Sarcoidosis

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Sarcoidosis

Title:

    Sarcoidosis

Definition:

    Sarcoidosis is a multisystem granulomatous disorder

    It usually affects young adults and presents with bilateral hilar lymphadenopathy, pulmonary infiltration,  skin and eye lesions.

Aetiology:

    Unknown

Pathogenesis

    The characteristic lesion in sarcoidosis is the granuloma

    Sarcoid granulomas are formed by accumulations of epithelioid histiocytes often including giant cells and lymphocytes (mainly T)

    Schaumann bodies (laminated, calcified proteinaceous concretions) may be present

    Asteroid bodies (stellate inclusions within giant cells) may occur

Clinical Features:

History:

    Peak incidence 3rd and 4th decades

    Females > Males

E&M:

    Fatigue

    Weight loss

    Fever

RS:

    Dyspnoea

CNS:

    Seizures

Examination:

IS:

    Erythema nodosum

    Lupus pernio

CVS:

    Conduction defects

    Ventricular dysrhythmias

    Cardiomyopathy

    Cor pulmonale

RS:

    Pulmonary infiltration

GIT:

    Keratoconjunctivitis sicca

    Uveoparotid fever:

Bilateral uveitis and parotid enlargement sometimes associated with facial palsy

    Hepatosplenomegaly

CNS:

    Uveitis

    Conjunctivitis

    Retinal lesions

    Cranial nerve palsies

    Transverse myelitis

    Peripheral neuropathy

    Meningism

LMS:

    Bone cysts:

Particularly in the digits

Investigations:

Fluids:

Blood

Biochemistry:

    Raised serum calcium:

Alpha hydroxylation of 25 hydroxycholecalciferol to 1,25 dihydroxycholecalciferol takes place in sarcoid macrophages in the lungs

This increases levels of calcitriol in the blood and results in hypercalcaemia

     

    Elevated serum ACE:

Useful in assessing activity of the disease

Imaging:

CXR:

    Bilateral hilar lymphadenopathy

    Mottling in the mid-zones

    Generalised fine nodular shadows

    Pulmonary line shadows

    Honeycomb appearance

Staging of Thoracic Sarcoid:

    Stage 1:

Normal

    Stage 2:

Hilar lymphadenopathy

    Stage 3:

Hilar lymphadenopathy + parenchymal infiltrate

    Stage 4

Parenchymal infiltrate+fibrosis

High Resolution CT Scan

    Show features characteristic of sarcoid

Physiological assessment

Lung Function Tests:

    Decreased TLC

    Decrease in FEV1 and FVC

    Decreased gas transfer

Endoscopy

Bronchoscopy and Bronchoalveolar Lavage (BAL)

    30 % lymphocyte counts

    Normal 10%

    May be as high as 50-60% in florid cases

Tissue Diagnosis:

Transbronchial lung biopsy:

    Positive in 90%               

Management:

Control

Drugs:

    Steroids

    Immunosuppressive agents:

Methotrexate, azathioprine, ciclosporin

    Chloroquine

Indications for Steroids:

    No spontaneous improvement in pulmonary disease 6 months after diagnosis

    Eye involvement

    Persistent hypercalcaemia

    Severe erythema nodosum

    Uveoparotid fever

    Myocardial sarcoid

    Neurological manifestations

Prognosis

Thoracic

    60% thoracic sarcoid resolves spontaneously

    20% resolve with steroids, which may then be discontinued

    10-20% chronic disease

Chronic extrathoracic sarcoid

    Responds poorly to treatment

    CNS poor response

 

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