Sarcoidosis

• What is sarcoidosis?


  Systemic granulomatous disease of unknown aetiology

  Characterised by formation of non-caseating granulomas (can affect any organ)
• What is the most commonly involved site for sarcoidosis?


  Lungs & intrathoracic lymph nodes
• Pathophys of sarcoidosis

  In genetically predisposed individuals, an unidentified antigen activates APCs (dendritic cells and macrophages) → present antigen to CD4+ Th cells -> activates Th cells -> differentiate into Th1 (produce IFN-y & IL-2) & Th17 (produce IL-17, IL-21 & IL-22) -> recruit macrophages -> macrophages transform into epithelioid & multinucleated giant cells -> forms granulomas to contain antigen -> granulomas can lead to fibrosis & potentially organ dysfunction
• How can sarcoidosis be classified?


  Based on...

  • organ involvement
  • radiology
  • clinical presentation
• Classification of sarcoidosis based on organ involvement 


  Pulmonary

  • most common

  Extra-pulmonary

  • common extra-pulmonary sites = eyes, lymph nodes, liver, heart & NS
• Classification of sarcoidosis based on radiology


  Stage 0 = normal CXR

  Stage 1 = bilateral hilar lymphadenopathy (BHL) alone

  Stage 2 = BHL with pulmonary infiltrates

  Stage 3 = pulmonary infiltrates w/o BHL

  Stage 4 = pulmonary fibrosis
• Classification of sarcoidosis based on clinical presentation

  Löfgren's syndrome

  • acute onset of erythema nodosum, bilateral hilar lymphadenopathy & arthralgia/arthritis

  Heerfordt's syndrome (uveoparotid fever)

  • parotitis, anterior uveitis, fever & facial nerve palsy

  Chronic sarcoidosis

  • lasts more than 2 years
  • often associated with pulmonary fibrosis

  Silent sarcoidosis
• What are the non-specific symptoms of sarcoidosis?


  Fatigue

  Weight loss

  Arthralgia

  Fever
• What are the respiratory system signs & symptoms of sarcoidosis?


  Persistent dry cough

  SOB OE

  Chest discomfort

  Bilateral hilar lymphadenopathy
• What are the skin signs & symptoms of sarcoidosis?

  Erythema nodosum

  Lupus pernio
• What is erythema nodosum?


  Tender red nodules predominantly on anterior shins
• What is lupus pernio?


  Violaceous skin lesions on nose & cheeks
• What are the ocular signs & symptoms of sarcoidosis?


  Uveitis

  Conjunctivitis

  Scleritis
• What are the CVD system signs & symptoms of sarcoidosis?


  Conduction abnormalities (heart block or arrhythmias)

  LV dysfunction

  Sudden cardiac death
• What are the nervous system signs & symptoms of sarcoidosis?

  Cranial nerve palsies

  Seizures

  Headache

  Psychiatric symptoms
• What are the MSK signs & symptoms of sarcoidosis?


  Arthralgia

  Arthritis
• What are the endocrine & renal signs & symptoms of sarcoidosis?


  Hypercalcaemia

  Renal involvement (nephrocalcinosis or nephrolithiasis) -> secondary to hypercalcaemia
• What are the hepatic & splenic signs & symptoms of sarcoidosis?


  Hepatomegaly

  Abnormal LFTs

  Splenomegaly
• What are the RFs of sarcoidosis?


  20-50 years old

  FHx

  Scandinavian origin

  Female

  Non-smokers
• What are the Inx for sarcoidosis?


  Obs

  Full Hx & examination

  Bloods (FBC, U&Es, LFTs, CRP, serum Ca+2 levels)

  CXR

  CT

  Pulmonary function tests (restrictive pattern usually seen)

  Tissue biopsy

  Organ specific Inx
• What are the DDx of sarcoidosis?


  TB

  Histoplasmosis

  Non-small cell lung cancer

  Hodgkin's lymphoma

  Lymphoma (non-Hodgkin's)

  Berylliosis

  Hypersensitivity pneumonitis (chronic)
• What does treatment of sarcoidosis depend on?


  Severity

  Extent of organ involvement
• What is the treatment for symptomatic sarcoidosis?


  Systemic corticosteroids (1st line) -> usually prednisolone (20-40 mg)

  Immunosuppressive drugs (2nd line) -> methotrexate, azathioprine, mycophenolate mofetil
• What is the treatment for refractory sarcoidosis or when there is severe organ involvement?


  Biological therapies (infliximab, adalimumab) -> do have increased risk of infections
• What is the monitoring of sarcoidosis?


  Regular assessment of disease progression and organ function.

  Utilise pulmonary function tests, imaging studies, and lab investigations to adjust management as needed
• What is Lofgren's Syndrome?


  
  

  Distinct clinical presentation of sarcoidosis

  Characterised by triad of acute arthritis, erythema nodosum & bilateral hilar lymphadenopathy

  Acute, self-limited form of sarcoidosis

  Predominantly affects young adults (more common in females & people of Scandinavian or Irish descent)
• What are the signs & symptoms of Lofgren's Syndrome?


  Acute arthritis

  Erythema nodosum

  Bilateral hilar lymphadenopathy

  Fever

  Fatigue

  Weight loss
• What are the possible complications of Lofgren's Syndrome?

  Pulmonary fibrosis

  Airway obstruction

  Resp infections

  Ocular involvement

  Neurological complications
• What is the treatment & management of Lofgren's Syndrome?

  NSAIDs (1st line)

  Corticosteroids (usually prednisolone) -> in pts with severe symptoms unresponsive to NSAIDs

  Colchicine

  Avoidance of immunosuppressants