Sarcoidosis: Difference between revisions

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== Pathophysiology ==
==Pathophysiology==


* Thought to be related to abnormal T-cell activation
*Thought to be related to abnormal T-cell activation


== Differential Diagnosis ==
==Differential Diagnosis==


* Hilar lymphadenopathy
*Hilar lymphadenopathy
** Sarcoidosis
**Sarcoidosis
** Infection: TB, fungal, HIV, mycoplasma
**Infection: TB, fungal, HIV, mycoplasma
** Malignancy: lymphoma
**Malignancy: lymphoma
** Others
**Others


== Scadding Classification ==
==Scadding Classification==


# Stage I: Bilateral hilar lymphadenopathy (70% resolve)
#Stage I: Bilateral hilar lymphadenopathy (70% resolve)
# Stage II: Above, with interstitial lung disease (50% resolve)
#Stage II: Above, with interstitial lung disease (50% resolve)
# Stage III: Interstitial lung disease alone (15% resolve)
#Stage III: Interstitial lung disease alone (15% resolve)
# Stage IV: Fibrotic, "burnt out" lungs (0% resolve)
#Stage IV: Fibrotic, "burnt out" lungs (0% resolve)


== Risk Factors ==
==Risk Factors==


* Women more than men (2:1)
*Women more than men (2:1)
* More common in African-Americans
*More common in African-Americans


== Clinical Manifestations ==
==Clinical Manifestations==


=== Extrapulmonary disease ===
===Extrapulmonary disease===


* Skin: Erythema nodosum and lupus pernio, and others
*Skin: Erythema nodosum and lupus pernio, and others
* Cardiac: 40% of patients, though only 5-10% are symptomatic
*Cardiac: 40% of patients, though only 5-10% are symptomatic
* CNS: 5-10%, multiple presentations
*CNS: 5-10%, multiple presentations
* Eyes: 10% of all uveitis cases, usually bilateral
*Eyes: 10% of all uveitis cases, usually bilateral
* Hypercalcemia
*Hypercalcemia
* Nephrocalcinosis
*Nephrocalcinosis


== Diagnosis ==
==Diagnosis==


* Evidence of granulomatous inflammation (often on BAL or EBUS) without infection
*Evidence of granulomatous inflammation (often on BAL or EBUS) without infection
* Either lung involvement or multiorgan involvement
*Either lung involvement or multiorgan involvement


== Investigations ==
==Investigations==


* Labs
*Labs
** CBC, lytes, creatinine, calcium, liver panel
**CBC, lytes, creatinine, calcium, liver panel
* Imaging
*Imaging
** High-res CT scan
**High-res CT scan
* Other
*Other
** PFTs: most commonly restrictive with decreased DLCO, but can show combined restriction-obstruction, or rarely any other pattern. Often normal.
**PFTs: most commonly restrictive with decreased DLCO, but can show combined restriction-obstruction, or rarely any other pattern. Often normal.
** EKG for cardiac involvement
**EKG for cardiac involvement
** Eye exam for uveitis
**Eye exam for uveitis


== Management ==
==Management==


* Only treat if symptomatic, as many will resolve spontaneously
*Only treat if symptomatic, as many will resolve spontaneously
** Spontaneous remission depends on Scadding stage (I 70%, II 50%, III 15%, IV 0%)
**Spontaneous remission depends on Scadding stage (I 70%, II 50%, III 15%, IV 0%)
* Prednisone 20-40mg daily for 8-12 weeks, then taper
*Prednisone 20-40mg daily for 8-12 weeks, then taper
** Add vitamin D and calcium if serum calcium is low
**Add vitamin D and calcium if serum calcium is low
** Can still use bisphosphates for bone protection
**Can still use bisphosphates for bone protection
* Second-line steroid-sparing agents include
*Second-line steroid-sparing agents include
** Methotrexate
**Methotrexate
** Azathioprine, leflonamide, MMF, hydroxychloroquine, thalidomide
**Azathioprine, leflonamide, MMF, hydroxychloroquine, thalidomide
** TNG-alpha inhibitors are last line
**TNG-alpha inhibitors are last line


