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Sarcoidosis is a systemic inflammatory disorder characterized by the presence of

noncaseating granulomatous inflammation in involved organs. The lungs, eyes,
skin, joints, lymph nodes, and upper respiratory tract are usually affected.


The incidence and prevalence of sarcoidosis is characterized by heterogeneity. It

appears to be 3 to 4 times more common in Afro Americans than Caucasians. The
female gender is more affected than the male one. The highest prevalence was
noted in northern Europe with a peak between 20 and 40 years of age.


The aetiology of sarcoidosis is unknown. The following factors (presumably

triggers/antigens) were noted in studies aiming the cause of sarcoidosis:
infectious agents: Mycobacterium tuberculosis, Propionibacterium acnes,
Human herpes virus. following transplantation ( eg. bone marrow, stem
cell, cardiac)
Kveim-Siltzbach reagent
genetic background: angiotensin converting enzyme genotypes, HLA-
B12,13, 27, 35; HLA-DR3,4,5
CD4+/CD8+ T cells the Th1/Th2 balance plays an important role in the
organisation of granulomas, the auto-resolution and/or progression of
the disease.
Cytokines Il-2 (proliferation or redistribution of the activated
lymphocytes population and B cell differentiation ), Il-6, Il-8 (disease
process), Il-15 (CD4+ T cell proliferation), Il-12, Il-18 (stimulate IFN-)
The characteristic of sarcoidosis is the non-caseating granuloma that can be
found in any organ. Sarcoidosis is mediated primarily by the CD4+ T cells (T
helper cells Th1). The initial lesion, at the level of the lung, is alveolitis. So
beside the CD4+ T cells, the alveolar macrophages (inflammation!!!) produce and
express pro-inflammatory cytokines such as: Il-12, Il-18, IFN-, TNF .
The granuloma is characterized by:
central epitheliod cells, activated monocytes-macrophages, and
multinucleated giant cells
periphery suppressor CD8+ T cells (Th2)
It is presumed that the extrapulmonary manifestations in sarcoidosis are due to
the migration of activated T cells through the blood and lymphatic vessels to
other tissues.


1. Pulmonary involvement
90% of cases
50% detected incidentally on chest radiography hilar
chest pain
wheezing (may be present)
self limited
spontaneous remission
2. Extrathoracic manifestations
cutaneous involvement: erythema nodosum, subcutaneous
nodules, plaques, paules, lupus pernio
ocular involvement: uveal tract, lacrimal gland and conjunctiva;
optic neuritis, pars planis (attention!!! differential diagnoses with
multiple sclerosis); uveitis is the most common ophthalmologic
arthralgia/arthritis, tenosynovitis, myopathies
unilateral facial nerve palsy
heart involvement: pericarditis, left ventricular dysfunctions,
diabetes mellitus hypothalamic-pituitary axis involved
3. Lofgrens syndrome is characterized by:
hilar adenopathy
acute arthritis/arthralgias
erythema nodosum
typically involves the ankles and knees
also associates: fever, myalgia and weight loss
excellent response to corticotherapy
90% remission rate
4. Heefordts syndrome:
anterior uveitis (iridocyclitis or iritis)
parotid gland enlargement
facial palsy


elevated ESR (non specific)

anemia secondary to chronicity
leukopenia, lymphopenia, eosinophilia, thrombocytopenia is rare
elevated angiotensin converting enzyme (ACE) is normal in acute
hypercalciuria, hypercalcemia consequence of enhance production of
1,25 dihydrocholecalciferol due to elevated levels of ACE
serum alkaline phosphatase might be elevated hepatic involvement


Chest radiographic stages

Stage 0
Stage 1
bilateral hilar adenopathy
Stage 2
bilateral hilar adenopathy with pulmonary infiltrates
Stage 3
pulmonary infiltrates with lung insufficiency

Chest High Resolution Computed Tomography (HRCT) the lesions are

localised in the mid-upper zones of the lungs:
lymphadenopathy (hilar and mediastinal)
ground glass phenomenon
parenchymal masses or bands
thickening of the bronchovascular bundles

Bone radiography - bone lesions usually occurs in the proximal and middle
cystic bone lesions (more cystic than sclerotic)
lytic bone lesions heads
sclerotic lesions
focal bone lesions

Pulmonary function tests

restrictive pattern - diffusing capacity for carbon monoxide
sometimes obstructive pattern endobronchial sarcoidosis

Bronchoalveolar lavage (BAL)

reduce number of CD8+ T cells
elevated CD4 to CD8 ratio
lymphocytosis is not specific
neutrophils > 2% or eosinophils > 1% - usually is not sarcoidosis!!!


It is suggested if the following are present:

clinical and imagistic manifestations
biopsy non-caseating granuloma
exclusions of other diseases such as: tuberculosis, Crohns disease,
lymphomas, bacterial, viral, parasitic or fungal infections etc.

Prognosis is negative influenced by:

Afro American race
onset after 40 years old
lupus pernio (be attentive it may be tuberculosis: TB)
chronic uveitis
chronic hypercalcaemia
progressive pulmonary involvement
nasal mucosal involvement
cystic bone lesions
cardiac involvement

The management of sarcoidoisis depends on the clinical manifestations lung or

extrapulmonary symptoms.

Pulmonary involvement treatment

progressive radiographic changes
deterioration of lung function
worsening of pulmonary symptoms


Corticosteroids are used for its suppressing effects on inflammatory response.

It is believed to reduce the progression of fibrosis and acts on pulmonary
sarcoidosis as well. There are not stipulated protocols concerning the dose of
corticosteroids in pulmonary sarcoidosis. The oral therapy can be started with
0.5 to 1 mg/kg ideal body weight for 4 to 6 weaks and after that tapered by 5 to
10mg every 4 to 8 weeks until the maintenance dose is reached (0.25mg/kg ideal
body weight for 6 to 12 months).

Immunosuppresive drugs
In case of a non response to corticosteroids the following drugs may be used:

Lung transplantation end stage pulmonary fibrosis.

Extrapulmonary involvement treatment

resistant symptomatic disease
resistant ophthalmic manifestations
progressive chronic pulmonary disease
cardiac involvement
bone and joint destruction


Corticosteroids similar doses as in pulmonary involvement.

Methotrexate (MTX) is used as a substitute for corticosteroids in acute

sarcoidosis (except pulmonary manifestations). MTX is often associated with
liver fibrosis and progressive interstitial lung changes (especially in sarcoidosis).

Colchicine can be effective for acute arthritis.

Antimalars (chloroquine/hydroxichloroquine) cutaneous and/or

musculoskeletal disease (+/- low dose of methotrexate).

Immunosuppresive drugs
In case of a non response to corticosteroids the following drugs may be used:
Azathioprine cutaneous disease
Cyclophosphamide cardiac and neurosarcoidosis


Sarcoidosis is characterized by a non-caseating granuloma, that can affect

any organ.
The most common manifestations are pulmonary involvemet hilar
adenopathy (bilateral) and anterior uveitis.
An increase CD4 to CD8 ratio in BAL fluid strongly supports the diagnosis
of sarcoidosis.
ACE can be normal in acute sarcoidosis.
Corticosteroids are the cornerstone therapy for active sarcoidosis.

1. 1. Stone JH, A Clinicians Pearls and Myths in Rheumatology, Springer
2009, 493: 23-26, ISBN: 978-1-84800-933-2
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