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Meded Flashcards 2020 > Vasculitis and Autoimmune disease > Flashcards

Vasculitis and Autoimmune disease Flashcards

1
Q

Sarcoidosis

Definition

Epidemiology

Symptoms and signs

Investigations

Management

A

Sarcoidosis

Definition

Multi system granulamtous inflammatory disease, characterised by non caseating granulomas

Epidemiology

Afro Carribeans

Scandanavians

Age- >50

Symptoms and signs

Lungs- dry cough, dyspnea [causes lung fibrosis]- Ninety percent of symptoms

General: fever, malaise and weight loss

Musc: arthralgia

Skin: erythema nodosum, lupus pernio

Eyes: uveitis, keratoconjuncitivis

Cardio: arrthymia, heart failure

Investigations

Bloods:

FBC

U + E

Ca- high

ACE- high

ESR- high

CXR:

Bilateral hilar lymphadenopathy

Pulmonary fibrosis

BIOPSY: non caseating granuloma

Management

Steroids

NSAIDS

Steroid sparing agents

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2
Q

SLE

Definition

Epidemiology

Symptoms and signs

Investigations

A

SLE

Definition

Multisystem autoimmune inflammatory disorder

Epidemiology

Females

Young

Other autoimmune disorders

Afro Carribean

Symptoms and signs

SOAP BRAIN MD

Serositis

Oral ulcers

Arthritis

Photosensitivity

Bloods- low blood cell counts

Renal failure

Antibodies - ANA

Immunological- other antibody- anti-ds DNA

Neurological symptoms- psychosis, seizures

Malar rash

Discoid rash

Investigations

  • Bloods:

FBC

U and E

ESR - high

Autoantibodies:

ANA

Anti ds DNA

Anti cardiolipin

  • Urine:
  • casts
  • proteinuria
  • haematuria
  • CXR
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3
Q

What are the three categories of vasculitides and which conditions come under them?

A

Large vessel vasculitides

Giant cell arteritis

Polymyalgia Rheumatica

Medium vessel vasculitides

Polyarteritis nodosa

Small vessel vasculitides

Behcet’s disease

Granulomatosis with polyangitis [Wegener’s]

Eosinophilic Granulomatosis with Polyangitis [Churg-Strauss]

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4
Q

Systemic Sclerosis [Scleroderma]

Definition

Types - definition, symptoms, antibody

A

Systemic Sclerosis [Scleroderma]

Definition

Rare connective tissue disease characterised by fibrosis in skin + organs + widespread blood vessel damage

Types

  • Diffuse cutaneous systemic sclerosis

- Widespread, all over skin, affects multiple organs

Symptoms:

Skin changes - trunk

Heart, GI, renal disease

Early lung disease

Tendon friction

Raynaud’s phenomenon

Antibody:

anti- topoisomerase II / anti_Scl 70

  • Limited cutaneous systemic sclerosis
  • limited to certain systems

Also known as CREST syndrome

Symptoms:

Calcinosis- high calcium

Raynaud’s phenomenon

Esophageal dysmotility

Sclerodactyly {thick tight skin around fingers + hard bumps on fingers}Teleangiectasia

Antibody:

  • Anti centromere
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5
Q

SBA 1

A 40-year-old Afro-Caribbean man has suffered from shortness of breath and a dry cough for the last 3 months. He also complains of some ‘sore lumps on his shins’. Closer inspection reveals tender violet nodules on both shins. A chest X-ray is requested, which shows bilateral hilar lymphadenopathy. Blood tests are also requested, including U&Es – which parameter would you expect to be raised?

A Sodium
B Potassium

C Calcium
D pH
E Urea

A

C Calcium

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6
Q

SBA 2

33-year-old female with SLE presents to the fertility clinic complaining that she has been desperately trying to start a family but has had repeated miscarriages. She has had 3 miscarriages in the past 5 years. She has a past medical history of asthma and two DVTs. Given the likely diagnosis, which of the following antibodies is associated with this disease?

A Anti-CCP antibody

B Anti-Jo-1 antibody

C Anti-centromere antibody

D Anti-cardiolipin antibody

E Anti-smooth muscle antibody

A

D Anti cardiolipin antibody

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7
Q

SBA 3

A 58-year-old woman presents to her GP complaining of difficulty swallowing which started 6 months ago. On examination, the skin on her hands appears thickened and tight around her fingers. Furthermore, a hard lump is felt under the skin on her left thumb. Which of the following additional clinical features would support a diagnosis of limited cutaneous systemic sclerosis?

