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Year 4 - MSK ANNA > MSK - CTD / autoimmune > Flashcards

MSK - CTD / autoimmune Flashcards

1
Q

What is systemic sclerosis?

A

An autoimmune connective tissue disease

Features:

  • 4 x more common in women
  • Scleroderma of skin (skin fibrosis):
    • Hardening / scarring
    • Red
    • Scaly
  • Internal organ fibrosis:
    • Lungs - many patients have impaired lung function on pulmonary function tests (may not feel SoB) + some can develop pulmonary HTN –> leads to right-sided heart failure
    • Heart - pulmonary HTN –> right-sided heart failure
    • GI tract - reflux oesophagitis, oseophageal strictures, malabsorption and many others.
    • Kidneys - renal involvement = poor prognosis, scleroderma renal crisis (symptoms: malignant HTN, hyperteninaemia, azotemia and microangiopathic haemolytic anaemia)
  • Microvascular abnormalities e.g. Raynaud’s
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2
Q

There are 3 patterns of systemic sclerosis - what are they?

A
  1. Limited cutaneous systemic sclerosis
  2. Diffuse cutaneous systemic sclerosis
  3. Scleroderma (without internal organ involvement)
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3
Q

What are the features following patterns of systemic sclerosis:

  1. Limited cutaneous systemic sclerosis
  2. Diffuse cutaneous systemic sclerosis
  3. Scleroderma (without internal organ involvement)
A
  1. Limited cutaneous systemic sclerosis
    • Scleroderma affects face, hands and feet predominantly
    • Associated with ACA antibodies (anti-centromere antibodies) 70-80%
    • Raynaud’s may be 1st sign
    • Used to be called CREST syndrome
  2. Diffuse cutaneous systemic sclerosis
    • Scleroderma affects trunk and proximal limbs predominately
    • Associated with scl-70 antibodies (anti-topoisomerase I antibody) 40%
    • Most common cause of death is due to resp issues: ILD and pulmonary HTN
    • Poor prognosis
  3. Scleroderma (without internal organ involvement)
    • Tightening + fibrosing of skin
    • Skin lesions can manifest as plaques (patch shape) or linear (resembles a longitudinal line)
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4
Q

What is CREST syndrome?

A

The old name for limited cutaneous systemic sclerosis

CREST = 5 main features:

  • C = calcinosis - soft tissue deposits of calcium
  • R = Raynaud’s phenomenon - stess/cold induce vasoconstriction –> white-blue-red transition in skin colour
  • E = esophageal dysmotility - dysphagia, needing liquid to swallow solids
  • S = sclerodactyly - local thickening + tightening of skin - can occur so much as to cause fingers to curl + lose mobility
  • T = telangiectasia (dilated capillaries) - face, palms, mucous membranes - no. tend to increase over time
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5
Q

Which antibodies might be +ve on serology in systemic sclerosis?

A
  • ANA (antinuclear antibody) > 90%
  • ACA (anti-centromere antibody) - often in limited systemic sclerosis
  • Anti-Scl-70 (anti-topoisomerase I antibody) - often in diffuse systemic sclerosis
  • RhF (rhematoid factor) - +ve in ~ 30% of systemic sclerosis
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6
Q

What monitoring do systemic sclerosis patients require?

A
  • Annual echocardiogram - to monitor for pulmonary HTN / right-sided heart failure
  • Spirometry (lung function tests) to monitor respiratory impact
  • Regular clinic appointments if there is renal involvement
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7
Q

How is systemic sclerosis managed?

A
  • No cure
  • Monitor BP + U+Es (renal function)
  • Pharmacological:
    • Immunosuppressants - with organ involvement or progressive skin disease e.g. IV cyclophosphamide
    • ACE-I or ARBs –> ↓ risk of renal crisis
  • Manage Raynaud’s if present:
    • Keep warm
    • Smoking cessation
    • Nifedipine (Ca2+ channel blocker) 5-20 mg/8h PO - may help
    • Sildenafil (viagra) - may help
    • Rarely - prostagladin infusion over 3-5 days (need to be monitored due to cardiac side effects)
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8
Q

Sclerodactyly is a feature of CREST syndrome (Limited cutaneous systemic sclerosis) but looks very similar to a complication of diabetes mellitus - what complication?

