Aidan: /* Management by organ system */

2020-09-07T19:56:53Z

Management by organ system

Aidan: Text replacement - "== Clinical Presentation" to "== Clinical Manifestations"

2020-08-02T10:50:50Z

Text replacement - "== Clinical Presentation" to "== Clinical Manifestations"

← Older revision		Revision as of 10:50, 2 August 2020
Line 23:		Line 23:
	* More common in African-Americans		* More common in African-Americans

	== Clinical ~~Presentation~~ ==		== Clinical Manifestations ==

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

Maintenance script: Imported from text file

2020-07-04T01:17:55Z

Imported from text file

New page

== 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 Presentation ==

=== 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. [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:Respirology]]

Sarcoidosis - Revision history

Aidan: /* Management by organ system */

Aidan: Text replacement - "== Clinical Presentation" to "== Clinical Manifestations"

Maintenance script: Imported from text file