Sarcoidosis

Question 1

Sarcoidosis: What is it?

Answer 1

Sarcoidosis= achronic granulomatous disorder.Granulomasareinflammatorynodulesfull ofmacrophages. Has pulmonary and extrapulmonary manifestations.

Question 2

Sarcoidosis: Epidemiology

Answer 2

• Aged 20-39 or around 60
• Women
• Black ethnic origin

Question 3

Sarcoidosis: Symptoms

Answer 3

The symptoms that present depend on the organs affected:
Systemic Symptoms:
- Fever
- Fatigue
- Weight loss
Lungs:
- Mediastinal lymphadenopathy
- Pulmonary fibrosis
- Pulmonary nodules
Heart:
- Bundle branch block
- Heart block
- Myocardial muscle involvement
Bones:
- Arthralgia
- Arthritis
- Myopathy
Eyes:
- Uveitis
- Conjunctivitis
- Optic neuritis
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
Skin:
- Erythema nodosum – raised, red, and painful nodules ofinflamedsubcutaneous faton the shins. Inflammation of fat is calledpanniculitis. Over time the nodules settle and appear as bruises. Not specific to sarcoidosis as there are many other causes of erythema nodosum.
- Lupus pernio– raised purple skin lesions, often on the cheeks and nose.

Question 4

Sarcoidosis: Physical Examination

Answer 4

N/A

Question 5

Sarcoidosis: Investigations

Answer 5

• bmi
• spirometry will show restrictive pattern
• blood tests:
• The blood test findings to remember are:
• Raisedangiotensin-converting enzyme(ACE) (often used as a screening test)
• Raisedcalcium(hypercalcaemia) - 10%
• raised esr
• fbc
• 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
• lung biopsy for histology – shows showsnon-caseating granulomaswithepithelioid cells.
• • Chest x-raymay showhilar lymphadenopathy
• High-resolution CTscanning**may showhilar lymphadenopathyandpulmonary nodules
• MRIcan showcentral nervous system involvement
• PET scancan show active inflammation in affected areas

A chest x-ray may show the following changes:
stage 0 = normal
stage 1 = bilateral hilar lymphadenopathy (BHL)
stage 2 = BHL + interstitial infiltrates
stage 3 = diffuse interstitial infiltrates only
stage 4 = diffuse fibrosis

Question 6

Sarcoidosis: Management

Answer 6

Conservative managementis considered in patients with no or mild symptoms.

Oral corticosteroids(prednisolone for 6-24 months) are usually first-line where treatment is required. Bisphosphonatesprotect against osteoporosis whilst on long-term steroids. Indications for corticosteroid treatment: parenchymal lung disease, uveitis, hypercalcaemia and neurological or cardiac involvement

Methotrexateis a second-line option.

Lung transplantis rarely required in severe pulmonary disease.

Sarcoidosis spontaneously resolves in around half of patients, usually within two years.

Indications for steroids:
- symptomatic + CXR stage 2 or 3 disease
- asymptomatic + stable stage 2 or 3 disease + mildly abnormal lung function
- hypercalcaemia
- eye, heart or neuro involvement

Question 7

Sarcoidosis: Complications

Answer 7

• pulmonary fibrosis
• pulmonary hypertension.

Question 8

Sarcoidosis: Differential Diagnosis

Answer 8

The top differentials for the varied presenting features of sarcoidosis are:
- Tuberculosis
- Lymphoma
- Hypersensitivity pneumonitis
- HIV
- Toxoplasmosis
- Histoplasmosis

Question 9

Sarcoidosis: Prognosis

Answer 9

Factors associated with poor prognosis:
insidious onset, symptoms > 6 months
absence of erythema nodosum
extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
CXR: stage III-IV features
black African or African-Caribbean ethnicity

Question 10

Sarcoidosis: Syndromes

Answer 10

Lofgren’s syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis

In Mikulicz syndrome* there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma

Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis

*this term is now considered outdated and unhelpful by many as there is a confusing overlap with Sjogren’s syndrome