A

Diabetic Chieroarthropathy

Features:

  • Thickened skin + limited joint mobility of hands / fingers
  • Results in flexion contractures
  • Seen in ~ 30% of longstanding diabetic patients
  • Classically: patients can’t put palms of hands/fingers flat against each other ‘prayer sign’ - +ve when gap between palms
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9
Q

What is the difference between primary and secondary Raynaud’s?

A
  • Primary = Raynaud’s disease
  • Secondary = Raynaud’s phenomenon (occurs secondary to underlying pathology)
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10
Q

What is Raynaud’s disease/phenomenon?

A

Raynaud’s = peripheral digital ischaemia due to paroxysmal vasospasm precipitated by cold or emotion

Features:

  • Fingers / toes ache
  • Colour change: pale (ischaemia) –> blue (deoxygenation / cyanosis) –> red (reactive hyperaemia)
  • Raynaud’s disease F:M = 9:1
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11
Q

How is Raynaud’s managed?

A
  • Nifedipine (Ca2+ channel blocker) 1st line: initially 5mg TDS PO –> then adjust according to response to 20mg TDS PO
  • Sildenafil (viagra)
  • IV prostacyclin (epoprostenol) infusions over 3-5 days - needs close monitoring due to cardiac side effects, but can provide relief for weeks/months
  • Keep warm (hand warmers)
  • Smoking cessation
  • Digital sympathectomy for severe disease (cutting of sympathetic nerve or removal of a ganglion)
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12
Q

What conditions can cause Raynayd’s phenomenon?

A

Secondary causes:

  • Connective tissue disorders: systemic sclerosis/scleroderma (most common), SLE
  • Rheumatoid arthritis
  • Leukaemia
  • Type I cryoglobulinaemia - pathological cold sensitive antibodies (cryoglobulins) in blood become insoluable at low temps
  • Cold aglutinins - rare autoimmune condition which causes agglutination of RBCs at low temps
  • Using vibrating tools
  • Drugs: COCP
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13
Q

Systemic sclerosis / scleroderma is the most common cause of Raynaud’s phenomenon. It is a connective tissue disease - what patient factors/symptoms mioght indicate an underlying connective tissue disease?

A
  • Raynaud’s onset after 40 yrs
  • Unilateral symptoms
  • Rashes
  • Presence of autoantibodies (autoimmune condition)
  • Features of RA, SLE e.g. arthritis, recurrent miscarriages
  • Hands: digitial ulcers, calcinosis
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14
Q

What is antiphospholipid syndrome (APLS)?

A

APLS is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies

  • Can be primary or secondary to other conditions e.g. SLE
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15
Q

What are the common features of APLS?

A

Features:

  • Venous + arterial thromboses
  • Recurrent miscarriage, stillbirth, preterm delivery
  • Thrombocytopenia (↓ platelets)
  • Livedo Reticularis - mottled reticulated (net-like) vascular pattern on skin (often legs)
    • Caused by swelling of venules due to obstruction of capillaries by small blood clots
  • Paradoxical rise in aPTT (activated partial thromboplastin time) - measures integrity of intrinsic clotting system + common clotting pathway
    • This is due to an ex-vivo reaction of the lupus anticoagulant (immunoglobulin which is prothrombotic) autoantibodies with phospholipids involved in the coagulation cascasde
  • Pre-eclampsia
  • Pulmonary HTN
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16
Q

What conditions can APLS occur secondary to?

A
  • SLE (main one)
  • Other autoimmune disorders
  • Lymphoproliferative disorders e.g. lymphomas, leukemias
  • Phenothiazines (rare) - used to treated schizophrenia / psychotic disorders
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17
Q

How is ATLS managed for the following evnets/scenarios?

  • Initial venous thromboembolic events
  • Recurrent venous thromboembolic events
  • Arterial thrombosis
A
  • Initial venous thromboembolic events:
    • Warfarin - target INR of 2-3 for 6 months
  • Recurrent venous thromboembolic events:
    • Lifelong warfarin - target INR 2-3 OR 3-4 (if thromboembolic event occured whilst taking warfarin)
  • Arterial thrombosis:
    • Lifelong warfarin - target INR of 2-3

N.B. Heparin is used instead of warfarin pre-pregnancy or during pregnancy as warfarin = cross the placenta and is teratogenic

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18
Q

What are the diagnostic criteria for antiphospholipid syndrome (updated Sapporo APLS classification or Sydney classification)?

