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Chem Path > Immune related multisystem disorders COPY > Flashcards

Immune related multisystem disorders COPY Flashcards

1
Q

What is Ankylosing Spondylititis?

A 
Chronic spinal inflammation that can result in spinal fusion and deformity
Site of inflammation includes the enthesis
No autoantibodies (‘seronegative’)
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2
Q

What are the main features of SLE?

A

Chronic tissue inflammation in the presence of antibodies directed against self antigens
Multi-site inflammation but particularly the joints, skin and kidney
Associated with autoantibodies

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3
Q

What are some examples of connective tissue diseases?

A 
Systemic lupus erythematosus
Sjogren’s syndrome
Autoimmune Inflammatory muscle disease
Systemic sclerosis (scleroderma)
Overlap syndromes
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4
Q

What are the key autoantibodies in SLE?

A

Antinuclear antibodies
Anti-double stranded DNA antibodies
Anti-phospholipid antibodies

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5
Q

What is Arthralgia?

A

Pain in joints
Tenderness but not obvious inflammation
Common in connective tissue disorders

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6
Q

What are the main features of autoantibodies?

A

Characteristic
May aid diagnosis
Correlate with disease activity
May be directly pathogenic

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7
Q

What is Raynaud’s phenomenon?

A

Intermittent vasospasm of digits on exposure to cold
Typical colour changes – white to blue to red
Vasospasm leads to blanching of digit
Cyanosis as static venous blood deoxygenates
Reactive hyperaemia

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8
Q

What are the main manifestations of Lupus?

A

Malar rash – erythema that spares the nasolabial fold
Photosensitive rash
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Arthralgia and sometimes arthritis
Serositis (pericarditis, pleuritis, less commonly peritonitis)
Renal disease – glomerulonephritis (‘lupus nephritis’)
Cerebral disease – ‘cerebral lupus’ e.g. psychosis, depression
Haematological - pancytopenia

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9
Q

Who typically gets Lupus?

A

female aged between 15 – 45 years

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10
Q

Which rheumatoid conditions are seronegative?

A

OA
Reactive arthritis
Gout
Ankylosing Spondylitis

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11
Q

What are the key investigations for Lupus?

A

ESR
CRP is usually normal
Autoantibodies (ANA - immunofluroscent)
Antiphospholipid antibodies

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12
Q

What haematological investigations are done in Lupus?

A

Haemolytic anaemia, Lymphopenia, Thrombocytopenia

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13
Q

What renal i

investigations are done in Lupus?

A

very important to measure urine protein (most commonly urine protein:creatinine ratio [uPCR])
look at albumin

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14
Q

How can we measure disease activity in SLE?

A

Immune complexes

Unwell lupus patient has LOW complement C3 and C4 levels and HIGH levels of anti-ds-DNA antibodies

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15
Q

What is the aim of SLE treatment?

A

Treatment in SLE aims at remission or low disease activity and prevention of flares

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16
Q

What should patients with SLE be assessed for?

A

antiphospholipid antibody status

infectious and cardiovascular diseases risk profile

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17
Q

How is SLE treated?

A

Hydroxychloroquine is recommended in all patients with lupus

Maintenance treatment glucocorticoids should be minimised and, when possible, withdrawn.

Appropriate initiation of immunomodulatory agents (methotrexate, azathioprine, mycophenolate) can expedite the tapering/discontinuation of glucocorticoids

In persistently active or severe disease we use cyclophosphamide and B cell targeted therapies (rituximab and belimumab)

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18
Q

What needs to be considered re management in Lupus patients?

A

Pregnancy planning

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19
Q

What is Sjorgren’s syndrom?

A

Autoimmune exocrinopathy

lymphocytic infiltration of especially exocrine glands and sometimes of other organs (extra-glandular involvement)

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20
Q

What does exocrine gland pathology result in?

A 
Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlarg
21
Q

What is inflammatory muscle disease?

A

Proximal muscle weakness due to autoimmune-mediated inflammation either with (dermatomyositis) or without (polymyositis) a rash

22
Q

What is systemic scelrosis?

A

Thickened skin with Raynaud’s phenomenon
Dermal fibrosis, cutaneous calcinosis and telangiectasia
Skin changes may be limited or diffuse

23
Q

What is overlap syndrome?

