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Stage 4 > Multisystem disease > Flashcards

Multisystem disease Flashcards

1
Q

Autoantibodies

  1. SLE (Smooth double)
  2. Limited Systemic sclerosis
  3. Polymyositis (Joe Hart has shit muscles and shit lung - ILD)
  4. Dermatomyositis (MIkel has shit skin+muscles)
  5. Sjogrens syndrome (ROLO)
  6. Diffuse Systemic Sclerosis (Kidney)
  7. Diffuse Systemic Sclerosis (Lung)
  8. Mixed Connective Tissue Disease (erosive arthritis and pulmonary artery hypertension)
A
  1. ANA, Anti-Ds DNA, Anti-SM
  2. Anti-centromere
  3. Jo-1
  4. Anti-Mi
  5. Anti-Ro, Anti-La
  6. Anti-RNA polymerase III
  7. Anti-topoisomerase

K before L = R before T

  1. RNP
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2
Q 
Sjorgens syndrome - F>M 9:1
Cause
Sx
Dx
Mx
Complications
A

Lymphocytic infiltration of exocrine glands either primary or due to SLE, RA, CTD

Dry eyes, dry mouth, parotid gland enlargement

Sx + ANA/RF/Anti-Ro-LA (ROLO) + Schirmers test (tear production) + high ESR but normal CRP

Eye drops etc

Risk of lymphoma

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3
Q

Difference between type 2 and type 3 hypersensitivity reaction

A

Type 2 antibodies attack on fixed antigen
Type 3 antigen & antibodies that circulate all over the body

Type 2 is kind of like a sniper in action. You’ve got a set target and you’re sending a hitman (Ig) to do the damage…targeted damage, to targeted tissues! For example in goodpasture we just have kidney and lung involvement, nothing else.

Type 3 on the other hand is carpet bombing. You have immune complexes flying all over the place, activating complement and causing inflammation, and coagulation cascade activation and what not. The inflammation can happen pretty much anywhere where immune complexes go

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4
Q

IBD
Sx
Examination
Ix

A

Bowel: Diarrhoea w/ blood/mucus, pain, urgency, tenesmus

Systemic: Fever, weight loss, joint pain, episcleritis, uveitis, angular stomatitis (Fe deficiency), swollen cheeks + lower lip + mouth ulcers = orofacial granulomatosis (also sarcoidosis)

O/E: Clubbing, apthous ulcers, erythema nodosum, pyoderma gangrenosum, conjunctivitis, seronegative arthropathy (ank spond, large joints)

Bloods: anaemia (low ferritin), high platelets, low albumin etc

Stool: Faecal calprotectin

Imaging: AXR to exclude toxic megacolon

Generally colonscopy is good for UC

Need upper and lower endocscopy with barium/capsule/MRI for small bowel

ASCA = Crohns
pANCA = UC
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5
Q

Patients presents with swollen bottom lip, what do?

A

Biopsy:
Granuloma + multinucleated cells, lymphocytic infiltration = Crohns

Crypt abscesses + goblet cell deletion = UC

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6
Q

Leg Sx in IBD

A

UC = Pyoderma gangremosum

Crohns = Erythema nodosum

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7
Q 
Sarcoidosis - Female 20s-40s
Pathology
Sx
Dx
Mx

Acute form

A

Non-caseating granulomas

Lung: Bilateral hilar lymphadenopathy + infiltrates > cor pulmonale + fibrosis

MSK: Lower limb arthritis, dactylitis

Other: Uveitis, heart block (annual ECG+/- echo)n fever, night sweats, weight loss

  1. CXR + PFTs (Reduced DLCO) + ACE, hypercalcaemia (vit d from granuloma)
  2. HRCT
  3. Biopsy = Non-caseating granuloma with epithelioid + multinucleated giant cells

-caseating granuloma = TB

Acute:
Lofgrens Syndrome: Triad of Erythema nodosum + arthritis (bilateral ankle) + bilateral hilar adenopathy

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8
Q

Reactive arthritis/Reiters syndrome
Arthritis following an infection, typically male returning from Thailand with STI, conjuctivitis and arthritis
Sx within and outside MSK

A

Usually starts with lower limb joints (toes, ankles, knees) but can affect anywhere

Also affects tendons and can cause dactylitis

Non MSK:
Conjuctivitis
Scaly rash over hands and feet = keratoderma blenorrhagica
Diarrhoea
Mouth ulcers
Urethritis discharge
Penis rash
Weight loss + fever
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9
Q

