36 medicine sarcoidosis

8/4/2019 36 Medicine Sarcoidosis

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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

36 medicine sarcoidosis

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