A Telangiectasia

B Thickened skin on the chest

C Macroglossia

D Buccal pigmentation

E Cherry haemangioma

A

A Telangiectasia

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8
Q

Features of anti phospholipiid syndrome?

+ antibody involved?

+ condition it’s related to?

A
  • Triad

o Recurrent miscarriages

o Thromboembolism/VTE- PE/DVT

o Thrombocytopenia

  • Antibody= anti cardiolipin
  • Complication of SLE
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9
Q

Features of diffuse cutaneous systemic sclerosis

+ antibody involved

A
  • Skin changes on trunk
  • Heart, GI and renal disease
  • Early lung disease
  • Tendon friction
  • Raynaud’s phenomenon

Antibody: anti-topoisomerase II or anti Scl-70

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10
Q

Features of Limited Cutaneous Systemic Sclerosis/CREST syndrome

+ antibody involved

A
  • Calcinosis
  • Raynaud’s phenomenon [hypoperfusion of fingers- go white, then red?]
  • Esophageal dysmotility
  • Sclerodactyly
  • Telangiectasia

Antibody- anti centromere

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11
Q

Vasculitides [in general]

Definition

Aetiology

Types

A

Vascultides

Definition:

Inflammation of the walls of blood vessels.

Systemic Effects.

Distinctive features.

Aetiology

Autoimmune, antibody mediated?

Often unknown

Types

Large vessel

Medium vessel

Small vessel

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12
Q

Vasculitides SBA 1

63-year-old woman presents to A&E with a headache, mainly affecting the left half of her forehead, that has gradually been getting worse over the past week. She has been eating less as she experiences pain in her jaw when she chews her food. She has, generally speaking, been healthy aside from experiencing some stiffness and pain in her shoulders over the past 6 months. What is the first step in her management?

A Check ESR

B Temporal artery biopsy

C IV hydrocortisone

D Oral prednisolone

E IV antibiotics

A

A Check ESR

Diagnosis likely temporal arteritis/GCA

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13
Q

Which condition is strongly associated with temporal arteritis?

[hint below- multiple choice]

A Takayasu’s aortitis

B Myalgic encephalomyelitis

C Fibromyalgia

D Polymyalgia rheumatica

E Polymyositis

A

D Polymyalgia rheumatica

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14
Q

Vasculitides SBA 3

47 y old man

Presents to GP

Skin rash

Feeling generally unwell for past three months

Abdo pain with rectal bleeding

On CT angiogram: Rosary sign

Regularly attends hospital for check ups since diagnosis of chronic hep B two years ago

What is the most likely diagnosis?

A Giant cell arteritis

B Dermatomyositis

C Polyarteritis nodosa

D Granulomatosis with polyangiitis

E Behcet’s disease

A
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15
Q

Giant cell arteritis

Definition

Aetiology

Symptoms [and signs]

Investigations

Treatment

Complications

A

Giant cell arteritis

Definition

Large vessel vasculitis

Associated with polymyalgia rheumatica

Red flag cause of headache

Aetiology

Idiopathic

Temporal artery inflammation

Epidemiology

Old

Female

Symptoms [and signs]

Unilateral headache - triggered by touching/brushing hair

Thickened temporal artery

Scalp tenderness

Jaw claudication- [pain worse on eating/talking]

Loss of vision [temporary-amaurosis fugax]

Systemic signs- malaise, fever, weight loss

Symptoms of polymyalgia rheumatica [bilateral morning shoulder and pelvic pain]

Investigations

Check ESR [before starting steroid treatment, but then start steroids ASAP]

Temporal artery biopsy

  • do in multiple regions- because often damage only seen in certain parts, so not v sensitive- skip lesions not continuous

[biopsy shows giant cells]

Treatment

High dose oral prednisolone ASAP

[Don’t delay treatment- could lead to permanent loss of sight]

Complications

Permanent loss of sight

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16
Q

Polymyalgia Rheumatica

Definition

Background

Symptoms and signs

Investigations

Management

A

Polymyalgia Rheumatica

Definition

Joint pain disorder associated with GCA [fifteen % of patients will get GCA]- chronic

Symptoms and signs

Chronic

Bilateral shoulder pain

Bilateral pelvic pain

Morning stiffness

For more than 6 weeks

No weakness

Investigations

ESR- high

CRP- high

Management

Steroids- oral

Analgesia initially?