Extra-cutaneous features of psoriasis

A 
Arthritis
Nail pitting
Onycholysis (separation of nail from nailbed)
Uveitis
Butterfly rash
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10
Q

Endocrine dermatology

  1. Striae, skin atrophy, acne
  2. Hyperpigmentation in palmar creases and buccal
  3. Periorbital oedema, dry skin, loss outer eyebrows
  4. Pretibial myxoderma, acropachy (clubbing), exophthalmos
A
  1. Cushings
  2. Addisons
  3. Hypothyroid
  4. Hyperthyroid
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11
Q

Metabolic dermatology

  1. Sun blisters, facial hirsutism
  2. Tendon xanthomas
  3. Eruptive xanthomas
  4. Acanthosis nigricans (armpit velvet), NECROBIOSIS LIPOIDICA (shin), candida
  5. Tophi/periarticular urate
A
  1. Porphyria
  2. Familial hypercholesterolemia
  3. High TGs so Familial Hypertriglyceridemia and to a lesser extent Remnant hyperlipidaemia
  4. Diabetes
  5. Gout
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12
Q

Haematological dermatology

  1. Neutrophilic dermatosis
  2. Pyoderma gangresnosum
  3. Mycosis Fungoides
A
  1. High neutrophils in the lesion, often associated with haem malignancy but also Sweet’s syndrome
  2. IBD (more UC), haem, RA, wegeners
  3. Cutaneous T cell lymphoma
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13
Q

Infective dermatology

  1. Red macular morbilliform rash, kopliks spots, profrome featuring 3C’s (coryza, cough, conjunctivitis)
  2. Vesicular viral rash
  3. 1’ chancre 2’ palms/soles 3’ gumma (nose ulcer)
  4. Erythema chronicum migrans ‘target lesion’
  5. Purpuric/vasculitis rash
A
  1. Measles virus
  2. chicken pox / shingles
  3. Syphillis
  4. Lyme disease
  5. Meninigits
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14
Q

Inflammatory dermatology

  1. Erythema nodosum
  2. Urethritis, uveitis, arthritis + psoriasiform rash (circinate balanitis + keratoderma blenorrhagica)
  3. Oral & genital ulcers, uveitis (triad), also lesions at injury site
  4. Lupus pernio (nose nodule)
  5. Photosensitive discoid plaques, butterfly rash, livedo reticularis
  6. Photosensitivity, vascular changes - nailfold changes and livedo and vasculitis
  7. CREST (Calcification, Raynauds, Esophageal dsymotility, Sclerodactyly, Telangiectasia)
  8. Dermatitis herpatiformis
A
  1. Sarcoidosis, crohns (IBD), TB, behcets, idiopathic and strep are most common
  2. Reactive arthritis
  3. Behcets
  4. Sarcoidosis
  5. SLE
  6. CTD
  7. Limited systemic sclerosis (diffuse is more widespread with nailfold changes)
  8. Coeliac - very itchy!
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15
Q

Paraneoplastic dermatology

  1. Acanthosis nigricans
  2. Dermatomyositis
A
  1. Black velvet
  2. Proximal weakness, heliotrope rash (red panda eyes), Gottrens sign (scales along fingers), muscle biopsy + Anti-Jo + Anti-Mi
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16
Q

Nutritional dermatology

  1. Scurvy
  2. Pellagra
A
  1. Vitamin c deficiency: Corkscrew hairs, perifollicular haemorrhages, bleeding gums!
  2. Vitamin B3 Niacin deficiency: Dermatitis (photosensitive), diarrhoea, dementia
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17
Q

Genetic dermatology

  1. Pseudoxanthoma elasticum
  2. Neurofibromatosis type 1
A
  1. Arterial aneurysms, angioid streaks, elastomas (plucked chicken
  2. Multiple café au lait spots, neurofibromas, axillary freckling (pathognomic), Lisch nodules (iris)
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18
Q

What does Vasculitis feature that is not present in thrombosis? Thus differentiating the vasculitic rash

Pathophysiology of vasculitis

A

PALPABLE and RAISED purpura.
Blisters
Purple/blue edge to ulcers.
Livedo reticularis

Type 3 hypersensitivity reaction causing immune complexes to deposit in endothelium of vessels > immune response attacks self

19
Q

Giant cell arteritis (LARGE VESSEL)
Pathology
Sx
Dx

A

Commonest LARGE vessel vasculitis
F>M >55yrs

Jaw claudication, temporal headache, scalp tenderness, PMR symptoms, visual loss, pale retina/optic disc on fundoscopy