A
  1. One of the following clinical findings:
    • Vascular thrombosis:
      • ≥ 1 episodes of arterial, venous or small-vessel thrombosis of any organ, without vessel wall inflammation (often DVT or PE for veins and TIA/Stroke for arteries)
    • Pregnancy morbidity:
      • ≥ 1 foetal deaths of normal fetuses at > 10w gestation
      • ≥ 1 premature births of normal fetuses before 34th week of gestation (prematurity must be due to eclampsia, pre-eclampsia or placental insufficiency)
      • ≥ 3 consecutive spontaneous abortions before 10th week gestation (hormonal, chromosomal or maternal abnormalities must be ruled out)
  2. One of the following lab findings:
    • Detection of lupus anticoagulant in plasma on 2 or more occasions, > 3 months apart
    • Detection of IgG or IgM subtype of anti-cardiolipin antibodies in serum or plasma on 2 or more occasions, > 3 months apart
    • Detection of Anti-beta2 glycoprotein-1 antibody of IgG and/or IgM isotype in serum or plasma on 2 or more occasions, > 3 months apart
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19
Q

What is sarcoidosis and what are its features?

A

It is a multisystem disorder of unkown aetiology - characterised by:

Features:

  • Non-caseating granulomas
    • Caseating = necrosis in which tissue has soft cheese-like appearance
  • More comon in young adults + African descent
  • Acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
  • Insidious: SoB, non-productive cough, malaise, weight-loss
  • Skin: lupus pernio (chronic indurated red/purplish lesion on nose, ears, cheeks, lips, forhead)
  • Hypercalcaemia - macrophages in the granulomas cause increased conversion of Vit D to active form i.e. calcitriol –> ↑ [Ca2+]
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20
Q

Describe lupus pernio?

A

Chronic red/purplish indurated lesion on

skin: nose, cheeks, ears, lips, forhead - seen in sarcoidosis

Lupus pernio = misnomer as it has nothing to do with lupus or pernio

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21
Q

What is Lofgren’s syndrome?

A

It is an acute form of sarcoidosis characterised by:

Features (first 3 = triad –> if present then diagnose):

  • Bilateral hilar lymphadenopathy (BHL)
  • Erythema nodosum
  • Polyarthralgia / arthritis - often acute and involving lower limbs
  • Fever
  • More common in Scandanavian population

N.B. It usually carries an excellent prognosis

22
Q

When a patient presents with a rash as a symptom, what questions should you ask?

A
  • How long have you had the rash?
  • Is it always there?
  • What does it look like?
    • Do you have a photo of it? (if it’s not there now)
  • What areas are affected by the rash?
  • Is it itchy? painful?
  • Does anything make it worse?
    • New medication, chemicals (allergy), sunlight (photosensitivity)
23
Q

What is the most likely explanation for peripheral oedema and heavy proteinuria in combination?

A

Nephrotic Syndrome

Can be caused by:

  • Systemic disorders affecting kidneys e.g. amyloidosis, lupus, diabetes
  • OR
  • Primary renal disorders e.g. membranous nephropathy, focal segmental glomerulosclerosis
24
Q

What is nephrotic syndrome?

A
  1. Heavy Proteinuria ( >3.5g in 24hrs, ++++ Protein Dipstick, urine looks frothy)
  2. Hypoalbuminaemia (Albumin lost in urine due to gaps in podocytes of glomerulus)
  3. Oedema (Albumin lost in urine –> ↓ intravascular oncotic pressure –> fluid moves into into surrounding tissues)
  4. Hyperlipidemia (Liver compensates for hypoalbuminaemia by ↑ production, but side effect is ↑ lipid production)

Also you will see:

  • Lipiduria
  • Loss of antithrombin-III, proteins C and S
25
Q

What are the defining characteristics of nephritic syndrome?

A
  1. Haematuria (+++ Blood dipstick - microscopic or macroscopic/cola-urine)
    • Possible ‘red cell casts’ = microscopic cylindrical structure, present in urine, produced in nephrons in diseased states
    • Haematuria occurs due to gaps in podocytes of glomerulus
  2. Proteinuria (++ Protein dipstick = small amount, usually < 2g/24hrs)
  3. Hypertension (usually mild - caused by salt + water retention)
  4. Low Urine Volume (i.e. oliguria, < 300 ml/day) - due to ↓ renal function

You also see:

  • Oedema - periorbital, leg or sacral
  • Uraemia - pathological features associated with azotemia (↑ blood [urea] )
26
Q

Describe the appearance of malar rash / butterfly rash.

What condition is it often seen in?

