Week Three - Case Three Flashcards

1
Q

what is sarcoidosis

A

multisystem granulomatous disorder of unknown aetiology

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2
Q

what does sarcoidosis produce

A

often produces non-caveating epithelioid granulomas, which can occur at any site in the body.

they give the appearance of TB on a chest x-ray

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3
Q

what races are highest risk of sarcoidosis

A

black africans followed by Scandinavians

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4
Q

what age is the typical presentation

A

25-45 years

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5
Q

which parts of the body are most commonly affected

A

Lungs are most commonly affected, followed by eyes and skin.
The liver is also often affected, but this is rarely clinically significant
Rarely it an affect the heart and nervous system

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6
Q

what percentage of patients are asymptomatic

A

50%

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7
Q

what are the chest associated symptoms

A

Dry cough
Chest pain
Fever
Dyspnoea (usually progressive)
A restrictive pattern may be seen on spirometry
Lymphadenopathy
Crackles on auscultation
Fatigue
Malaise
Weight loss
Weakness

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8
Q

what are the range of non-pulmonary symptoms sarcoidosis presents with - these make it a differential diagnosis for almost any unidentifiable disease

A

Hepatomegaly, splenomegaly, lymphadenopathy, Bell’s palsy, uveitis, conjunctivitis and cataracts.

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9
Q

what are the skin signs and symptoms

A

Papules – often resembling Rosacea, or the malaria rash seen in SLE

Plaques that may mimic psoriasis

Erythema nodosum

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10
Q

what are the eye signs and symptoms

A

Occur in 20% of patients
Typically a uveitis

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11
Q

what are the neurological symptoms

A

Granulomas can form anywhere int he nervous system
Therefore – can cause any neurological sign

Sometimes cases Bell’s Palsy

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12
Q

what is a granuloma

A

collection of monocular cells and macrophages (WBCs) surrounded by lymphocytes, plasma cells, mast cells, fibroblasts and collagen.

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13
Q

where do granulomas tend to by distributed in the lung

A

along the line of lymph nodes

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14
Q

what would the blood results be in sarcoidosis

A

FBC – may cause anaemia or raised WCC

ESR – often raised

U+E

LFTs – may be derranged

Serum ACE (angiotensin converting enzyme)
Elevated in 60% of cases
Falls with treatment
Not routinely used for diagnosis or monitoring of treatment (lung function and CXR are better indicators)

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15
Q

what are the classical findings on X ray of sarcoidosis

A

The classical finding is hilar lymphadenopathy, where there are clear lung fields, with ‘fluffy’ opacities in the hilar region. Other changes may include lung infiltrates and fibrosis.
It can also cause non-specific CXR changes.

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16
Q

what would pulmonary function tests show

A

a reduced lung volume and restrictive pattern

17
Q

what is the diagnosis confirmed by

A

tissue biopsy that will show the presence of non-caseating granuloma

18
Q

what is the most common steroid used in sarcoidosis

A

prednisolone

19
Q

what are patients who cannot take steroids given

A

anti-TNF drugs and monoclonal antibody agents

20
Q

what are the eye manifestations of sarcoidosis treated with

A

This may be treated with intra-ocular steroid injections (deeper than anterior chamber), or corticosteroid eye-drops (shallower disease)

Sarcoidosis can cause optic neuritis which can cause blindness if untreated. In these cases, IV corticosteroids are usually given

21
Q

what are the differential diagnoses for the history of weight loss, fever and night sweats, and the CXR finding of bihilar lymphadenopathy are indicative of three possible diagnoses:

A

sarcoidosis
tuberculosis
lymphoma

22
Q

what investigations are done to confirm diagnosis of sarcoidosis

A

inflammatory markers (CRP/ESR)

CT scan of thorax, abdomen and pelvis

bronchoscopy with endo-bronchial ultrasound scan (EBUS), lymph node biopsy and bronchoalveolar lavage (BAL)

slit-lamp examination

23
Q

what is ‘uveoparotid fever’ or ‘Heerfordt’s syndrome’ and what does it represent

A

it is a combination of acute uveitis, fever, and parotid gland swelling

it represents a form of neurosarcoidosis and in some patients, a facial palsy may also be seen

24
Q

what is the triad of Lofgrens syndrome

A

joint pain, erythema nodosum and hilarious lymphadenopathy

25
Q

what would a HRCT show

A

diffuse micronodulatiry and bilateral fissures nodularity; no fibrosis

26
Q

what is the CXR appearance for stage 1 sarcoidosis and what is the prognosis

A

hilar lymphendopathy only and may resolve spontaneously without treatment

27
Q

what is the CXR appearance for stage 2 sarcoidosis and what is the prognosis

A

hilar lymphandeopathy and parenchymal involvement

may resolve spontaneously without treatment

28
Q

what is the CXR appearance for stage 3 sarcoidosis and what is the prognosis

A

parenchymal involvement only and likely to progress and need treatment

29
Q

what is the CXR appearance for stage 4 sarcoidosis

A

pulmonary fibrosis and is likely to progress and need treatment

30
Q

why is it important to wean steroid treatment slowly rather than finish a course abruptly

A

Administering exogenous steroids causes suppression of endogenous cortisol production by the adrenal glands. Abrupt withdrawal of exogenous steroids can therefore precipitate an adrenal crisis. A gradual reduction in exogenous steroid dose enables the adrenal glands to resume their normal function.

31
Q

when do we treat sarcoidosis

A

treat only if organ threatening

32
Q
A