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Sarcoidosis

Title:

Ñ    Sarcoidosis

Definition:

Ñ    Sarcoidosis is a multisystem granulomatous disorder

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

Aetiology:

Ñ    Unknown

Pathogenesis

Ñ    The characteristic lesion in sarcoidosis is the granuloma

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

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

Ñ    Asteroid bodies (stellate inclusions within giant cells) may occur

Clinical Features:

History:

Ñ    Peak incidence 3^rd and 4^th decades

Ñ    Females > Males

E&M:

Ñ    Fatigue

Ñ    Weight loss

Ñ    Fever

RS:

Ñ    Dyspnoea

CNS:

Ñ    Seizures

Examination:

IS:

Ñ    Erythema nodosum

Ñ    Lupus pernio

CVS:

Ñ    Conduction defects

Ñ    Ventricular dysrhythmias

Ñ    Cardiomyopathy

Ñ    Cor pulmonale

RS:

Ñ    Pulmonary infiltration

GIT:

Ñ    Keratoconjunctivitis sicca

Ñ    Uveoparotid fever:

Bilateral uveitis and parotid enlargement sometimes associated with facial palsy

Ñ    Hepatosplenomegaly

CNS:

Ñ    Uveitis

Ñ    Conjunctivitis

Ñ    Retinal lesions

Ñ    Cranial nerve palsies

Ñ    Transverse myelitis

Ñ    Peripheral neuropathy

Ñ    Meningism

LMS:

Ñ    Bone cysts:

Particularly in the digits

Investigations:

Fluids:

Blood

Biochemistry:

Ñ    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

Ñ     

Ñ    Elevated serum ACE:

Useful in assessing activity of the disease

Imaging:

CXR:

Ñ    Bilateral hilar lymphadenopathy

Ñ    Mottling in the mid-zones

Ñ    Generalised fine nodular shadows

Ñ    Pulmonary line shadows

Ñ    Honeycomb appearance

Staging of Thoracic Sarcoid:

Ñ    Stage 1:

Normal

Ñ    Stage 2:

Hilar lymphadenopathy

Ñ    Stage 3:

Hilar lymphadenopathy + parenchymal infiltrate

Ñ    Stage 4

Parenchymal infiltrate+fibrosis

High Resolution CT Scan

Ñ    Show features characteristic of sarcoid

Physiological assessment

Lung Function Tests:

Ñ    Decreased TLC

Ñ    Decrease in FEV₁ and FVC

Ñ    Decreased gas transfer

Endoscopy

Bronchoscopy and Bronchoalveolar Lavage (BAL)

Ñ    30 % lymphocyte counts

Ñ    Normal 10%

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

Tissue Diagnosis:

Transbronchial lung biopsy:

Ñ    Positive in 90%               

Management:

Control

Drugs:

Ñ    Steroids

Ñ    Immunosuppressive agents:

Methotrexate, azathioprine, ciclosporin

Ñ    Chloroquine

Indications for Steroids:

Ñ    No spontaneous improvement in pulmonary disease 6 months after diagnosis

Ñ    Eye involvement

Ñ    Persistent hypercalcaemia

Ñ    Severe erythema nodosum

Ñ    Uveoparotid fever

Ñ    Myocardial sarcoid

Ñ    Neurological manifestations

Prognosis

Thoracic

Ñ    60% thoracic sarcoid resolves spontaneously

Ñ    20% resolve with steroids, which may then be discontinued

Ñ    10-20% chronic disease

Chronic extrathoracic sarcoid

Ñ    Responds poorly to treatment

Ñ    CNS poor response

 

 

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