[9] Sarcoidosis Flashcards
What is sarcoidosis?
Multisystem granulomatous disorder of an unknown cause
What age is sarcoidosis most common?
20-40 years
What gender is sarcoidosis more common in?
Female
What ethnicity is sarcoidosis more common in?
Afro Caribbean
What genes are associated with sarcoidosis?
HLA-DRB1 and DQB1
What % of cases of sarcoidosis present incidentally on CXR?
20-40%
What are the symptoms of acute sarcoidosis?
Erythema nodosum
Fever
Polyarthralgia
Bilateral hilar lymphadenopathy
What pneumonic can be used to remember the clinical features of sarcoidosis?
GRANULOMAS G = General R = Respiratory A = Arthralgia N = Neurological U = Urine L = Low hormones O = Opthalmological M = Myocardial A = Abdominal S = Skin
What are the general features of sarcoidosis?
Fever
Anorexia and weight loss
Fatigue
Lymphadenopathy and hepatosplenomegaly
What are the upper respiratory features of sarcoidosis?
Otitis
Sinusitis
What are the lower respiratory features of sarcoidosis?
Dry cough
Chest pain
Decreased lung function
What % of people with sarcoidosis have lower respiratory features?
90%
What can the lower respiratory features of sarcoidosis be divided into?
Stage 1-4
What are the stage 1 lower respiratory features of sarcoidosis?
Bilateral hilar lymphadenopathy
What are the stage 2 lower respiratory features of sarcoidosis?
BHL + peripheral infiltrates
What are the stage 3 lower respiratory features of sarcoidosis?
Peripheral infiltrates alone
What are the stage 4 lower respiratory features of sarcoidosis?
Progressive mid-zone fibrosis with bullae
What are the arthralgia features of sarcoidosis?
Polyarthralgia
Dactylitis
What are the neurological features of sarcoidosis?
Peripheral and cranial polyneuropathy, especially Bell’s palsy
Meningitis, transverse myelitis, or space-occupying-lesions
What are the urine features of sarcoidosis?
Increased calcium, leading to renal stones, nephrocalcinosis, and diabetes insipidus
What are the hormonal features of sarcoidosis?
Pituitary dysfunction, e.g. amenorrhoea
What are the ophthalmological features of sarcoidosis?
Uveitis
Keratoconjunctivitis
Sicca/Mikulicz/Sjorgen’s syndrome
What are the myocardial features of sarcoidosis?
Restrictive cardiomyopathy
Pericardial effusion
What are the abdominal features of sarcoidosis?
Hepatomegaly and cholestatic LFTs
Splenomegaly
What are the skin features of sarcoidosis?
Erythema nodosum
Lupus pernio
What is erythema nodosum?
Painful erythematous nodules in shins
What is lupus pernio?
Raised, dusky purple plaque on nose, cheek, fingers
What investigations should be done in sarcoidosis?
Bloods Imaging Tuberculin skin test Lung function tests Tissue biopsy Ophthalmology assessment
What may be found on bloods in sarcoidosis?
Increased ESR Increased calcium Lymphopenia Increased serum ACE Increased Ig Increased LFTs
What imaging should be done in sarcoidosis?
CXR
CT
MRI
What proportion of people with sarcoidosis have a positive tuberculin skin test?
1/3
What might be found on lung functioning testing in sarcoidosis?
Restrictive pattern with decrease FVC
Decreased transfer factor
What tissue biopsies should be taken in sarcoidosis?
Lung
Lymph nodes
Skin nodules
Liver
What finding on tissue biopsy is diagnostic for sarcoidosis?
Non-caseating granulomas
Do patients who are asymptomatic with BHL require management?
No
How is acute sarcoidosis managed?
Bed rest
NSAIDs
What is the prognosis of acute sarcoidosis?
Usually resolve spontaneously
What is the first line management for chronic sarcoidosis?
Preg 40mg/day for 4-6 weeks
What additional immunosuppression can be used in chronic sarcoidosis?
Methotrexate
Ciclosporin
Cyclophosphamide
What % of cases of thoracic sarcoidosis resolve over 2 years?
60%
What % of cases of chronic sarcoidosis respond to steroids?
20%
What % of cases of chronic sarcoidosis have no improvement despite management?
20%
What are the differential diagnoses for BHL?
Sarcoidosis
Infection
Malignancy
Interstitial disease
What infections can cause BHL?
TB
Mycoplasma
What cancers can cause BHL?
Lymphoma
Carcinoma
What forms of interstitial disease can cause BHL?
EAA
Silicosis
What are the differential diagnoses for granulomatous disease?
Infections Autoimmune Vasculitis Idiopathic Interstitial lung disease
What infections can cause granulomatous disease?
TB Leprosy Syphilis Cryptosporidium Schistosomiasis
What autoimmune conditions can cause granulomatous disease?
Primary biliary cholangitis
What vasculitic conditions can cause granulomatous disease?
Giant cell arteritis
Polyarteritis nodosa
Wegener’s
Takayasu’s
What are the idiopathic causes of granulomatous disease?
Crohn’s
Sarcoid
What are the interstitial lung causes of granulomatous disease?
Extrinsic allergic alveolitis
Silicosis
