Transcript
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Sarcoidosis
Dr. Ali A. Al-nasiry MRCP
Assistant professor
Hashemite university
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Sarcoidosis
, commonlyA multisystem granulomatous disorder
and usually presenting withaffecting young adultspulmonary,bilateral hilar lymphadenopathy
skin or eye lesionsandinfiltration
Aetiology & epidemiology
- Sarcoidosis is a common disease of unknowncause, often detected by routine chest X-ray
,atypical mycobacteriumThere is some links with-occupational,,Barr virus-Epstein,fungus
environmental factorsand othersocialgenetic,
- Prevalence in the UK is 19 in 100000 of population
- It is common in USA but uncommon in Japan
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Pathology- Sarcoid granulomas consist of focal accumulation
of epithelioid cells, macrophages and lymphocytes,
mainly T cell
- There is depressed cell-mediated reactivity to
tuberculin and other antigen such as Candida
albicans.- There is overall lymphopenia( T lymphocytes are
low but B cells are slightly increased).
- There is increase in alveolar lavage cells mainly
CD4 helper cells- Transbronchial biobsy shows infiltration of the
alveolar walls with leucocytes mainly T cells
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Sarcoid granuloma
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Clinical features
- The peak incidence is in the third and fourth
decade.- Females are more affected than males
- The most common presentation is with respiratory
symptoms or abnormal X-ray (50%)
- Fatigue or weight loss (5%)- Peripheral lymphadenopathy (5%)
- Fever (4%)
- Neurological presentations are rare
- Chest X-ray may be negative in 20% of non-
respiratory cases
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Bilateral hilar lympnadenopathy
- This is a characteristic feature of sarcoidosis- Usually asymptomatic and simply detected
on routine chest X- ray.
- Occasionally it presents with dull chest ache,
malaise and mild fever- Although it look like there is no infiltration in lung
fields on chest X- ray ,but evidence from CT scan,
transbronchial biobsies and bronchalveolar lavage
indicates that the lung paranchyma is nearly
always involved
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The differential diagnosis
- lymphoma-- which usually affect other sites- pulmonary tuberculosis here the hilar
enlargement is asymmetrical
- carcinoma of the bronchus with malignant
spread to hilar lymph nodes---- again here
the enlargement is asymmetrical
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Pulmonary infiltration- This type of sarcoidosis may be so progressiveleading to increasing effort dyspnoea and eventuallycorpulmonale and death
- The chest X- ray shows mottling shadows in themid-zones, proceeding over time to generalizedfine nodular shadows, then to widespreadpulmonary line shadows which indicate fibrosis.
A honeycomb appearance can occasionally occur.
- Pulmonary function tests show restrictive defect.
- Diffrential diagnosis is from TB, pneumoconiosis,
idiopathic pulmonary fibrosis and alveolar cellcarcinoma
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Lung sarcoidosis
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Extrapulmonary manifestation
Skin- lesions occur in 10% of cases
- sarcoidosis is the most common cause of
erythema nodosum,it is an immunological reaction
and not duo to sarcoid tissue infiltration- lupus pernio which is a chil-blain- like lesion
- polymorphic lesions( papules,nodules and plaques)
- it is more common in black race
- swollen fingers (dactilitis) may also be present
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Eye
- anterior uveitis presents with misting of vision,
painful red eye- posterior uveitis may present with progressive
loss of vision
- cojuctivitis
- retinal lesion
- keratocojuctivitis sicca with lacrimal gland
enlargement
Metabolic
Hypercalcaemia and hypercalciuria (10%), it cause
renal stones and nephrocalcinosis
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Central nervous system
- is rare (2%)
- it can lead to sever disease
- chronic meningoencephalitis, cord lesion, cranial
nerve palsy particularly bilateral 8th nerve palsy
- polyneuropathy and myopathy
Bone and joint
Arthritis associated with E.nodosum , bone cyst
Hepatosplenomegaly
%)3involvement is rare (Cardiacventricular dysrhythmias, conduction defects and
cardiomyopathy with congestive heart failure
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Investigaions
-:Imaging
- Chest X-ray
- high resolution CT for assessment of diffuse
paranchymal lesion
- multislice CT for small nodules
Full blood count
- mild normochromic, normocytic anaemia
- raised ESR or CRP
Serum biochemistry- hypercalcaemia
- hypergammaglobulinaemia
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Transbronchial biobsy
- It is the most useful investigation
- It is positive in 90% of cases, even in the absenceof radiological involvement
- The typical non- caseating granulomas are found in50% of patients with extrapulmonary sarcoidosis
in whom chest X-ray is normal
ACE)converting enzyme (-Serum level of angiotensin
This enzyme is raised by two standard deviation
above the normal in over 75%
Lung function tests
There is restrictive lung defect i.e. decrease in TLC
decrease in FEV1 and FVC, and decrease in gastransfer, these tests are negative in extrapulmonary
sarcoidosis or with hilar adenopathy
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Treatment
- Hilar lymphadenopathy on its own with no evidence
on chest X- ray of involvement of lung or decrease
in lung function tests does not require treatment
- Persisting infiltration or abnormal lung function
tests need to be treated with corticosteroid
- If the disease is not improving spontaneously
6 months after diagnosis, start treatment with
prednisolone30 mg for 6 weeks.
- Reduce to alternate day treatment with predniso-
lone 15 mg for 6-12 months
- Eye involvement or persistent hypercalcaemia are
definite indication for systemic steroids
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- Erythema nodosum if sever is treated by a 2 weeks
course of prednisolone 5-15 mg daily, the same is
applied to uveoparotid fever.- Myocardial sarcoidosis and neurological
manifestation are also treated with prednisolone
Prognosis
- sarcoidosis is more sever in American blacks
where death rates of up to 10%
- fatality is less than 5% in UK
- the cause of death is usually respiratory failure
and cor pulmonale, rarely from myocardial or renal
damage
- the disease remits within 2 years in over two-third
of patients with hilar lymphadenopathy
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