High ESR
USS temporal artery - ‘halo’ sign
Biopsy is GOLD STANDARD
Consider CT-PET scan if no sure where is affected

20
Q

Takayasu arteritis (LARGE VESSEL)
Pathology
Sx
Dx

A

F>M <40yrs
Phase 1: Inflammatory
Phase 2: Absent pulses in arms + Claudication due to stenotic lesions + aneurysms + bruits

Angiography of aorta and branches

Diagnostic = CT-PET scan

21
Q

Polyarteritis Nodosa (MEDIUM VESSEL)
Pathology
Sx
Association

A

Affects branches of aorta so pain when eating - mesenteric angina

Associated with Hep B

22
Q

Kawasaki disease (MEDIUM VESSEL)
Pathology
Sx

A

Children <5yrs

3 Phases:
Affects coronary arteries
Fever >5 days
Strawberry tongue
Conjunctivitis
Groin rash
Thrombocytosis
23
Q 
ANCA associated vasculitis
Microscopic polyangiitis
Churg Strauss (eosinophilic)
Wegener's
Differentiating features
A

Wegeners’ features ELK: ENT (saddle nose deformity + chronic sinusitis), Lung (nodules) and Kidney (cresenteric/sclerosing RPGN) and cANCA and saddle nose deformity

MPA has pANCA and MPO antibodies, but biopsy reveals a lack of granulomatous inflammation formation and the upper respiratory tract is spared

Churg-Strauss features peripheral neuropathy and cardiac issues, eosinophilia and asthma and pANCA

24
Q

Immune complex associated vasculitis
HSP/IgA vasculitis
Anti-GBM
Differentiating features

A

IgA has the triad of abdo pain, arthritis and purpura and usually affects children. Features C3 and IgA deposits

Anti-GBM only really affects kidney and lung whilst obviously Anti-GBM features Anti-GBM antibodies

25
Q

SLE
Sx
Dx criteria
Disease associations

A 
Mouth ulcers on hard palate is pathognomonic
Jaccoud arthropathy (ulnar deviation + MCP subluxation)

Lesser known Sx in criteria: Non-scarring alopecia, pleuritis, haemolytic anaemia, low WCC or platelets, neurology ie psychosis, photosensitivity, rash, arthritis, renal

ANA, Anti-DNA, Anti-SM, low complement, direct coombs test to detect autoantibodies on RBCs (unless in presence of haemolytic anaemia)

OR biopsy proven nephritis + ANA or dsDNA

Pericarditis (Saddle shaped ST elevation)
Renal disease

26
Q

Nailfold capillaroscopy

A

Abnormal in secondary Raynauds (CTD causes etc)

Normal in primary Raynauds

27
Q

Antiphospholipid Dx

A

The diagnostic criteria require one clinical event, i.e. thrombosis or pregnancy complication, and 2 of the following 3 months apart:
Anti-β2 glycoprotein I
Anti-cardiolipin
Lupus anticoagulant

Lupus anticoagulant antibodies paradoxically prolongs APTT despite its clotting effects thus this is just an artefact. Hence its name saying anticoagulant despite being pro-coagulant

28
Q

Systemic sclerosis classification

A

Localised is purely skin.

Systemic is skin and organs. Two types:

  1. Limited: CREST + pulmonary hypertension + hands/face rash. Anti-centromere. spares kidney
  2. Diffuse: Rapidly progressing and rash affects larger area of proximal trunk/skin and more organs. Interstitial lung disease. Anti-RNA polymerase = kidney. Anti-topo = lung
29
Q

Systemic sclerosis complications and Ix

A

Pulmonary fibrosis - HRCT

Pulmonary hypertension - ECHO + R heart catheter

Gastric antral vascular ectasia aka watermelon gut = iron def - Endoscopy

30
Q

Three things which can induce Raynauds

A

Smoking

Beta blockers

cold temps

31
Q

Systemic sclerosis monitoring

A

PFTs: ILD is restrictive, PAH is reduced TLCO

Echo: Estimate PASP (pulmonary artery pressure)

Renal: BP and urine dip - VERY IMPORTANT SCREENING TEST if suspect multisystem disease

32
Q 
Diffuse systemic sclerosis RENAL CRISIS
Antibody
Pathology
Cause
Sx
Mx
A

Renal issues more associated with Anti-RNA

Acute sclerosis of blood vessels in kindey = renal crisis

Steroids often precipitate

Malignant hypertension 
High renin
Azotemia
Microangiopathic hemolytic anaemia
flash pulmonary oedema
Seizures