A

It is a red/purpleish, mildly scaly, rash across both cheeks and the bridge of the nose (nasolabial folds are spared)

It is associated with SLE

27
Q

What is the epidemiology of SLE?

A
  • W >>> M (9:1)
  • More common in Afro-Caribbeans and Asian communities
  • Onset usually 20-40yrs
28
Q

What is the pathophysiology of SLE?

A
  • Autoimmune disease
  • Associated with HLA B8, DR2, DR3
  • Immune dysregulation thought to cause immune complex deposition which can affect any organ e.g. skin, joints, kidneys and brain
29
Q

What are the features of SLE?

A

General:

  • Fatigue
  • Low-grade fever
  • Mouth ulcers
  • Lymphadenopathy

Skin:

  • Malar (butterfly) rash: spares nasolabial folds
  • Discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas
    • Lesions may progress to become pigmented and hyperkeratotic before becoming atrophic
  • Photosensitivity
  • Raynaud’s phenomenon
  • Livedo reticularis
  • Non-scarring alopecia

MSK:

  • arthralgia
  • non-erosive arthritis

CVD:

  • Pericarditis (most common cardiac manifestation)
  • Myocarditis
  • Venous or arterial thrombosis

Resp:

  • Pleurisy/pleuritis (more common than pericarditis)
  • Fibrosing alveolitis (IPF)

Renal:

  • Proteinuria
  • Glomerulonephritis (most common type = diffuse proliferative glomerulonephritis)

Neuro-psych:

  • Anxiety and depression
  • Psychosis
  • Seizures
30
Q

What investigations might you order in suspected SLE?

A
  • FBC - looking for thrombocytopenia, leukopenia due to lymphopenia, anaemia
  • ESR + CRP - SLE can have ↑ ESR but normal CRP due to immunoglobulins increasing ESR, if ↑ CRP then exclude infection
  • U+Es and BUN - SLE with renal manifestations may have ↑ BUN and creatinine
  • Urinanalysis e.g. MSU - assess renal involvement, haematuria, casts, proteinuria
  • 24h urine collection or protein-creatinine ratio - if urinalysis is abnormal, may show proteinuria
  • CXR - pleural effusion, cardiomegaly, IPF
  • Joint X-ray - in pts with arthalgia - should show inflammation, nonerosive arthritis
  • ECG - exclude other causes of chest pain
  • Immunology:
    • 99% are ANA positive - ANA not specific to SLE as can be +ve in dRA, systemic sclerosis, Sjogren’s etc.
    • anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
    • anti-Smith: most specific (> 99%), sensitivity (30%)
    • 20% are rheumatoid factor positive
    • also: anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
  • Anti-phospholipid antibodies - in anyone with history of VTE, miscarriages or prolonged aPTT

N.B. there are LOADS more tests as SLE affects many systems

31
Q

Is a +ve ANA always diagnostic of connective tissue disease?

A

NO!!

  • A +ve ANA is seen in a number of rheumatological and autoimmune conditions and also healthy people
  • ~ 5% of the population will have a positive ANA test
32
Q

For the following conditions which anti-nuclear antibodies (ANA) are associated with them?

  • SLE
  • Sjogren’s
  • Dermatomyositis (type of chronic muscle inflammation)
  • SCLE (subacute cutaneous lupus erythematosus)
  • Diffuse cutaneous systemic scleroderma
  • CREST (limited cutaneous systemic sclerosis)
  • MCTD (mixed conntective tissue disease)
A
  • SLE = dsRNA, anti-Smith, anti-nRNP
  • Sjogren’s = Ro (SSA), La (SSB)
  • Dermatomyositis = anti-Jo-1
  • SCLE = Ro (SSA), La (SSB)
  • Diffuse cutaneous systemic scleroderma = anti-Scl-70
  • CREST = anti-centromere
  • MCTD = anti-nRNP
33
Q

What important considerations are there in a patient with SLE who wishes to become pregnant?

A
  • Pregnancy planning is required as patient’s SLE needs to be stable
  • If on immunosuppressants –> ensure compatible with pregnancy
  • Check for presence of Ro (SSA), La (SSB) and antiphospholipids antibodies
    • Ro (SSA) and La (SSB) +ve = risk of neonatal lupus as they can cross placenta –> lupus rash, complete heart block and blood abnormalities e.g. cytopaenias (↓ in mature blood cells e.g. thrombocytopenia)
34
Q

What renal complication can SLE cause?

How is this complication monitored?