=== Management by organ system ===
===Management by organ system===


{| class="wikitable"
{|
! Organ
!Organ
! Clinical Features
!Clinical Features
! Treatment
!Treatment
|-
|-
| Lungs
|Lungs
| Dyspnea w FEV1 or FVC <70%
|Dyspnea w FEV1 or FVC <70%
| Prednisone 24-40 mg/day
|Prednisone 24-40 mg/day
|-
|-
| Lungs
|Lungs
| Cough, wheeze
|Cough, wheeze
| Inhaled corticosteroid
|Inhaled corticosteroid
|-
|-
| Eyes
|Eyes
| Anterior uveitis
|Anterior uveitis
| Topical corticosteroid
|Topical corticosteroid
|-
|-
| Eyes
|Eyes
| Posterior uveitis
|Posterior uveitis
| Prednisone 20-40 mg/day
|Prednisone 20-40 mg/day
|-
|-
| Eyes
|Eyes
| Optic neuritis
|Optic neuritis
| Prednisone 20-40 mg/day
|Prednisone 20-40 mg/day
|-
|-
| Skin
|Skin
| Lupus pernio
|Lupus pernio
| Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day<br />Thalidomide 100-150 mg/day<br />Methotrexate 10-15 mg/week
|Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day<br />Thalidomide 100-150 mg/day<br />Methotrexate 10-15 mg/week
|-
|-
| Skin
|Skin
| Plaques or nodules
|Plaques or nodules
| Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day
|Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day
|-
|-
| Skin
|Skin
| Erythema nodosum
|Erythema nodosum
| NSAID
|NSAID
|-
|-
| CNS
|CNS
| Cranial nerve palsies
|Cranial nerve palsies
| Prednisone 20-40 mg/day
|Prednisone 20-40 mg/day
|-
|-
| CNS
|CNS
| Intracerebral involvement
|Intracerebral involvement
| Prednisone 40 mg/day<br />Azathioprine 150 mg/day<br />Hydroxychloroquine 400 mg/day
|Prednisone 40 mg/day<br />Azathioprine 150 mg/day<br />Hydroxychloroquine 400 mg/day
|-
|-
| Heart
|Heart
| Complete heart block
|Complete heart block
| Pacemaker
|Pacemaker
|-
|-
| Heart
|Heart
| Ventricular fibrillation or tachycardia
|Ventricular fibrillation or tachycardia
| AICD
|AICD
|-
|-
| Heart
|Heart
| Decreased LVEF &lt;35%
|Decreased LVEF &lt;35%
| AICD and prednisone 30-40 mg/day
|AICD and prednisone 30-40 mg/day
|-
|-
| Liver
|Liver
| Cholestatic hepatitis with constitutional symptoms
|Cholestatic hepatitis with constitutional symptoms
| Prednisone 20-40 mg/day<br />Ursodiol 15 mg/kg per day
|Prednisone 20-40 mg/day<br />Ursodiol 15 mg/kg per day
|-
|-
| MSK
|MSK
| Arthralgias
|Arthralgias
| NSAID
|NSAID
|-
|-
| MSK
|MSK
| Granulomatous arthritis
|Granulomatous arthritis
| Prednisone 20-40 mg/day
|Prednisone 20-40 mg/day
|-
|-
| MSK
|MSK
| Myositis or myopathy
|Myositis or myopathy
| Prednisone 20-40 mg/day
|Prednisone 20-40 mg/day
|-
|-
| Calcium
|Calcium
| Kidney stones, fatigue
|Kidney stones, fatigue
| Prednisone 20-40 mg/day<br />Hydroxychloroquine 400 mg/day
|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 ==
==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.
*[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.
*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 19:56, 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

  1. Stage I: Bilateral hilar lymphadenopathy (70% resolve)
  2. Stage II: Above, with interstitial lung disease (50% resolve)
  3. Stage III: Interstitial lung disease alone (15% resolve)
  4. 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

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