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Y3 Medicine: Respiratory > Sarcoidosis > Flashcards

Sarcoidosis Flashcards

1
Q

What is sarcoidosis?

A

Sarcoidosis is a chronic granulomatous disorder of unknown aetiology, commonly affecting the lungs, skin, and eyes. It is characterised by accumulation of lymphocytes and macrophages and the formation of non-caseating granulomas in the lungs and other organs. Although lungs and lymph nodes are involved in more than 90% of patients, virtually any organ can be involved.

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2
Q

Who is commonly affected by sarcoidosis?

A

Usually affects adults aged 20–40yrs, more common in women. There are two spikes in incidence, in young adulthood and again around age 60.

African–Caribbeans are affected more frequently and more severely than Caucasians, particularly by extra-thoracic disease.

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3
Q

What is the typical patient who presents with sarcoidosis?

A

The typical MCQ exam patient is a 20-40 year old black woman presenting with a dry cough and shortness of breath. They may have nodules on their shins suggesting erythema nodosum.

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4
Q

What are the risk factors for sarcoidosis?

A
  • Age 20-40
  • FHx of sarcoidosis
  • Associated with HLA-DRB1 and DQB1 alleles
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5
Q

What organ systems can be affected by sarcoidosis?

A

Sarcoidosis can affect almost any organ in the body. The most commonly affected are the lungs so sarcoidosis is usually managed by respiratory physicians.

Extra-pulmonary manifestations:

  • Liver
  • Eyes
  • Skin
  • Heart
  • Kidneys
  • CNS
  • PNS
  • Bones
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6
Q

How does sarcoidosis affect the lungs?

A
  • Mediastinal lymphadenopathy
  • Pulmonary fibrosis
  • Pulmonary nodules
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7
Q

What are the systemic symptoms of sarcoidosis?

A
  • Fever
  • Fatigue
  • Weight loss
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8
Q

How does sarcoidosis affect the liver?

A
  • Liver nodules
  • Cirrhosis
  • Cholestasis
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9
Q

How does sarcoidosis affect the eyes?

A
  • Uveitis
  • Conjunctivitis
  • Optic neuritis
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10
Q

How does sarcoidosis affect the skin?

A
  • Erythema nodosum (tender, red nodules on the shins caused by inflammation of the subcutaneous fat)
  • Lupus pernio (raised, purple skin lesions commonly on cheeks and nose)
  • Granulomas develop in scar tissue
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11
Q

How does sarcoidosis affect the heart?

A
  • Bundle branch block
  • Heart block
  • Myocardial muscle involvement
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12
Q

How does sarcoidosis affect the kidneys?

A
  • Kidney stones (due to hypercalcaemia)
  • Nephrocalcinosis
  • Interstitial nephritis
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13
Q

How does sarcoidosis affect the CNS?

A
  • Nodules
  • Pituitary involvement (diabetes insipidus)
  • Encephalopathy
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14
Q

How does sarcoidosis affect the PNS?

A
  • Facial nerve palsy
  • Mononeuritis multiplex
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15
Q

How does sarcoidosis affect the bones?

A
  • Arthralgia
  • Arthritis
  • Myopathy
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16
Q

What are the signs of sarcoidosis?

A
  • Wheeze
  • Rhonchi
  • Lymphadenopathy
  • Erythema nodosum
  • Lupus pernio
  • Conjunctival nodules
  • Facial palsy
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17
Q

What are the symptoms of sarcoidosis?

A
  • Cough
  • Dyspnoea
  • Chronic fatigue
  • Arthalgia
  • Blurred vision
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18
Q

What % of sarcoidosis diagnoses are found incidentally?

A

In 20–40%, the disease is discovered incidentally, after a routine cxr, and is thus asymptomatic.

19
Q

What investigations should be ordered for sarcoidosis?

A
  • CXR
  • Serum ACE
  • Serum calcium
  • CRP
  • High-resolution CT
  • Flexible bronchoscopy with transbronchial lung biopsy
  • Skin biopsy
  • MRI
  • PET scan

Tests for other organ involvement: U&E, urine dipstick, LFTs, opthamology, ECG, echocardiogram and ultrasound.

20
Q

Why investigate using CXR?

A

90% have abnormal CXRs with bilateral hilar lymphadenopathy ± pulmonary infiltrates or fibrosis.

21
Q

What is shown on the CXR?

A

PA chest radiograph showing bilateral hilar lymphadenopathy. The important differentials for this appearance are: sarcoidosis, tb, lymphoma, pneumoconioses, and metastatic disease. This patient has sarcoidosis but there are no other stigmata (such as the presence of infiltrates, fibrosis, and honeycombing) on this image.

22
Q

Why investigate serum ACE?

A

Elevated in about half of patients. This is often used as a screening test.

23
Q

Why investigae serum calcium?

A

Due to dysregulated production of calcitriol by activated macrophages and granulomas.

Hypercalcaemia.

24
Q

Why investigate CRP?

A

Raised CRP.

25
Q

Why investigate using high-resolution CT?

A

High-resolution CT thorax shows hilar lymphadenopathy and pulmonary nodules.