A

Lupus nephritis

Monitor: urinalysis at regular check-up appointments to rule out proteinuria

35
Q

What are the classes in the WHO classification of lupus nephritis?

A
  • class I: normal kidney
  • class II: mesangial glomerulonephritis
  • class III: focal (and segmental) proliferative glomerulonephritis
  • class IV: diffuse proliferative glomerulonephritis
    • Most common + severe form
  • class V: diffuse membranous glomerulonephritis
  • class VI: sclerosing glomerulonephritis
36
Q

How is SLE managed?

A
  • Lifestyle: smoking cessation, sun protection, exercise
  • Treat hypertension
  • Corticosteroids - if clinical evidence of disease
    • GI + bone protection if use long term e.g. PPI + bisphosphonates
  • Hydroxychloroquine (DMARD) - joint symptoms, lupus nephritis, flares etc.
  • Immunosuppressants:
    • Cyclophosphamide
    • Azathiopine
    • Tacrolimus
37
Q

What criteria are included for the diagnosis of SLE?

N.B. some common symptoms are not included and the criteria weren’t designed for diagnostic purposes but are used this way.

A

1997 ACR Classification of SLE:

4 or more = diagnose SLE

  1. Malar rash
    • Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
  2. Discoid rash
    • Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions
  3. Photosensitivity
    • Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
  4. Oral ulcers
    • Oral or nasopharyngeal ulceration, usually painless, observed by physician
  5. Nonerosive Arthritis
    • Involving ≥ 2 peripheral joints, characterized by tenderness, swelling, or effusion
  6. Pleuritis or Pericarditis
    • Pleuritis – convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion
      • OR
    • Pericarditis – documented by electrocardigram or rub or evidence of pericardial effusion
  7. Renal Disorder
    • Persistent proteinuria > 0.5 g/day or > +++ on urine dip
      • OR
    • Cellular casts – may be red cell, hemoglobin, granular, tubular, or mixed
  8. Neurologic Disorder:
    • Seizures – in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance
      • OR
    • Psychosis – in the absence of offending drugs or known metabolic derangements, e.g., uremia, ketoacidosis, or electrolyte imbalance
  9. Hematologic Disorder:
    • Hemolytic anemia – with reticulocytosis
      • OR
    • Leukopenia – < 4,000/mm3 on ≥ 2 occasions
      • OR
    • Lyphopenia – < 1,500/ mm3 on ≥ 2 occasions
      • OR
    • Thrombocytopenia – < 100,000/ mm3
  10. Immunologic Disorder:
    • Anti-DNA
      • OR
    • Anti-Smith
      • OR
    • Positive finding of antiphospholipid antibodies
  11. Positive Antinuclear Antibody (ANA)
    • ​​Very sensitive but not specific
38
Q

What is the single most useful test to monitor for renal involvement and relapses after treatment in a 40 year old man diagnoses with SLE?

  • Blood pressure
  • dsDNA
  • ESR
  • Urinalysis
  • U+Es
A

Urinalysis

  • dsDNA and ESR are helpful as a markers of disease and going up with disease respectively, but aren’t the best options
  • U+Es - This is helpful but renal involvement may not always result in an abnormality of the U&Es
39
Q

Which of the following patients have anti-phospholipid syndrome?

  1. A 25 year old lady who presents with an unprovoked deep vein thrombosis
  2. A 33 year old man who tests positive for Lupus anticoagulant and anti-cardiolipin antibodies twice at a 2 month interval
  3. A 33 year old man who tests positive for Lupus anticoagulant and anti-cardiolipin antibodies twice at a 3 month interval, the first test is done after a pulmonary embolus
  4. A 37 year old lady who suffers three miscarriages
  5. A 54 year old lady with Factor V Leiden thrombophilia who has a pulmonary embolus
A

Answer (3) - Interval should be a minimum of 3 months. He meets the clinical and lab criteria

  1. She could have but the diagnosis is based on clinical and lab criteria
  2. Interval should be a minimum of 3 months, also laboratory criteria only
  3. Interval should be a minimum of 3 months. He meets the clinical and lab criteria
  4. Clinical criteria only
  5. This is the most common inherited thrombophilia but is nothing to do with anti-phospholipid syndrome
40
Q

What is Sjogren’s syndrome?

A

Sjogren’s is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces

  • Primary or secondary to RA and other connective tissue disorders e.g. SLE or systemic sclerosis (usually develops ~10yrs after initial onset of RA/CTD)
  • F >>>> M (9:1)
  • 20’s - 30’s (most common ages)
41
Q

Sjogren’s syndrome is associated with an increased risk of what?

