Sarcoidosis

Cards (21)

  • Sarcoidosis
    A chronic granulomatous disorder
  • Granulomas
    Inflammatory nodules full of macrophages
  • The cause of granulomas in sarcoidosis is unknown
  • Sarcoidosis is usually associated with respiratory symptoms but has many extra-pulmonary manifestations such as erythema nodosum and lymphadenopathy
  • Symptoms of sarcoidosis can vary dramatically from asymptomatic to severe or life-threatening
  • Groups more commonly affected by sarcoidosis
    • Aged 20-39 or around 60
    • Women
    • Black ethnic origin
  • The typical MCQ exam patient with sarcoidosis is a 20-40 year old black female presenting with a dry cough and shortness of breath. They may have nodules on their shins, suggesting erythema nodosum
  • Skin Features of Sarcoidosis
    • Less than half of patients have skin involvement
    • Erythema nodosum is characterised by nodules of inflamed subcutaneous fat on the shins. It presents as raised, red, tender, painful, subcutaneous nodules across both shins. Over time, the nodules settle and appear as bruises
    • Lupus pernio is specific to sarcoidosis and presents with raised purple skin lesions, often on the cheeks and nose
  • Erythema nodosum is a type of panniculitis
  • Organs affected by sarcoidosis include almost any organ in the body. The lungs are most commonly affected, followed by various systemic symptoms and involvement of organs like the liver, eyes, heart, kidneys, central nervous system, peripheral nervous system, and bones
  • Systemic Symptoms of Sarcoidosis
    • Fever
    • Fatigue
    • Weight loss
  • Organ Involvement in Sarcoidosis
    • Lungs: Mediastinal lymphadenopathy, Pulmonary fibrosis, Pulmonary nodules
    • Liver: Liver nodules, Cirrhosis, Cholestasis
    • Eyes: Uveitis, Conjunctivitis, Optic neuritis
    • Heart: Bundle branch block, Heart block, Myocardial muscle involvement
    • Kidneys: Kidney stones (due to hypercalcaemia), Nephrocalcinosis, Interstitial nephritis
    • Central nervous system: Nodules, Pituitary involvement (diabetes insipidus), Encephalopathy
    • Peripheral Nervous System: Facial nerve palsy, Mononeuritis multiplex
    • Bones: Arthralgia, Arthritis, Myopathy, Lofgren’s Syndrome
  • Manifestations of sarcoidosis
    • Nephrocalcinosis
    • Interstitial nephritis
    • Nodules
    • Pituitary involvement (diabetes insipidus)
    • Encephalopathy
    • Facial nerve palsy
    • Mononeuritis multiplex
    • Arthralgia
    • Arthritis
    • Myopathy
    • Lofgren’s Syndrome
  • Lofgren’s Syndrome

    Refers to a specific presentation of sarcoidosis with a classic triad of symptoms: Erythema nodosum, Bilateral hilar lymphadenopathy, Polyarthralgia (joint pain in multiple joints)
  • Differential Diagnosis for sarcoidosis
    • Tuberculosis
    • Lymphoma
    • Hypersensitivity pneumonitis
    • HIV
    • Toxoplasmosis
    • Histoplasmosis
  • Blood test findings in sarcoidosis
    • Raised angiotensin-converting enzyme (ACE) (often used as a screening test)
    • Raised calcium (hypercalcaemia)
  • Imaging investigations for sarcoidosis
    • Chest x-ray may show hilar lymphadenopathy
    • High-resolution CT scanning may show hilar lymphadenopathy and pulmonary nodules
    • MRI can show central nervous system involvement
    • PET scan can show active inflammation in affected areas
  • Histology in sarcoidosis
    Helps establish the diagnosis, often by bronchoscopy with an ultrasound-guided biopsy of mediastinal lymph nodes. Characteristically shows non-caseating granulomas with epithelioid cells
  • Other tests for organ involvement in sarcoidosis
    • U&Es for kidney involvement
    • Urine albumin-creatinine ratio to look for proteinuria
    • LFTs for liver involvement
    • Ophthalmology assessment for eye involvement
    • ECG and echocardiogram for heart involvement
    • Ultrasound for liver and kidney involvement
  • Management of sarcoidosis
    1. Conservative management is considered in patients with no or mild symptoms
    2. Oral steroids (for 6-24 months) are usually first-line where treatment is required
    3. Bisphosphonates protect against osteoporosis whilst on long-term steroids
    4. Methotrexate is a second-line option
    5. Lung transplant is rarely required in severe pulmonary disease
  • Sarcoidosis spontaneously resolves in around half of patients, usually within two years. In some patients, it progresses to pulmonary fibrosis and pulmonary hypertension. Overall mortality is less than 10%