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17
Q

Polyarteritis Nodosa

Definition

Aetiology

Associated disease

Symptoms and signs

A

Polyarteritis Nodosa

Definition

Aetiology

Idiopathic

Associated disease

  • Hep B

Symptoms and signs

  • Rash
  • Abdominal pain
  • Rectal bleeding
  • Renal failure
  • Hypertension
  • Peripheral neuropathy
  • Fever, malaise, lethargy

Rosary sign

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18
Q

Vasculitides SBA 5

45-year-old man from Cyprus presents with recurrent ulcers on his penis. He has not noticed any discharge from his penis or pain whilst urinating. He adds that he has also developed mouth ulcers several times over the past year. During the consultation, you notice that his eyes are quite red. When questioned, he says that his eyes have been itchy recently, and thinks that he might have hay fever.

What is the most likely diagnosis?

A Inflammatory bowel disease

B Behçet’s disease

C Herpes simplex virus

D Syphilis

E Reactive arthritis

A

B Behçet’s disease

19
Q

Vasculitides SBA 4

A 52-year-old man has suffered from rhinitis and recurrent nosebleeds for the past 5 months. Initially, he did not think much of it, until he began coughing up a small about of blood about 3 weeks ago. A urine dipstick reveals proteinuria and haematuria. Blood tests reveal:

ESR: 72 mm/hr (< 20 mm/hr)

cANCA: positive

What is the most likely diagnosis?

A Microscopic polyangiitis

B Goodpasture’s syndrome

C Granulomatosis with polyangiitis

D Churg-Strauss syndrome

E Behçet’s disease

A

C Granulomatosis with polyangitis [also known as Wegener’s]

20
Q

Vasculitides SBA 6

Churg-Strauss syndrome is associated with:

A pANCA

B cANCA

C Anti-GBM antibodies

D Anti-LKM antibodies

E Anti-smooth muscle antibodies

A

B cANCA

21
Q

Eosinophilic granulomatosis with polyangitis [Churg Strauss]

Definition

Antibody type

Symptoms and signs

Investigations

A

Eosinophilic granulomatosis with polyangitis [Churg Strauss]

Definition

Small vessel vasculitis which causes high eosinophil infiltration

Antibody type

pANCA

[EosinoPhil}

Symptoms and signs

Triphasic:

Allergic: asthma, rhinitis

Eosinophilic: tissue damage in lungs and GI tract

Vasculitis: widespread organ damage and death

Other symptoms:

Haemoptysis

Kidney damage

Rash

Local neuropathy

Investigations

pANCA

22
Q

Granulomatosis with polyangitis [Wegener’s]

Definition

Symptoms and signs

Investigations

- antibody

A

Granulomatosis with polyangitis [Wegener’s]

Definition

Small vessel vasculitis, affects lungs and kidneys

Symptoms and signs

Upper resp: rhinitis, nose bleed

Lower resp: haemoptysis

Kidneys: haematuria, proteinuria

Saddle nose

Investigations

cANCA

CXR- cavitating lesions

23
Q

Behcet’s disease

Definition and background

-

-

Epidemiology

-

Symptoms and signs

Investigations

A

Behcet’s disease

Definition and background

  • Small vessel vasculitis
  • HLA B51

Epidemiology

  • Medittereanean- Greece and Cyprus

Symptoms and signs

TRIAD:

Oral ulcers

Genital ulcers

Uveitis

Also [a bunch of inflammation]:

Rash- eg erythema nodosum

Arthritis

Pericarditis

Colitis

Investigations

Clinical diagnosis

Pathergy test- needle prick becomes inflamed and sterile pustule forms in 48 hr

24
Q

Large vessel vasculitis- fill in table

A
25
Q

Medium vessel vasculitis table- fill in

A
26
Q

Small vessel vasculitis tables- fill in

A

If in doubt for small vessel vasculitis: remember-

overall likely to affect:

Lungs

Kidneys

GI tract

Rash

Rhinitis

27
Q

Unrelated difficult SBA courtesy of Laz

43-year-old gardener is brought into A&E after having a seizure. His seizure lasts about 2 mins and disappears by itself. He says that this has never happened before and his only health problem has been a persistent headache that has developed over the past 6 months. On examination, a single heavily pigmented skin lesion is noticed on his right forearm. An MRI head scan reveals several lesions in both hemispheres.

What is the most likely diagnosis?

