Week Three - Case Three Flashcards
what is sarcoidosis
multisystem granulomatous disorder of unknown aetiology
what does sarcoidosis produce
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
what races are highest risk of sarcoidosis
black africans followed by Scandinavians
what age is the typical presentation
25-45 years
which parts of the body are most commonly affected
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
what percentage of patients are asymptomatic
50%
what are the chest associated symptoms
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
what are the range of non-pulmonary symptoms sarcoidosis presents with - these make it a differential diagnosis for almost any unidentifiable disease
Hepatomegaly, splenomegaly, lymphadenopathy, Bell’s palsy, uveitis, conjunctivitis and cataracts.
what are the skin signs and symptoms
Papules – often resembling Rosacea, or the malaria rash seen in SLE
Plaques that may mimic psoriasis
Erythema nodosum
what are the eye signs and symptoms
Occur in 20% of patients
Typically a uveitis
what are the neurological symptoms
Granulomas can form anywhere int he nervous system
Therefore – can cause any neurological sign
Sometimes cases Bell’s Palsy
what is a granuloma
collection of monocular cells and macrophages (WBCs) surrounded by lymphocytes, plasma cells, mast cells, fibroblasts and collagen.
where do granulomas tend to by distributed in the lung
along the line of lymph nodes
what would the blood results be in sarcoidosis
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)
what are the classical findings on X ray of sarcoidosis
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.
what would pulmonary function tests show
a reduced lung volume and restrictive pattern
what is the diagnosis confirmed by
tissue biopsy that will show the presence of non-caseating granuloma
what is the most common steroid used in sarcoidosis
prednisolone
what are patients who cannot take steroids given
anti-TNF drugs and monoclonal antibody agents
what are the eye manifestations of sarcoidosis treated with
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
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:
sarcoidosis
tuberculosis
lymphoma
what investigations are done to confirm diagnosis of sarcoidosis
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
what is ‘uveoparotid fever’ or ‘Heerfordt’s syndrome’ and what does it represent
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
what is the triad of Lofgrens syndrome
joint pain, erythema nodosum and hilarious lymphadenopathy
what would a HRCT show
diffuse micronodulatiry and bilateral fissures nodularity; no fibrosis
what is the CXR appearance for stage 1 sarcoidosis and what is the prognosis
hilar lymphendopathy only and may resolve spontaneously without treatment
what is the CXR appearance for stage 2 sarcoidosis and what is the prognosis
hilar lymphandeopathy and parenchymal involvement
may resolve spontaneously without treatment
what is the CXR appearance for stage 3 sarcoidosis and what is the prognosis
parenchymal involvement only and likely to progress and need treatment
what is the CXR appearance for stage 4 sarcoidosis
pulmonary fibrosis and is likely to progress and need treatment
why is it important to wean steroid treatment slowly rather than finish a course abruptly
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.
when do we treat sarcoidosis
treat only if organ threatening
