Sarcoidosis

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  • 1. Sarcoidosis DEAPCIT
  • 2. definition • Idiopathic, multi-system, chronic granulomatous disease characterised by noncaseating granuloma formation
  • 3. epidemiology • Prevalence = 50/100 000 • Common in African Americans, not in Africans living in Africa • Common in Japan, uncommon in rest of Asia • Clusters in health workers • Age = 20 – 40 yrs • F>M
  • 4. aetiology • Genetic predisposition + environmental trigger • Genetic – Most common allele found in sarcoidosis is HLA-B8 • ? Infectious Ag triggers CMI response & starts disease process – Many organisms have been linked to sarcoidosis: • M Tb • Mycoplasma species; Borrelia burgdorferi; Propionibacterium acnes • Fungi - Histoplasma and Cryptococcus species • Viruses - EBV, CMV, HSV, hepatitis C virus, and rubella
  • 5. pathophysiology • May begin with lung alveolitis • This overstimulated local cellular immune response may involve overactive TH-cells, which release chemotactic factors to attract monocytes from blood to lungs & nose, resulting in chronic inflammation • Macrophages transform into giant cells, which then form epithelioid cells, which form granuloma • Mature epithelioid granulomas release ACE
  • 6. pathology • Giant cells, epithelioid cells & macrophages in noncaseating granulomas, which have fewer lymphocytes surrounding = ‘naked tubercle’ • Fibrinoid necrosis • Giant cells have prominent inclusion bodies (e.g. asteroids, Schaumann) • Asteroid bodies are star-shaped eosinophilic structures • Schaumann bodies are round or oval, laminated structures, which are usually calcified at the periphery • Granulomas are referred to as ‘naked’ because they only have a sparse lymphocytic infiltrate at the margins • Giant cells are larger & have more nuclei than in TB
  • 7. Schaumann Bodies non-caseating granulomas Asteroid bodies
  • 8. Clinical (2 patterns) • Acute – Sudden, with remission in 2 years – More common in Caucasians – May present with Lofgren’s Syndrome • • • • Bilateral hilar LN Ankle arthritis Erythema nodosum • Constitutional symptoms – – Nodular, erythematous eruption on anterior LL Type IV hypersensitivity reaction Chronic – Gradual onset, with relapses – More organ-specific symptoms, which result from irreversible fibrosis
  • 9. Course of disease • Asymptomatic (30-50%) • Constitutional Symptoms (30%) • Organ-Specific Symptoms (20-40%) – Lung LN (90%, 30% symptomatic) – LN = 30% – splenomegaly in 40 – 80% – Skin = 25% – Eye = 20 – 30% – Neuro = < 10% – Cardiac = 5% – GIT – Renal
  • 10. ENT • Occur in 9%-15% of patient with sarcoidosis • Most common with – Cervical LN – Parotidomegaly – Facial nerve palsy
  • 11. Laryngeal = 1 – 5% – Hoarseness, dyspnoea / stridor – Rarely painful – Early stage = whitebrown mucosal nodules – Later stage = pale, oedematous epiglottis • Lesser changes in remaining supraglottis (arytenoids, aryepiglottic folds, false VC) • Glottis rarely involved
  • 12. • Sinonasal = 10%: – NOT often presenting symptom – Nasal obstruction, rhinorrhoea, PND – Anosmia, recurrent sinusitis, epiphora – Firm, raised subcutaneous yellow papule on nose, may become confluent – Diffuse nasal crusting & thick mucous – Submucosal nodules on septum & IT, with dry friable mucosa • Sarcoidosis interferes with submucosal gland function, causing dryness – Septal perforations & polyps – Rarely, granulomas may erode through palate • Ear - rare – Pinna = nodules – ME = ET obstruction from NP involvement – SNHL < 1% = sudden, asymmetric, fluctuating, high or low frequency – Permanent loss or complete recovery – Vestibular involvement recovers less well than acoustic – Vestibulo-acoustic involvement is often concurrent with facial palsy & uveitis
  • 13. • Oropharyngeal – Tonsils in 2% – Pharyngeal & oesophageal stenosis may result from fibrosis • Neurosarcoid = 5% – CN > CNS symptoms (e.g. pituitary involvement causes DI) – Basal Granulomatous Meningitis • Causes multiple cranial neuropathies by involvement of nerves with perivascular lymphocytic infiltrates in IAC, causing fluctuating ischaemia • Facial > optic > glossopharyngeal > vagus > cochlear nerves – TV channels + 8 • Fluctuating ischaemia of facial nerve caused by perivascular lymphocytic infiltration proximal to geniculate ganglion, leads to reversible neuropraxia – often sudden, bilateral palsies, which resolve • Cervical LN = 50% – FNA has high yield • Parotidomegaly = 6% – Usually bilateral & FNA is useful – Heerfordt’s disease (Uveoparotid Fever) • Uveitis, parotitis, facial paralysis, fever
  • 14. Investigation • Biopsy – Submucosal nodules or transbronchial lung, LN, lip gland – histopath, AFB, culture • Bronchoalveolar lavage = elevated CD4:CD8 ratio • Haematologic – Ca (serum & urine) = elevated – Serum ACE • Correlates with active disease in 40 – 90% • Used in monitoring • CXR (respiratory symptoms are most common) – Bilateral hilar lymphadenopathy & lung infiltrates • Kveim Test – Part of a spleen from a patient with known sarcoidosis is injected into skin of a patient suspected to have the disease – If granulomas are found (4–6 weeks later), the test is positive
  • 15. Management • MEDICAL – Steroids = mainstay – Systemic steroids in eye / organ involvement – Topical steroids in skin / eye disease – Inhaled steroids in URT / LRT disease – MTX / Azathioprine • Surgical
  • 16. • Laryngeal – Steroids – Intralesional steroid injection – If unresponsive, consider low-dose RTH – Tracheostomy or RTH / laser to obstructing lesions