Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

CSIM > Multisystem > Flashcards

Multisystem Flashcards

1
Q

Natural Hx of SLE

A

Chronic inflammatory mulitsystem AI disease which releases and remits. Natural progression of the disease and its treatment leads to comorbidites.

Increased rate of atherosclerosis, malignancy and infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Epidemiology of SLE

A

10:1 women:men, 20/100000
Increased incidence in afrocarribeans 16-40 y/o
HLADR2 and 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Aetiology of SLE

A

Homozygous deficiencies of C1q, C2 or C4 confer a high risk but are very rare
24% risk in monozygotic twins
Seen in premenopausal women more

Viruses and UV can both trigger flares

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pathogenesis of SLE

A

Cell death is triggered via viral illness, UV light, smoking and occurs via apoptosis leads to DNA and histone presentation on the cell surface. These are interpreted as foreign antigens and phagocytosed . In SLE this process is inefficient leading to APC presenting the cell fragments to T cells which intern activate B cells to produce antinuclear antibodies. These form circulating complexes or deposit in tissues leading to complement activation and influx of neutrophils. This perpetuates the response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

PC SLE - Bones and non specific

A

Most patients suffer non specific fatigue, fever, symmetrical small join arthralgia with pain but structurally normal joints +/- some swelling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Discoid lupus

A

Confined to the skin, rash appears on the face as a well defined erythematous plaque that progresses to a plaque and pigmentation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

SLE and the skin

A

Photosensitive rash with a butterfly distribution over the bridge of the nose.
Vasculitic lesions on the finger tips, purpura and urticaria
Livedo reticularis
Scarring alopecia can = irreversible bald patches
Raynauds - 20%
Oral and mucosal ulcers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Jaccouds arthritis

A

Deforming non erosive arthropathy characterised by ulnar deviation of the second to 5th fingers with MCP subluxation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

SLICC Critera for SLE

A

Need > 4 criteria (1 clinical and 1 lab)
or
biopsy proven lupus nephritis with +ve ANA or antidsDNA stain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Lung and Heart SLE

A

Pleurisy and recurrent exudative pleural effusions. These can lead to restrictive lung disease

Pericarditis and pericardial effusions (25%). Increased risk of IHD and strokes due to HTN, hypercholestrolemia and chronic inflammation

Very rarely there may be myocarditis - arrhythmias, aortic valve lesions and a non infective endocarditis (Libman-Sachs syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Renal and SLE

A

Risk of lupus nephritis a RPGN which can lead to end stage renal failure. Screening with urine dipstick for proteinuria and haematuria is crucial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Nervous system and SLE

A

60% of cases may be mild depression but occasionally more severe psychotic features

Epilepsy, seizures, migraine, ataxia, aseptic meningitis etc

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Raynaud’s disease

A

Peripheral digital schema often precipitated by cold or emotion.

PC = digit pain can be very severe
white to blue to red
In severe cases can = digital infarction and self amputation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Aetiology of raynauds

A

1 - Raynauds phenomenen

2 - SLE, RA, Sjogrens, systemic sclerosis, polymyositis
Obstructive causes - Buegers, takayasu’s atherosclerosis
B-blockers, occupational drugs involving vibration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Mx Raynauds

A

Look for a diagnosis! Gloves stop outdoor work, CCB’s - DHP i.e. nifedipine or ACEi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

O/E SLE

A

Hands - raynauds, thickening of the flexor tendons
Lymphadenopathy
Diffuse scarring alopecia, discoid or photosensitive rash
Oral or mucosal ulcers
BP and urine dip to check for lupus nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Investigations SLE

A

High ESR,
Leucopenia, lymphopenia +/- thrombocytopenia
Normocytic anaemia of chronic disease or AIHA
Low C3 and C4 complement in acute disease

Biopsy = deposition of IgG and complement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Autoantibodies in SLE

A

95% ANA +ve but poor sensitivity seen in old age and increased in many other AI conditions

anti-dsDNA high specificity - homologous pattern of staining

Anti smith - increase prevalence in black african populations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