A

When features of more than 1 connective tissue disorder are present e.g. SLE and inflammatory muscle disease we can use the term overlap syndrome

24
Q

Describe the pathology of Malar rash?

A

Lymphocytic invasion of the upper dermis
Immune complex deposition at the dermis/epidermis junction

25
Q

How does SLE affect kidneys?

A

Thickening of Y-loop capillaries in glomeruli due to immune-complex deposition in the basement membranes

26
Q

How does SLE affect the heart?

A

Libman sacks - non-infective endocarditis
Present with emboli/stroke
Depositions on the valves

27
Q

What causes scleroderma aka systemic sclerosis?

A

Fibrous and excess collagen in the skin

28
Q

What are the two forms of Scelroderma? What antibodies are involved?

A

Diffuse form: Antibodies to DNA topoisomerase
(Trunk involvement)

Limited form: Anticentromere antibody
(No Trunk involvement)

29
Q

What are the limited form manifestations of scleroderma?

A

CREST
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasia

30
Q

What immunoflurosecnce pattern is seen in scleroderma?

A

Nucleolar immunofluorescence

31
Q

What is indicated by a speckled ANA pattern?

A

Mixed connective tissue disease

32
Q

What are some features of Dermatomyositis?

A

Tender inflamed muscles
Gottron’s papules (erythematous rash on dorsal aspect of hand on knuckles)
High CK

33
Q

Where in the body is affected by Sarcoidosis?

A

Joints
Skin
Lungs
Lymphadenopathy

Heart
Eyes
Neuro
Liver

34
Q

What are some cutaneous manifestations of sarcoidosis?

A

Lupus pernio (nasal lesion)
Erythem Nodosum (lower limb lesions)

35
Q

What CNS affects seen in sarcoidosis?

A

Meningitis
CN lesions

36
Q

What affects of sarcoid on the eyes?

A

Uveitis
Keratoconjuctivitis

37
Q

What are the affects of sarcoid on the lungs?

A

Bi-hilar lymphadenopathy
Lymphocytosis
Fibrosis

38
Q

How can sarcoid affect the liver?

A

Hepatitis
Cholestasis
Cirrhosis

39
Q

What happens to Ca in Sarcoidosis?

A

Hypercalcaemia
Vit D hydroxylation by activated macrophages

40
Q

How can we classify Vasculitis?

A

By the size of vessels:
Large vessel vasculitis
Medium vessel vasculitis
Immune complex small vessel vasculitis
ANCA-associated small vessel vasculitis

41
Q

What are some large cell vasculitides?

A

Giant cell arteritis - elderly w headache (jaw claudication, scalp tenderness)

Takayasu Arteritis

42
Q

What are some medium cell vasculitides?

A

Polyarteritis nodosa - commonly renal/mesenteric vessles (beading of vessels), Associated with Hep B

Kawasaki disease

43
Q

What are some ANCA-associated small cell vasculitides?

A

Granulamatosis with polyangitis (prev Wegners’)

Eosinophilc granulamatosis with polyangitis (prev Churg-Strauss)

44
Q

What rash is characteristic of vasculitis?

A

Palpable purpuric rash

45
Q

Diagnosis and management of temporal arteritis

A

Biopsy - granulomas, narrowing of lumen, lymphocytic infilitration of tunic media, multinuclear giant cells
ESR will be high
High dose steroids

46
Q

What are the features of polyarteritis nodosa?

A

Necrotising arteritis
Often renal/mesenteric arteries
Nodular appearance on angiography (small aneurysms)

47
Q

What are the features of granulmatosis with polyangitis?

A

ENT - nosebleeds
Lung - breathlessness
Kidneys - proteinuria/haematuria
c-ANCA directed against proteinase

48
Q

What are the features of eosinophilic granulomatosis with polyangitis?

A

Asthma
Eosiniphilia
Vasculitis
P-ANCA directed against myeloperoxidase

49
Q

What are the presenting features of Kawasaki’s disease?

A

Fever
Erythema of palms & soles
Conjunctivitis
Lymphadenopathy
Can affect coronary arteries (MI)

Chem Path (5 decks)

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