This document provides information on sarcoidosis, including: 1) It is a chronic granulomatous disease characterized by noncaseating granuloma formation of unknown cause, often affecting the lungs and lymph nodes. 2) It has a higher prevalence in African Americans and Japanese populations. The disease often affects people aged 20-40 and has a female predominance. 3) Granulomas form as a result of an abnormal cellular immune response potentially triggered by infectious agents. This leads to the formation of noncaseating granulomas containing giant cells. 4) The disease can have acute or chronic presentations and involve multiple organs, with lungs and lymph nodes most commonly affected. Corticost

1. Sarcoidosis
DEAPCIT

2. definition
• Idiopathic, multi-system, chronic
granulomatous disease characterised by
noncaseating granuloma formation

3. epidemiology
• Prevalence = 50/100 000
• Common in African Americans, not in Africans
living in Africa
• Common in Japan, uncommon in rest of Asia
• Clusters in health workers
• Age = 20 – 40 yrs
• F>M

4. aetiology
• Genetic predisposition + environmental trigger
• Genetic
– Most common allele found in sarcoidosis is HLA-B8
• ? Infectious Ag triggers CMI response & starts
disease process
– Many organisms have been linked to sarcoidosis:
• M Tb
• Mycoplasma species; Borrelia burgdorferi;
Propionibacterium acnes
• Fungi - Histoplasma and Cryptococcus species
• Viruses - EBV, CMV, HSV, hepatitis C virus, and rubella

5. pathophysiology
• May begin with lung alveolitis
• This overstimulated local cellular immune
response may involve overactive TH-cells, which
release chemotactic factors to attract monocytes
from blood to lungs & nose, resulting in chronic
inflammation
• Macrophages transform into giant cells, which
then form epithelioid cells, which form
granuloma
• Mature epithelioid granulomas release ACE

6. pathology
• Giant cells, epithelioid cells & macrophages in noncaseating granulomas, which have fewer lymphocytes
surrounding = ‘naked tubercle’
• Fibrinoid necrosis
• Giant cells have prominent inclusion bodies (e.g. asteroids,
Schaumann)
• Asteroid bodies are star-shaped eosinophilic structures
• Schaumann bodies are round or oval, laminated structures,
which are usually calcified at the periphery
• Granulomas are referred to as ‘naked’ because they only
have a sparse lymphocytic infiltrate at the margins
• Giant cells are larger & have more nuclei than in TB

7. Schaumann Bodies
non-caseating granulomas
Asteroid bodies

8. Clinical (2 patterns)
•
Acute
– Sudden, with remission in 2 years
– More common in Caucasians
– May present with Lofgren’s Syndrome
•
•
•
•
Bilateral hilar LN
Ankle arthritis
Erythema nodosum
•
Constitutional symptoms
–
–
Nodular, erythematous eruption on anterior LL
Type IV hypersensitivity reaction
Chronic
– Gradual onset, with relapses
– More organ-specific symptoms, which result from irreversible fibrosis

9. Course of disease
• Asymptomatic (30-50%)
• Constitutional Symptoms (30%)
• Organ-Specific Symptoms (20-40%)
– Lung LN (90%, 30% symptomatic)
– LN = 30%
– splenomegaly in 40 – 80%
– Skin = 25%
– Eye = 20 – 30%
– Neuro = < 10%
– Cardiac = 5%
– GIT
– Renal

10. ENT
• Occur in 9%-15% of patient with sarcoidosis
• Most common with
– Cervical LN
– Parotidomegaly
– Facial nerve palsy

11. Laryngeal = 1 – 5%
– Hoarseness, dyspnoea /
stridor
– Rarely painful
– Early stage = whitebrown mucosal nodules
– Later stage = pale,
oedematous epiglottis
• Lesser changes in
remaining supraglottis
(arytenoids, aryepiglottic
folds, false VC)
• Glottis rarely involved

12. • Sinonasal = 10%:
– NOT often presenting
symptom
– Nasal obstruction,
rhinorrhoea, PND
– Anosmia, recurrent sinusitis,
epiphora
– Firm, raised subcutaneous
yellow papule on nose, may
become confluent
– Diffuse nasal crusting & thick
mucous
– Submucosal nodules on
septum & IT, with dry friable
mucosa
• Sarcoidosis interferes with
submucosal gland function,
causing dryness
– Septal perforations & polyps
– Rarely, granulomas may
erode through palate
• Ear - rare
– Pinna = nodules
– ME = ET obstruction from NP
involvement
– SNHL < 1% = sudden,
asymmetric, fluctuating, high
or low frequency
– Permanent loss or complete
recovery
– Vestibular involvement
recovers less well than
acoustic
– Vestibulo-acoustic
involvement is often
concurrent with facial palsy &
uveitis

13. • Oropharyngeal
– Tonsils in 2%
– Pharyngeal & oesophageal
stenosis may result from fibrosis
• Neurosarcoid = 5%
– CN > CNS symptoms (e.g.
pituitary involvement causes DI)
– Basal Granulomatous Meningitis
• Causes multiple cranial
neuropathies by involvement of
nerves with perivascular
lymphocytic infiltrates in IAC,
causing fluctuating ischaemia
• Facial > optic > glossopharyngeal
> vagus > cochlear nerves
– TV channels + 8
• Fluctuating ischaemia of facial
nerve caused by perivascular
lymphocytic infiltration
proximal to geniculate ganglion,
leads to reversible neuropraxia –
often sudden, bilateral palsies,
which resolve
• Cervical LN = 50%
– FNA has high yield
• Parotidomegaly = 6%
– Usually bilateral & FNA is
useful
– Heerfordt’s disease
(Uveoparotid Fever)
• Uveitis, parotitis, facial
paralysis, fever

14. Investigation
• Biopsy
– Submucosal nodules or transbronchial lung, LN, lip gland
– histopath, AFB, culture
• Bronchoalveolar lavage = elevated CD4:CD8 ratio
• Haematologic
– Ca (serum & urine) = elevated
– Serum ACE
• Correlates with active disease in 40 – 90%
• Used in monitoring
• CXR (respiratory symptoms are most common)
– Bilateral hilar lymphadenopathy & lung infiltrates
• Kveim Test
– Part of a spleen from a patient with known sarcoidosis is injected into skin of a
patient suspected to have the disease
– If granulomas are found (4–6 weeks later), the test is positive

15. Management
• MEDICAL
– Steroids = mainstay
– Systemic steroids in eye / organ involvement
– Topical steroids in skin / eye disease
– Inhaled steroids in URT / LRT disease
– MTX / Azathioprine
• Surgical

16. • Laryngeal
– Steroids
– Intralesional steroid injection
– If unresponsive, consider low-dose RTH
– Tracheostomy or RTH / laser to obstructing lesions