SLE and Sjogrens

A

Anti Ro70 and Ro52 showing a speckled ANA pattern and Anti Ui RNP showing nuclear ANA pattern are linked to an increased risk of Sjogrens and high risk of Ab crossing the placenta and causing congenital heart block and rash in foetus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Drug induced SLE

A

Sulphasalazine, procaimide, antieplieptics all anti-histone +ve

Anti-TNF = anti dsDNA +ve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Mx of SLE

A

Immunosuppression - hyrdroxychoroquine and NSAIDs

IV steroids for acute flares (prednisolone and cyclophosphamide)

Monitor anti ds-DNA and C3/C4 levels - sensitive for relapse

Check urine for evidence of lupus nephritis

Reduce risk of IHD and osteoporosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Lofgrens syndrome

A

Subtype of sarcoidosis. Arthralgia, bilateral hilarlympadenopathy and erythema nodosum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Antiphospholipid syndrome PC

A

Thrombosis
Recurrent miscarriage
Livedo reticularis
Thrombocytopenia, high APT, +ve Coombs test

20% of pt suffer ischemic strokes, 40% DVTs. 27% of women who have 2+ spontaneous miscarriage = anti phospholipid syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

SLE and preganacys

A

Hydroxycholoquine and azathioprine, low dose steriods are safe. Beware those with anti-Ro and La. 2% risk of fatal heart block

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Aetiology of antiphospholipid

A 
70% = idiopathic
30% = SLE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Autoantibodies in APS

A

Anti-cardiolipin - Detects antibodies that bind to the negatively charged phospholipid cardiolipin

Lupus anticoagulant - Caused by anti phospholipid antibodies causes increased APTT (opposite effect to within the body)

Anti B2 glycoprotein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Mx APS

A

Aspirin

If recurrent thrombosis = anticoagulation with warfarin INR 3.5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Vasculitis

A

Inflammation of the blood vessels often characterised by multi-system involvement, fatigue, wt loss. Classified by size of vessels effected and the presence of ANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Large cell vasculitis

A

GCA and takayasu’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Giant cell arteritis

A

Inflammatory granulomatous arteritis of the large cerebral arteries. Seen above the age of 50, 2:1 female:male, highest incidence in northern europe.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

PC GCA

A

Often debilitating bitemporal headache - not improved by analgesia, lasts the whole day

Ocular symptoms

Scalp tenderness, neck ache, jaw claudication

Systemically - wt loss, fever, limb claudication due to subclavian stenosis, abdo pain SMA stenosis, HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Ocular symptoms in GCA

A

Amaurosis fungax - temporary blindness , reduced visual acuity, RAPD, reduced colour vision and visual field defects

O/E - diffuse pale optic disc swelling, cotton wool spots - nerve ischemia

60% due to arctentic anterior ischemic optic neuropathy this occludes the posterior ciliary vessels leading to infarction of the optic nerve head

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Complications of GCA

A

Increased risk of thoracic aneurysms
Stenosis of cerebral and subclavian arteries
Ischemic consequences of coronary and mesenteric arteries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Investigations into GCA

A

ESR >50
Normocytic anaemia, thrombocytopenia
Deranged LFTs - high ALP

USS doppler - Black halo sign shows vessel ischemia, , loss of radio-echo signal = infiltrate and oedema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Biopsy in GCA

A

From affected side. Inflammatory infiltrate from T cell, B lymphocytes and giant cell. Can be -ve biopsy due to skip lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Central retinal artery occlusion

A

Rapid visual loss, reducing acuity and RAPD.

O/E = cherry red spot @ fovea due to alternate blood supply

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Diagnostic criteria of GCA

A

Age >50, new headache, temporal artery abnormality, ESR >60, +ve biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Mx GCA no visual symptoms

A

60mg prednisolone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Mx GCA + visual symptoms

A

3 days methyprednisolone 0.5-1g stat

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Steroids in GCA

A

Reduced the dose by 5mg every 2weeks, as the dose gets lower the dosing interval should shrink to 1mg

SE = wt gain, infection, cataracts, skin fragility and purpura, mood changes, proximal weakness, Cushings, osteoporosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Polymyalgia rheumatica PC

A

Sudden onset severe pain and stiffness in the muscles of the shoulders and the neck. Often symmetrical crucially with no muscle weakness. Worse in morning lasting from 30 mins to several hours.

