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sarcoidosis

sarcoidosis

introduction

  • a chronic disease in which granulomas form as nodules in multiple organs, especially lungs and LNs, but also skin (25%), eyes, liver, heart, kidneys
  • cause is unknown
  • commonly improves or clears up spontaneously - most have no symptoms after 9 years but 50% have relapses and 10% develop serious disability - usually respiratory failure

clinical features

  • may be asymptomatic
  • symptoms may include chronic fatigue, LOW, aches and pains, arthritis, dry eyes, blurry vision, SOB, dry hacking cough, skin rashes, skin nodules
  • Löfgren syndrome: combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia
  • 90% eventually develop an abnormal the chest X-ray (CXR)
    • 75-90% develop hilar &/or paratracheal LN enlargement
  • generalised lymphadenopathy is common, especially cervical nodes
  • 5-15% develop pulmonary fibrosis
  • anaemia occurs in 4-20%
  • 40% develop mild-mod lymphopenia
  • 20-30% develop hepatomegaly
  • 5% develop symptomatic cardiac disease
  • 5% develop neurologic involvement such as cranial nerve palsies, chronic meningitis or peripheral neuropathy
  • uveitis
  • up to 5% develop parotid involvement which is usually bilateral and may cause xerostomia
  • uveoparotid fever - combination of anterior uveitis, parotitis, VII cranial nerve paralysis and fever
  • may cause hypervitaminosis D ⇒ compensatory hyoparathyroidism, fatigue, metallic taste, cognitive dysfunction, immune dysfunction
  • up to 1/3rd develop hyperprolactinaemia
  • some may develop thyroid dysfunction
  • association with coeliac disease

Diagnosis

  • essentially is based on clinical features combined with radiographic features and preferably histology showing non-caseating granulomas.
  • CXR
  • lung function tests
  • serum ACE is controversial
    • is a marker of sarcoid granuloma burden
    • has poor sensitivity (60%) and specificity for diagnosis though
      • may also be elevated in other conditions such as Gaucher’s disease, disseminated tuberculosis, hyperthyroidism and fungal infections
      • ACE levels are reduced in patients on ACE inhibitors
sarcoidosis.txt · Last modified: 2018/08/18 22:33 (external edit)