Sarcoidosis Flashcards

1
Q

Epidemiology of sarcoidosis.

A

Highest in northern europe.

Usually affects adults aged 20-40 years more common in women.

Associated with HLA-DRB1 alleles.

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2
Q

How is sarcoidosis often detected?

A

Incidentally on route CXR

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3
Q

Explain the immunopathology in sarcoidosis.

A

Typical sarcoid granulomas consisting of focal accumulations of epitheloid cells, macrophages and lymphocytes, mainly T cells.

There is a depressed cell mediated reactivity to tuberculin.

Overall lymphopenia but B cells can be raised.

Bronchoalveolar lavage shows a great increase in the overall number of cells.

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4
Q

Define sarcoidosis.

A

A multisystemic disorder with chronic inflammation and granuloma formation.

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5
Q

Symptoms of 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.

Asymptomatic

Fatigue

Weight loss

Aches and pains

Arteritis

Dyspnoea

Dry eyes

Cough

Extertional dyspnoea

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6
Q

Signs of sarcoidosis.

A

Erythema nodosum

Lymphadenopathy

Rashes

Hepatomegaly

Arrhytmia

Anaemia

Nerve palsy

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7
Q

Common general findings in sarcoidosis.

A

Bilateral lymph node enlargement

Erythema nodosum in forms of a maculopapular rash

Nodularity of the trachea

Bluish discoloration of nose

Pulmonary infiltration

Uveitis in eyes

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8
Q

Organs involved in sarcoidosis.

A

Lungs, bronchi and trachea (90%)

Liver (50-80%)

Lymph nodes (70%)

Eyes

Skin (25%)

Heart (30%)

Spleen (18%)

Brain (5%)

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9
Q

What do people with acute sarcoidosis generally present with?

A

Fever

Erythema nodosum

Polyarthralgia and bilateral hilar lymphadenopathy

This is also called Löfgren syndrome and usually resolves spontaneously.

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10
Q

Non-pulmonary signs of sarcoidosis.

A

Lymphadenopathy

Hepatomegaly

Splenomegaly

Uveitis

Conjunctivitis

Bell’s palsy

Neuropathy and meningitis

Erythema nodosum

Arrhythmias

Hypercalcaemia

Hypercalciuria

Renal stones

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11
Q

Most common cause of erythema nodosum

A

Sarcoidosis

Can also be idiopathic or due to bacterial infection.

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12
Q

Explain erythema nodosum.

A

Usually found in young adult women.

Commonly found on shins and face.

There are bilateral deep tender nodules that are pre-tibial.

The overlying skin is shiny red.

It is called a maculopapular rash.

There can be onset of acute arthralgia, malaise and oedema.

After 2-3 days the lesions flatten and will show up as a bruised appearance.

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13
Q

Diagnostic criteria of sarcoidosis.

A

Clinical findings

Exclude differentials

Evidence of non-caseating granulomas on lymph node biopsy of bilateral lymph node enlargement.

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14
Q

Differentials of bilateral hilar lymphadenopathy.

A

Sarcoidosis

TB

Lymphoma

Lung cancer

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15
Q

Investigations of sarcoidosis.

A

Bloods - ESR, lymphopenia, LFTs, serum ACE, Ca2+, immunoglobulins

24h urine for Ca2+

CXR

HRCT

ECG

PFTs

Tissue biopsy

Bronchoalveolar lavage

USS of kidneys and liver/spleen

Bone x-ray

MRI

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16
Q

Blood findings in sarcoidosis.

A

Raised serum ACE. This is often used as a screening test.

Raised LFTs

Hypercalcaemia is a key finding.

Raised serum soluble interleukin-2 receptor

Raised CRP

Raised immunoglobulins

17
Q

24h urine findings in sarcoidosis.

A

Ca2+ raised

18
Q

Differentials of raised serum ACE.

A

Lymphoma

Pulmonary TB

Asbestosis

Silicosis

This makes raised serum ACE supportive but not diagnostic.

19
Q

ECG findings in sarcoidosis.

A

May show arrhythmias or bundle branch block.

20
Q

Radiological stages of sarcoidosis.

A

Stage 0 - Normal

Stage 1 - BHL

Stage 2 - BHL + peripheral pulmonary infiltrates

Stage 3 - Peripheral pulmonary infiltration alone

4 - Lung fibrosis, bulla formation (honeycomb) and pleural involvement.

21
Q

Findings on PFTs in sarcoidosis.

A

May be normal or show redcued lung volumes, restrictive pattern and reduced transfer factors.

22
Q

What is the diagnostic test of sarcoidosis?

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.

23
Q

Eye involvement in sarcoidosis.

A

Anterior uveitis

Keratoconjunctivitis sicca

24
Q

Neurological involvement in sarcoidosis.

A

Cognitive dysfunction

Nodules

Central diabetes insipidus

Headache

Cranial nerve palsies

Mononeuritis multiplex

Peripheral neruopathy

Seizures

25
Q

Cardiac involvement in sarcoidosis.

A

Arrhythmias

Heart block

Cardiomyopathy

Sudden death

26
Q

Kidney involvement in sarcoidosis.

A

Hypercalciuria

Nephrocalcinosis

Calculi

Tubulointerstitial nephritis

It can be secondary to hypercalcaemia or due to granulomas in kidneys.

27
Q

Hepatic involvement in sarcoidosis.

A

Deranged LFTs

Hepatomegaly

Liver nodules

Cirrhosis

Cholestasis

28
Q

Explain the indication of treatment in sarcoidosis.

A

Not all need treatment -> continous follow-up should be done -> acute sarcoidosis should be treated with bed rest and NSAIDs -> spontaneous remission is common.

However if there is progressive pulmonary disease, disfiguring skin disease, hypersplenism, uveitis, persistent hypercalcaemia or other organ involvement treatment should be considered.

29
Q

Give indications for corticosteroids.

A

Parenchymal lung disease that is symptomatic, static progressive.

Uveitis

Hypercalcaemia

Neurological or cardiac involvement

30
Q

Treatment of sarcoidosis indicating management.

A

Prednisolone 40mg OD PO for 4-6 weeks then decrease the dose over 1 year.

Patients should be given bisphosphonates to protect against osteoporosis whilst on such long term steroids.

31
Q

Treatment of severe sarcoidosis.

A

IV methylprednisolone or

Methotrexate, hydroxychloroquine, ciclosporin or cyclophosphamide or azathioprine.

Anti-TNFalpha treatment can be tried in refractory cases.

Lung transplant

32
Q

Complications of sarcoidosis.

A

Pulmonary fibrosis

Pulmonary respiratory failure

Cor pulmonale

Resp failure