A

Lymphoid malignancy (Lymphoma) 40-60 fold

42
Q

What are the features of Sjogren’s syndrome?

A
  • Dry eyes (keratoconjunctivitis sicca)
    • Sensation of sand/gravel, itch or burning, multiple episodes weekly for > 3 months
  • Dry mouth
    • ​Can cause poor dentition e.g. dental caries, as saliva is protective - means dentists often notice Sjogren’s 1st
  • Vaginal dryness
  • Arthralgia
  • Fatigue, myalgia
  • Raynaud’s
  • Sensory polyneuropathy - usually purely sensory loss but pts commonly have neuropathic pain
  • Recurrent episodes of parotitis
  • Renal tubular acidosis (usually subclinical)
43
Q

What investigations might you order for suspected Sjogren’s syndrome?

A
  • Rheumatoid factor (RF) +ve (~ 100% of pts)
  • ANA +ve in 70%
  • anti-Ro (SSA) antibodies in 70% of patients with PSS (primary)
  • anti-La (SSB) antibodies in 30% of patients with PSS (primary)
  • Schirmer’s test - filter paper (I-DEW strips) near conjunctival sac to measure tear formation ( 15ml in 5 mins is normal )
  • Histology - focal lymphocytic infiltration
  • Also:
    • Hypergammaglobulinaemia
    • low C4 (complement component 4)
44
Q

How is Sjogren’s syndrome managed?

A
  • Artificial tears and saliva
  • Humidifiers - reduce loss of sections due to evaporation
  • Pilocarpine 5mg TDS PO - muscarinic cholinergic receptor agonist may help stimulate saliva production
  • Sugar free sweet to encourage salivation
  • Massage face in morning to encourage mucous gland activity
45
Q

What condition can longstanding Sjogren’s cause?

A

AA amyloidosis

  • This is a type of amyloidosis, characterised by abnormal deposition of fibres of insoluable protein in the extracellular space of various tissues / organs
  • Protein = serum amyloid A protein
  • AA amyloidosis is a complication of several inflammatory diseases / infections e.g. RA, ankylosing spondylitis, Crohns, UC etc.
  • Most common presentation = renal:
    • Proteinuria
    • Nephrotic syndrome
    • Progressive CKD –> ESKD –> dialysis / transplant
46
Q

What blood test is commonly used to test for TB?

A

QuantiFERON-TB Gold (QFT)

  • Used to detect Mycobacterium tuberculosis - bacteria which causes TB
  • It is unaffected by previous BCG vaccination
  • It can’t distinguish between ACTIVE and LATENT TB infection
  • Once a pt has had TB, they will always test +ve on QuantiFERON test
47
Q

What retinal side effect can long-term hydroxychloroquine have?

A

Chloroquine retinopathy - also called Bull’s eye maculopathy

  • It is a form of toxic retinopathy
  • Earliest signs include bilateral paracentral visual field changes
  • Associated with gradual decreasing visual acuity
  • Can cause widespread funal atrophy and visual loss
48
Q

What factors might suggest underlying connective tissue disease in a patient with Raynaud’s?

A
  • Onset > 40 years
  • Unilateral symptoms
  • Rashes
  • Presence of autoantibodies
  • Features suggestive of RA or SLE e.g. arthritis or recurrent miscarriages
  • Digital ulcers or calcinosis
49
Q

What is the typical presentation for Raynaud’s disease?

A

Young (e.g. 30 yrs) female with bilateral fingers going pale white in cold, then blue then red.

50
Q

What are common features of Raynaud’s phenomenon (secondary)?

A
  • Digit pallor (must be present)
  • Blue and/or red discolouration of digits (one must be present)
  • Dilated, torturous capillary nail beds - looks like small red pen marks (mainly in secondary)
51
Q

What are the risk factors for Raynaud’s phenomenon (secondary)?

A
  • Female
  • FHx of Raynaud’s
  • Presence of connective tissue disease:
    • RP occurs in >90% with systemic sclerosis, >80% with mixed connective tissue disease, 40-45% with SLE, 20% RA and 13-17% with Sjogren’s
  • Vibration injury
  • Smoking
  • Beurger’s disease - inflammation + thrombosis of small + medium sized BVs, often in the legs, causing gangrene
  • Colder climate
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Year 4 - MSK ANNA (13 decks)

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