A Neurofibromatosis Type 1

B Meningioma

C Tuberous sclerosis

D Metastases

E Glioblastoma multiforme

A

D Metastases

Melanoma tend to have brain mets

Just because neuro signs and skin lesions doesn’t mean neurofibromatosis

Symptoms suggest space occupying lesions - persistent headaches for 6 months + focal neurological signs: seizures, parasthesia, changes in speech, vision and hearing

Majority of brain tumours are mets

Skin lesion likely to be a melanoma

Gardener means likely to have risk factor of high UV exposure

Glioblastoma multiforme and meningoma tend to be single discrete lesions

Tuberous sclerosis is a hereditary condition that leads to the formation of multiple benign tumours across the body. It is an autosomal dominant condition so most people with tuberous sclerosis will be aware that they have it and will not be presenting at the age of 43.

Neurofibromatosis type 1 tends to cause fewer central nervous system lesions and has more widespread peripheral features, such as neurofibromas, café au lait macules (> 5 is significant), axillary freckling, Lisch nodules and spinal scoliosis.

28
Q

SBA

51-year-old secretary visits her GP complaining that her shoulders are painful and that she has found it difficult to lift herself out of her chair at work. She has not, however, experienced any difficulty typing on her computer. An upper limb neurological examination is performed, shoulder abduction is 3/5 bilaterally. She has no other symptoms.

Which investigation is most likely to provide a definitive diagnosis?

A Autoantibody screen

B Creatine kinase

C EMG

D CRP

E Muscle biopsy

What is the likely condition?

A

E Muscle biopsy

Polymyositis

29
Q

What is the difference between dermatomyositis and polymyositis?

A
30
Q

SBA

A 46 year old woman presents with symmetrical PIP and DIP finger swelling and erythematous scaly plaques over the same joints. She has also noticed a purple coloured swelling/ rash on her upper eyelids. Lab results reveal raised ESR, raised CRP, and raised CK (Creatine Kinase). ANA positive, Anti-Mi2 antibodies positive

What is the most likely diagnosis?

A.Rheumatoid Arthritis

B.SLE

C.Dermatomyositis

D.Haemochromatosis

E.Polymyositis

A

C Dermatomyositis

Picture shows: Gottron’s papules

Rash around eyes is called: Heliotrope rash

31
Q

Non ANCA related small vessel vasculitis- fill in table

A

Anti GBM= antibody against glomerular basement membrane

32
Q

Dermatomyositis

Definition

Symptoms/Main features

A

Dermatomyositis

Definition

Idiopathic inflammatory myopathy characterised by cutaneous skin lesions

Symptoms/key features

Heliotrope rash- purple rash on eye

Gottron’s papules- [see other flashcards for picture]

Skin lesions are frequently the initial presenting complaint, with muscle involvement developing later.

33
Q

Polymyositis

Definition

A

a connective tissue disease characterised by inflammation of the muscles.

Differs from dermatomyositis in that polymyositis does not cause a rash.

Patients experience diffuse weakness in the proximal muscles (distal muscles tend to be spared).

34
Q

Takayasu’s arteritis

Definition

Aetiology/risk factors

Epidemiology

Symptoms

Signs

Investigations

A

Takayasu’s arteritis

Definition

Large vessel vasculitis in aortic arch

Epidemiology

Female

< Forty years old

Asian

Genetic predisposition

Symptoms

Limb claudication

Chest pain

Systemic: fever, lethargy, myalgia etc.

Signs

Bruits

Unequal blood pressure in both arms

High ESR/CRP

Absent/weak pulses

Investigations

35
Q

Microscopic polyangitis

Definition

Aetiology/risk factors

Epidemiology

Symptoms

Signs

Investigations

A

Microscopic polyangitis

Definition

Small vessel vasculitis

Aetiology/risk factors

Unknown

Epidemiology

50 years average onset

More males but both sexes affected

Symptoms and Signs

Glomerulonephritis

Haemoptysis

Skin rash

Joint pain

Investigations

pANCA

36
Q

What does this guy have?

A

wegner’s granulomatosis

37
Q

what does this guy have

A

wegner’s granulomatosis

38
Q

What does this guy have?

A

Behcet’s

39
Q

What is this sign?

What is it indicative of?

A

Rosary sign

polyarteritis nodosa

40
Q

What does this guy have?

What is it associated with

A

polymyaligia rheumatica

temporal arteritis

41
Q

what does this guy have?

A

sarcoidosis

42
Q

What does this lady have?

What is she at higher risk of getting?

A

Malar rash - SLE

Antiphospholipid syndrome

43
Q

What are these antibodies prominent in which condition

A

Meded Flashcards 2020 (36 decks)

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