ACEi may be protective

33
Q

Polymyositis
Sx
Dx

A

Proximal symmetrical muscle weakness in 50-60s

  1. Elevated Creatinine Kinase, AST/ALT, LDh and anti-Jo (check for ILD)
  2. EMG or MRI can show oedema which can then help guide site for muscle biopsy
  3. Definitive diagnosis = muscle biopsy - endomysial infiltrates
34
Q

Dermatomyositis
Pathognomonics
What association?
Muscle biopsy result

A

Gottrens papules - lichenoid rash on fingers extensor surface
+
Dilated nailfold capillaries

Very strong link with malignancy + smoking

Perivascular infiltrate

35
Q

Livedo reticularis is seen in?

A

Antiphospholipid syndrome
SLE
Rheumatoid arthritis
Mixed connective tissue disease

36
Q

Myasthenia gravis diagnosis test

A

Tensilon test

37
Q

Always do what test when talking about multisystem vasculitis type conditions?

A

ALWAYS do urinalysis as prognosis is often determined by renal involvement

38
Q

Eponymous syndromes

  1. Felty’s
  2. Caplan’s
  3. Lofgrens
  4. Bechets
  5. Burkitts
  6. Cowdens
  7. DiGeorge
  8. Fanconi anaemia
  9. HSP
  10. Kallmann
  11. Li-Fraumeni
  12. Marfan
  13. Prader-Willi
  14. Sjogrens
  15. Stevens-Johnson
  16. Von Hippel-Lindau
  17. Wilsons
A
  1. RA + neutropenia + splenomegaly
  2. RA + pneumoconiosis (pulmonary nodules)
  3. Bilateral hilar lymphadenopathy + arthralgia + erythema nodosum (sarcoidosis)
  4. Small vessel vasculitis with orogenital ulcers
  5. Caused by EBV/HIV
  6. PTEN mutation. hamartomas on mucus membranes
  7. Ch22deletion + cleft palate + cardiac issues
  8. Hereditary cause of pancytopenia/AML/bone marrow failure
  9. Purpura + abdo pain + arthritis + IgA deposition
  10. Failure to thrive + anosmia + infertility
  11. Sarcoma, breast, leukaemia and adrenal gland tumours
  12. AD tall CTD
  13. Ch15deletion due to genomic imprinting gone wrong + always hungry
  14. Dryness
  15. Cell death causes epidermis to separate from dermis
  16. Tumour supressor gene dysfunction, may cause pheochromocytoma & renal cell carcinoma
  17. High urinary copper, low ceruloplasmin
39
Q

A 43-year-old female, Ghanaian anthropology lecturer presented to her GP complaining of a facial rash, dry eyes and mouth, oral ulceration, alopecia, raynauds and inflammatory joint pain in the hand and wrist.
On examination, she had a butterfly rash, oral ulcers on the hard palate and ++ proteinuria.
Bloods were performed.
Which combination of blood tests would indicate the most active Systemic Lupus Erythematosus?

A

High titre ANA (1:640), elevated anti-dsDNA, low C3 and C4 complement, lymphopaenia

40
Q

When does titre become significant?

61 year old cleaner. Her right hand has been blue for last 3 months and in last 4 weeks both arms and hands have become swollen and feel warm. Her face is swollen and she feels dizzy and breathless. ANA -ve, ENA negative, Capillaroscopy normal. ?

A

> 1:60ish (speckled pattern = lupus)

SVC obstruction due to thoracic malignancy

41
Q

Difference between reactive and malignant (lymphoma) lymphadenopathy?

A

Reactive is usually just in one area and is mobile

Cancerous lymphadenopathy is firm and diffuse

42
Q

Which LFT can be raised in myositis?

A

ALT

43
Q

Diagnose dermatomyositis, whats next step in management?
Start high dose immunosuppression immediately.
Refer to dermatolology to get a diagnosis for the rash.
Arrange for Occupational Therapy and Physiotherapy to do a home visit and consider fitting a stair lift
Arrange a malignancy screen to include CT chest abdomen pelvis, mammography, tumour markers
Arrange pulmonary function tests & echo

A

Arrange a malignancy screen to include CT chest abdomen pelvis, mammography, tumour markers

DERMATOMYOSITIS = high risk of malignancy

Stage 4 (12 decks)

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