26
Q

Why investigate using flexible bronchoscopy with transbronchial lung biopsy?

A

The gold standard for confirming the diagnosis of sarcoidosis is by histology from a biopsy. This is usually done by doing bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes.

The histology shows characteristic non-caseating granulomas with epithelioid cells.

27
Q

Why investigate using skin biopsy?

A

Skin biopsy of lesion suspicious for sarcoidosis may histologically confirm diagnosis.

Presence of non-caseating granulomas.

28
Q

Why investigate using MRI?

A

May be useful in identifying areas of sarcoidosis involvement, including the heart and the brain.

Heart findings include nodular lesions and/or focal myocardial thickening.

Brain findings are varied and non-specific, and include meningeal enhancement with or without periventricular white matter lesions.

29
Q

Why investigate using PET scan?

A

PET scan can show active inflammation in affected areas.

30
Q

What other tests can be used to investigate other organ involvement in sarcoidosis?

A

U&Es for kidney involvement.

Urine dipstick or urine albumin-creatinine ratio to look for proteinuria indicating nephritis.

LFTs for liver involvement.

Ophthalmology review for eye involvement.

ECG and echocardiogram for heart involvement.

Ultrasound abdomen for liver and kidney involvement.

31
Q

What is the first line in patients with no or mild symptoms?

A

Patients with bilateral hilar lymphadenopathy alone don’t need treatment as most recover spontaneously.

Acute sarcoidosis: bed rest and NSAIDs.

32
Q

What are the indications for corticosteroids?

A
  • Parenchymal lung disease (symptomatic, static, or progressive)
  • Uveitis
  • Hypercalcaemia
  • Neurological or cardiac involvement
33
Q

Briefly describe the use of corticosteroids in management of sarcoidosis

A

Oral steroids (e.g. prednisolone) are usually first line where treatment is required and are given for between 6 and 24 months. Patients should be given bisphosphonates to protect against osteoporosis whilst on such long term steroids.

Second line options are methotrexate or azathioprine

34
Q

When is lung transplantation needed?

A

Lung transplant is rarely required in severe pulmonary disease.

35
Q

What is the prognosis of sarcoidosis?

A

Sarcoidosis spontaneously resolves within 6 months in around 60% of patients.

In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant. Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system.

36
Q

Give examples of causes of bilateral hilar lymphadeopathy

A
  • Sarcoidosis
  • Infection (e.g. TB and mycoplasma)
  • Malignancy (e.g. lymphoma, carcinoma and mediastinal tumours)
  • Organic dust disease (e.g. silicosis and berylliosis)
  • Hypersensitivity pneumonitis
  • Histocytosis x (Langerhan’s cell histiocytosis).
37
Q

What is Lofgren’s Syndrome?

A

This is a specific presentation of sarcoidosis. It is characteristic by a triad of:

  • Erythema nodosum
  • Bilateral hilar lymphadenopathy
  • Polyarthralgia (joint pain in multiple joints)
38
Q

What are the complications of sarcoidosis?

A
  • Severe or life-threatening haemoptysis
  • Corticosteroid related hyperglycaemia
  • Pulmonary related hypertension
  • Corticosteroid osteoporosis
39
Q

What differentials should be considered for sarcoidosis?

A
  1. Tuberculosis
  2. Lymphoma
  3. Hypersensitivity pneumonitis
  4. Histoplasmosis
40
Q

How does sarcoidosis and TB differ?

A

Differentiating signs and symptoms:

  • History of exposure or high-risk patients.
  • Differential diagnosis is especially challenging when patients present with upper lobe infiltrates, fever and weight loss

Differentiating investigations:

  • Positive PPD
  • Smear or culture positive for TB
  • Caseating granulomas on lung biopsy
41
Q

How does sarcoidosis and lymphoma differ?

A

Differentiating signs and symptoms:

  • Extrathoracic involvement is more common (cervical and supraclavicular lymphadenopathy)

Differentiating investigations:

  • Biopsy of mediastinal lymph nodes shows features of lymphoma
42
Q

How does sarcoidosis and hypersensitivity pneumonitis differ?

A

Differentiating signs and symptoms:

  • History of exposure to one of a multitude of agents (e.g., birds’ feathers and droppings, compost, peat moss, metalworking fluids)
  • Best described in farmers exposed to mouldy hay

Differentiating investigations:

  • Positive precipitins to known antigens
  • CT chest shows poorly defined nodules in the acute phase and fibrosis with bronchiectasis in the chronic form
  • Poorly formed granulomas and intra-alveolar foci of organising pneumonia on lung biopsy
43
Q

How does sarcoidosis and histoplasmosis differ?

A

Differentiating signs and symptoms:

  • History of living in an endemic area (e.g., the Mississippi River valley)

Differentiating investigations:

  • Fungus isolation on silver stain from lung biopsy
  • Positive urinary Histoplasma antigen
  • The most common finding on CXR is pulmonary nodules; can also present as infiltrates, lymphadenopathy, fibrosis, and thickened pleura

Y3 Medicine: Respiratory (15 decks)

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