1/3rd of its have wt loss, fever, lethargy and depression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Invx in to PMR

A

high ESR, ALP
normal CK
possibly a normocytic anaemia

43
Q

Diagnostic criteria of PMR

A 
Morning stiffness > 45mins = 2
Rh or anti-CCP -ve = 2
Pelvic girdle pain = 1
No other painful joints = 1
USS changes @ shoulders = 1
44
Q

Takayau’s arteritis PC

A

Isolated aortitis with granulomatous nodules. Young japanese females

Pre vasculitis stage of fever, fatigue, wt loss

PC - vascular bruits @ carotids and abdo vessels
renal artery stenosis = HTN
diminished or absent pulses in arms due to complete stenosis
upper limb claudication with BP difference >20mmHg
symptoms of visual loss and dizziness if involving the carotids
coronary artery involvement = HF and aortic root dilation

45
Q

Mx Takayasu’s

A

CT PET to show areas of inflammation

Immunsupression with methotrexate

46
Q

Medium vessel vasculitis

A

Polyarteritis nodosa

47
Q

Polyarteritis nodosa (PAN)

A

Seen in middle aged men, no ANCA association; 30% of cases are linked to hep B

48
Q

PC PAN

A

Constitutional symptoms of fever, malaise, wt loss and myalgia

Acute features
Neuro - mononeuritis multiplex due to arteritis of the vasa nervorum
Skin - subcutaneous haemorrghe and gangrene. In chronic cases persistent livedo reticularis
Cardiac - can = MI, pericarditis and HF

Abdo - most common 1/3rd can mimic pancreatitis, appendicitis. GI haemorrhage due to mucosal ulceration

49
Q

Invx and Mx PAN

A

Anaemia, leucocytosis and a raised ESR

Mx = corticosteriods

50
Q

Kawasaki’s PC

A

Acute systemic vasculitis.
fever lasting >5 days, bilateral conjunctival congestion, dryness and redness of the lips and oral cavity
cervical lymphadenopathy
Polymorphic rash
Redness and oedema of the palms and soles 2-5 days after onset

Mx = single stat dose of immunoglobulin 2g/kg

51
Q

Small vessel vasculitis

A

ANCA associated = GPA, eGPA, MPA
Immune complex associated (ANCA -ve)
= SLE, anti-GBM, HSP, cryoglobulinemia

52
Q

Pulmonary renal syndromes

A

SLE, Goodpastures, GPA, MPA

53
Q

Vasculitic signs and symptoms

A

Non specific - wt loss, fever, night sweats

Skin - purpuric rash - painful and non-blanching
digital infarction - terminal capillary
livedo reticularis
oral and nasal ulcers and polyps

ENT - Epistaxis, crusting, sinusitis, hoarse voice, can get a saddle shaped nose deformity due to loss of nasal cartilage

Eyes - Episcleritis - red eye ball, pain and photophobia, tis is also seen in RA, IBD and systemic vasculitis. Can lead to scleromalacia where the pigment of the underlying choroid can be seen

MSK - arthralgia, myalgia and muscle wasting
Abdo - Iron deficiency anaemia from GI blood loss, abdo pain, diarrhoea and intestinal obstruction

Cardio and resp - Pain, SOB, haemoptysis - pul haemorrhage

Neuro - transverse myelitis, peripheral neuropathy, mononeuritis multiplex

54
Q

Mono-neuritis multiplex

A

Simultaneous involvement of individual nerve trunks evolving over days to years. Acute/subacute sensory and motor loss of individual neurones

