Sarcoidosis

Multisystem chronic inflammatory disease characterised by non-caseating epithelioid granulomata. It has a predilection for the lungs and thoracic cavity. The skin and eyes are the next most commonly affected followed by the liver, heart and nervous system.

Sarcoidosis affects mid20s to mid 40s

Presentation
Half the patients diagnosed are asymptomatic and found incidentally on CXR. Black patients have more serious pulmonary involvement with poorer long-term prognosis and increased frequency of relapses.

Patients may present with non-specific constitutional symptoms

Symptoms
Acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia

Insidious: dyspnoea, non-productive cough, malaise, weight loss

Hypercalcaemia- macrophages inside the granulomas cause increased conversion of vit D to it's active form
 * Constitutional
 * Lungs/skin/heart/liver/joints
 * Eyes (ant/post uveitis)
 * Neurosarcoidosis
 * Lymphadenopathy
 * Hypercalcaemia and hypercalciuria
 * Other organs/systems

Signs on examination:
Unilateral pain but bilateral CXR signs

Investigations
Scans: Biopsy- show non-caseating granuloma (liver, lymph nodes and lacrimal glands)
 * CXR- classical finding is hilar lymphadenopathy, with clear lung fields and fluffy opacities in the hilar region
 * High res CT to detect interstitial lung disease
 * ECG- check early signs

Pulmonary function tests- may show reduced lung volume and restrictive lung pattern

Bloods: FBC (anaemia/raised WBC), ESR (raised), raised serum ACE, Mantoux test to rule out TB

Staging on CXR
Stage 0- normal CXR

Stage 1 - BHL

Stage 2- BHL + pulmonary infiltrates

Stage 3- Peripheral pulmonary filtrates alone

Stage 4- Progressive pulmonary fibrosis +/- honeycombing (bullae)

Management
Stage 0/1 usually resolves spontaneously

Stage 2 and acute disease- may improve with NSAIDs and bed rest

 Consider steroids if: 
 * patients with chest xray stage 2-3, mod/severe/progressive symptoms
 * hypercalcaemia
 * parenchymal lung disease (even if asymp)
 * eye, heart or neuro involvement

In very severe cases: IV methyprednisolone/ immunosuppresants

Complications

 * Pulmonary (infections, PE, progressive fibrotic lung disease, pulmonary HT)
 * Cardiac (arrhythmia, sudden death, cardiac failure)
 * Ocular (uveitis, conjunctivitis, severe sign impairement)
 * Skin (chronic refractory skin disease)
 * NS (cranial and peripheral nerve damage, neuropsychiatric illness, permanent CNS impairment, stroke, TIA)
 * ENT (salivary gland dysfunction, nosebleeds, nasal obstruction)

Prognosis
Die from resp failure/ morbidity from pulmonary fibrosis

Prevention
Sarcoidosis cannot be prevented but measures may reduce complications Questions
 * Flu jab
 * osteoporosis prophylaxis
 * education
 * smoking cessation