Sarcoidosis

Question 1

What is sarcoidosis?

Answer 1

Multisystem disorder (most commonly lungs, skin, and eyes) characterised by non-caseating granulomatous (no central necrosis) inflammation which deposit around the body

Question 2

What are the risk factors for sarcoidosis?

Answer 2

UNKNOWN 
- Transmissible agents (e.g. viruses), environmental triggers and genetic factors have all been suggested 

suggested risk factors:
- Agricultural exposures
- Insecticides
- Mouldy environments
- Firefighters
suggested protective factors:
- Cigarette smoking

Question 3

What are the presenting symptoms of sarcoidosis?

Answer 3

• Anterior Uveitis → photophobia, red painful eye, blurred vision
• Chronic Dry Cough
• Wheezing
• Dyspnoea
• Lymphadenopathy (typically cervical & submandibular nodes)
• Erythema Nodosum → rash on shin
• Lupus Pernio → rash on face, nose, ears
• Chronic Fatigue
• Arthralgia (pain in knees, ankles & wrists)

Question 4

What investigations are used to diagnose/ monitor sarcoidosis?

Answer 4

1. CXR → determine stage of disease. Best initial test. Shows bilateral hilar lymphadenopathy.
   - Stage 0 - may be clear  
   - Stage 1 - bilateral hilar lymphadenopathy 
   - Stage 2 - stage 1 with pulmonary infiltration and paratracheal node enlargement  
   - Stage 3 - pulmonary infiltration alone
   - Stage 4 - Pulmonary fibrosis  
2. High-Resolution CT may also be done if suspicious CXR.
3. Bronchoscopy with Lung Biopsy → gold standard for diagnosis. Will show non-caseating granulomas.
4. Spirometry → may show restrictive defect
5. Hypercalcaemia
6. Raised Serum ACE
7. Bloods:
   - FBC → anaemia & leukopenia
   - Urea & Creatinine → elevated if renal involvement

Question 5

How is sarcoidosis managed?

Answer 5

1. 1st Line → Corticosteroids (Prednisolone, Hydrocortisone)
   - Indications ⇒ patients with CXR stage 2/3 who are symptomatic / patients who have hypercalcaemia / patients with eye, heart or neuro involvement.
   - Stage 1 may resolve spontaneously
2. Alternative immunosuppressive therapy → methotrexate or azathioprine
3. Antimalarial Drugs → chloroquine, hydroxychloroquine
4. NSAIDs for symptomatic relief
5. Last resort in severe disease → lung transplant

Question 6

What signs of sarcoidosis can be found on physical examination?

Answer 6

1. Acute Sarcoidosis
   Signs and symptoms of acute sarcoidosis, also known as Löfgren syndrome, include:
   - Fever
   - Polyarthralgia
   - Erythema nodosum
   - Bilateral hilar lymphadenopathy
2. Chronic Sarcoidosis
   - Pulmonary: Dry cough, dyspnoea, reduced exercise tolerance, and crepitations on examination
   - Constitutional: Fatigue, weight loss, arthralgia, low-grade fever, lymphadenopathy, and enlarged parotid glands
   - Neurological: Meningitis, peripheral neuropathy, bilateral Bell’s palsy
   - Ocular: Uveitis, keratoconjunctivitis sicca
   - Cardiac: Arrhythmias, restrictive cardiomyopathy
   - Abdominal: Hepatomegaly, splenomegaly, renal stones
   - Dermatological: Erythema nodosum, lupus pernio