Sarcoidosis Flashcards
(14 cards)
What is sarcoidosis?
Sarcoidosisis achronic granulomatous disorder.
Granulomasareinflammatorynodulesfull ofmacrophages. The cause of these granulomas is unknown.
It is usually associated with respiratory symptoms but has manyextra-pulmonary manifestations, such aserythema nodosumandlymphadenopathy.
Symptoms can vary dramatically from asymptomatic to severe or life-threatening.
Therefore sarcoidosis is a multisystem disease of unknown aetiology
What is the epidemiology?
can affect anyone
2 spikes in incidence; one in young adulthood and one from age 60
What re the risk factors?
slightly more common in
Aged 20-39 or around 60
Women
Black ethnic origin
What are the organs that are affected?
Sarcoidosis can affect almost any organ in the body.
The lungs are most commonly affected (in over 90% of patients), so condition falls under respiratory system
Lungs:
Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules
Liver:
Liver nodules
Cirrhosis
Cholestasis
Kidneys:
Kidney stones (due to hypercalcaemia)
Nephrocalcinosis
Interstitial nephritis
Eyes:
Uveitis
Conjunctivitis
Optic neuritis
Heart: (5% of people)
Bundle branch block
Heart block
Myocardial muscle involvement
Systemic Symptoms:
Fever
Fatigue
Weight loss
What are the skin symptoms of sarcoidosis?
Less than half of patients with sarcoidosis have skin involvement. (15% of people)
Erythema nodosumis characterised by nodules ofinflamedsubcutaneous faton the shins. Inflammation of fat is calledpanniculitis.
Erythema nodosum presents as raised, red, tender, painful, subcutaneous nodules across both shins. Over time the nodules settle and appear as bruises. There are many causes of erythema nodosum.
Lupus perniois specific to sarcoidosis and presents with raised purple skin lesions, often on the cheeks and nose.
How could the bones be potentially effected in sarcoidosis?
Arthralgia
Arthritis
Myopathy
How can the Nervous system be affected in sarcoidosis?
Central nervous system:
Nodules
Pituitary involvement (diabetes insipidus)
Encephalopathy
Peripheral Nervous System:
Facial nerve palsy
Mononeuritis multiplex
What is Lofgren’s syndrome?
Lofgren’s syndromerefers to a specific presentation of sarcoidosis with a classic triad of symptoms:
-Erythema nodosum (tender, red nodules (bumps) usually on the shins)
-Bilateral hilar lymphadenopathy
Polyarthralgia (joint pain in multiple joints)
What are the top differentials for the varied presenting features of sarcoidosis are?
Tuberculosis
Lymphoma
Hypersensitivity pneumonitis
HIV
Toxoplasmosis
Histoplasmosis
What are the investigations?
General observations - RR, O2 sats, temp, pulse
Respiratory examination
Blood Tests:
Raisedangiotensin-converting enzyme(ACE) (often used as a screening test)
Raisedcalcium(hypercalcaemia)
Other Tests – maybe be used to determine which organs are affected:
U&Esfor kidney involvement
Urine albumin-creatinine ratioto look forproteinuria
LFTsfor liver involvement
Ophthalmologyassessment for eye involvement
ECGandechocardiogramfor heart involvement
Ultrasoundfor liver and kidney involvement
What will imaging show?
Chest x-raymay showhilar lymphadenopathy
High-resolution CTscanningmay showhilar lymphadenopathyandpulmonary nodules
MRIcan showcentral nervous system involvement
PET scancan show active inflammation in affected areas
How can histology be used?
Histologyhelps establish the diagnosis, often bybronchoscopywith anultrasound-guided biopsyofmediastinal lymph nodes. Histology characteristically showsnon-caseating granulomaswithepithelioid cells.
How is sarcoidosis managed?
Conservative managementis considered in patients with no or mild symptoms.
Oral steroids(for 6-24 months) are usually first-line where treatment is required.Bisphosphonatesprotect against osteoporosis whilst on long-term steroids.
Methotrexateis a second-line option. (or Azathioprine)
Lung transplant- in severe pulmonary disease.
What is the prognosis of sarcoidosis?
Sarcoidosis spontaneously resolves in around half of patients, usually within two years.
In some patients, it progresses topulmonary fibrosisandpulmonary hypertension. Overall mortality is less than 10%.
Death – Usually due to cardiac causes as a result of arrythmias or due to CNS involvement
