Sarcoidosis Flashcards
What is sarcoidosis?
Typical demographics?
Typical patient in exams?
What organs are affected?
What syndromes are associated with sarcoidosis?
- Granulomatous inflammatory condition affecting multiple organs within the body. It is usually associated with chest symptoms but has multiple extra-pulmonary manifestations.
Typical demographic
- Women > Men
- 2 spikes in incidence –> once in young adulthood and once around the age of 60
- Black > other ethnicities
- Typical patient
> 20-40 year old black female, with dry cough, SOB and nodules on her shins (erythema nodosum).
Organs affected - Lungs (90%) > Pulmonary fibrosis > Bilateral hilar lymphadenopathy > Pulmonary nodules
- CNS (5%)
> Encephalopathy
> Nodules in the brain
> Pituitary involvement –> diabetes insipidus
PNS (5%)
- Facial nerve palsy
- Mononeuritis multiplex
Eyes (20%)
- Uveitis
- Conjunctivitis
- Optic neuritis
Heart (5%)
- Heart block
- Bundle branch block
- Heart failure due to granuloma affecting the myocardium
Liver (20%)
- Liver nodules
- Liver cirrhosis
- Cholestasis (slowing of bile)
Kidneys (5%)
- Kidney stones (due to hypercalcaemia)
- Nephrocalcinosis (deposition of calcium)
- Interstitial nephritis
Joints (2%)
- Arthralgia
- Arthritis
- Myopathy
Skin
- Erythema nodosum
- Lupus pernio
Syndromes associated with sarcoidosis - Lofgren's syndrome > Erythema nodosum > Hilar lymphadenopathy > Polyarthralgia
- Mikulicz syndrome
> Enlargment of parotid and lacrimal glands due to sarcoidosis, TB, or lymphoma - Heertfordt syndrome (uveparotid fever)
> Parotid enlargement
> Fever
> Uveitis
Differential diagnosis for sarcoidosis ?
Investigations?
Management?
Prognosis?
Ddx
- TB
- Lymphoma
- Hypersensitivity pneumonitis
- HIV
- Toxoplasmosis
- Histoplasmosis
Investigations > Blood tests - Serum ACE --> raised - Serum calcium --> raised - Serum IL2R --> raised - CRP --> raised - immunoglobulins --> raised
> Imaging
- Chest X ray
> Hilar lymphadenopathy
- High resolution CT
> Hilar lymphadenopathy
> Pulmonary nodules
> Pulmonary fibrosis
- MRI
> CNS involvement
- PET scan - shows active areas of inflammation
> Histology - GOLD standard –> usually by doing bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes
Non-caseating granuloma with epitheloid cells
Tests for other organ involvement
- Kidney function.
> U&Es
> Urine dipstick test and Urine albumin-creatinine ratio to look for proteinuria indicating nephritis
Liver function - LFTs
Eye function - Opthalmology review
Ultrasound of the abdomen including liver and kidneys to further look at liver and kidney function.
Management
- No first line treatment in most cases in asymptomatic/mild cases.
- Oral steroids are used first line when treatment is required and are given for 6-24 months. Patients should also be given bisphosphonates to protect their bone health from excessive steroid exposure.
- Immunosuppressants (e.g. methotrexate and azathioprine) for second line options
- Lung transplant in patients with severe pulmonary disease.
Prognosis?
- 60% –> resolve within 6 months
- Some patients may progress to develop pulmonary hypertension and pulmonary fibrosis
- Deaths may be due to the disease affecting the heart or CNS.
Chest X ray findings for sarcoidosis? Staging?
Indications for steroids?
Stage 0 = normal –> the patient presents with a normal CXR. There is no sign of granuloma.
Stage 1 = bilateral hilar lymphadenopathy –> granulomas are only present in the lymph nodes
Stage 2 = BHL + interstitial infiltrates –> sarcoidosis is present in both the lymph nodes and the lung tissue
Stage 3 = Diffuse interstitial infiltrates only –> granulomas are only found in the lung tissue
Stage 4 = Diffuse fibrosis –> there is scarring in the tissues of the lungs indicating irreversible damage.
Indications for steroids
- Patients with CXR stage 2 or 3 who are symptomatic. Asymptomatic patients with stage 2 or 3 of disease do not require treatment.
- Hypercalcaemia
- Eye, heart or neuroscience involvement
