Sarcoidosis

• Multisystem granulomatous disease of unknown origin
• Commonly affects adults aged 20-40
• Afro-Caribbeans more frequently affected

Clinical features

• Often asymptomatic – discovered with routine CXR
• Acute sarcoidosis can present with erythema nodosum and polyarthralgia

Pulmonary disease

• Bilateral hilar lymphadenopathy
• Pulmonary infiltrates
• Fibrosis
• Symptoms – dry cough, progressive dyspnoea, decreased exercise tolerance and chest pain

Non-pulmonary manifestations

• Lymphadenopathy
• Hepatomegaly
• Splenomegaly
• Uveitis, conjunctivitis, keratoconjunctivitis, sicca, glaucoma
• Terminal phalangeal bone cysts
• Enlargement of parotid and lacrimal glands
• Bell’s palsy, meningitis, neuropathy, SOL
• Erythema nodosum, subcutaneous nodules
• Cardiomyopathy
• Arrhythmias
• Hypercalcaemia, hypercalciuria, renal stones
• Pituitary dysfunction

Tests

Bloods – raised ESR, lymphopenia, raised LFTs, raised serum ACE, raised Ca+, raised Ig

• 24hr urine – raised Ca+
• Tuberculin skin test –generally negative
• CXR
  • Stage 0 – normal
  • Stage 1 – BHL
  • Stage 2 – BHL plus peripheral pulmonary infiltrates
  • Stage 3 – peripheral pulmonary infiltrates alone
  • Stage 4 – progressive pulmonary fibrosis, honeycombing, pleural involvement
• ECG – may show arrhythmia or BBB
• Lung function – may be normal or may show restrictive defect
• Tissue biopsy – non-caseating granuloma
• Bronchoalveolar lavage – shows increased lymphocytes in active disease and increased neutrophils with pulmonary fibrosis

Management

• Patients with BHL don’t require treatment as the majority will resolve on their own
• Acute sarcoidosis – bed rest, NSAIDs

Indications for corticosteroid treatment;

• Parenchymal lung disease
• Uveitis
• Hypercalcaemia
• Neurological or cardiac involvement
• Prednisolone 40mg/24hr for 4-6 weeks and then reduced dose for 1yr according to clinical status
• In severe disease IV methylprednisolone or immunosuppressants may be required

Prognosis

• 60% show spontaneous resolution after 2 years
• 20% respond to standard steroids
• 20% don’t improve despite therapy

Causes of BHL

• Sarcoidosis
• Infection
  • TB
  • Mycoplasma
• Malignancy
  • Lymphoma
  • Carcinoma
  • Mediastinal tumours
• Organic dust disease
  • Silicosis
  • Berylliosis
• Extrinsic allergic alveolitis