between revisions
From IDWiki
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| − | == |
+ | ==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=== |
| + | {| class="wikitable" |
||
| − | {| |
||
| − | ! |
+ | !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<br />Hydroxychloroquine 400 mg/day<br />Thalidomide 100-150 mg/day<br />Methotrexate 10-15 mg/week |
|- |
|- |
||
| − | | |
+ | |Skin |
| − | | |
+ | |Plaques or nodules |
| − | | |
+ | |Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day |
|- |
|- |
||
| − | | |
+ | |Skin |
| − | | |
+ | |Erythema nodosum |
| − | | |
+ | |NSAID |
|- |
|- |
||
| − | | |
+ | |CNS |
| − | | |
+ | |Cranial nerve palsies |
| − | | |
+ | |Prednisone 20-40 mg/day |
|- |
|- |
||
| − | | |
+ | |CNS |
| − | | |
+ | |Intracerebral involvement |
| − | | |
+ | |Prednisone 40 mg/day<br />Azathioprine 150 mg/day<br />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<br />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<br />Hydroxychloroquine 400 mg/day |
|} |
|} |
||
from the [NEJM sarcoidosis review article][1] |
from the [NEJM sarcoidosis review article][1] |
||
| − | == |
+ | ==Further Reading== |
| − | * |
+ | *[1]: Iannuzzi MC, Rubicki BA, and Teirstein AS. [https://doi.org/10.1056/NEJMra071714 Sarcoidosis]. ''N Engl J Med''. 2007 Nov 22;357(21):2153-65. |
| − | * |
+ | *Costabel U and Hunninghake GW. [https://erj.ersjournals.com/content/14/4/735.long ATS/ERS/WASOG statement on sarcoidosis]. ''Eur Respir J''. 1999 Oct;14(4):735-7. |
[[Category:Rheumatology]] |
[[Category:Rheumatology]] |
||
Latest revision as of 15:56, 7 September 2020
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.