Causes = DM, vasculitis, SLE, sarcoid

55
Q

Granulomatosis with polyangitis (GPA) PC

A

Upper airway disease - recurrent sinusitis and epistaxis, can lead to destruction of the nasal cartilage = saddle shaped nose deformity. Bronchial/tracheal stenosis

Pulmonary parenchymal disease - granuloma formation leads to cough, SOB and pleuritic chest pain - can lead to pulmonary haemorrhage
- CXR multiple nodules, consolidation and ground glass

Renal - RPGN
Eyes - scleritis and conjunctivitis

56
Q

Invx GPA

A

PR3 +ve in 90%, biopsy - granulomatosis inflammation in vessel walls,

57
Q

Microscopic polyangitis (MPA) PC

A

Never URTI involvement. Usually presents with RPGN and skin involvement - maculopapular purpura. Has a higher incidence of neurological involvement.=

Low incidence of pulmonary parenchymal disease but high risk of pulmonary haemorrhage.

58
Q

Inv MPA

A

Mixed picture MPO (p-anca) +ve in 90%, can have +ve c-anca PR3 in 20% of cases

Crucially on biopsy = no granulomas

59
Q

Eosinophilic granulomatosis with polyangitis PC

A

Early adulthood onset allergic rhinitis and asthma like symptoms. Features of a systemic vasculitis often appear later.
Skin - tender subcutaneous nodules, petichae and purpura
Neuro (80%) - mononeuritis multiplex

Very rare = alveolar haemorrhage.

60
Q

EGPA Inv

A

MPO, panca +ve

Biopsy = eosinophils, no granulomas

61
Q

Mx of all small vessel vasculitis

A

Induced remission with high dose of steroids

Maintenance with methotrexate and azithprione

62
Q

Henoch-Scholein purpura PC

A

Most common childhood vasculitis 3-8y/o post URTI

Triad of colicky abdo pain, palpable purpuric rash over buttocks and thighs. Haematuria and proteinuria leading to nephrotic syndrome

+/- arthralgia, GI haemorrhage or intusseception

63
Q

HSP Inv and Mx

A

High IgA, CRP, high platelets - not ITP

May need steroids, most return to baseline with no renal impairment. Much higher incidence of ESRF in adults

64
Q

Cryoglobulinemia

A

Simple -  Monoclonal IgM due to myeloma / CLL / Waldenstroms

PC = Hyperviscosity Visual disturbance Bleeding from mucus mems  Thrombosis Headache, seizures

Mixed (80%)
 Polyclonal IgM secondary to SLE, Sjog, HCV, Mycoplasma 

PC = Immune complex disease GN Palpable purpura Arthralgia Peripheral neuropathy

65
Q

SLE auto antibodies

A

Anti-dsDNA = specific for SLE and TNFa induced SLE
Anti histone = drug induced - isoanzid, methotrexate, hydralazine, procainmide

Both show homogenous patterns of staining

Anti-smith = speckled staining

66
Q

Sjogrens auto antibodies

A

Anti Ro and anti La speckled pattern

67
Q

Systemic sclerosis

A

Diffuse - Scl70 antitopisomerase

Limited - anti-centromere

68
Q

PC Raynauds

A

Classically white - blue - red. Worse at times of stress and cold. In severe can lead to digital ulceration and gangrene

69
Q

Inv Raynauds

A

Rule out a secondary cause. Nail fold cappilaroscopy

70
Q

Causes of Raynauds

A

1 - idiopathic

2 - B blockers, systemic sclerosis, SLE, RA, Sjorgens, Vasculitis, polycythemia

71
Q

Pathogenesis of systemic sclerosis

A

Widespread vascular damage involving small arteries, arterioles and capillaries. There is initial endothelial cell damage this perpetuates with cytokine involvement leading to fibroblast activation. Increased levels of type I and III collagen in lower layer of dermis and internal organs

72
Q

Limited systemic sclerosis PC

A

Calcinosis - fingers
Raynauds - 100% often begins 15yrs before skin changes
Eosophegal dysmotility
Sclerodactyl - shortened fat fingers + microstomia
Telengectasia (facial blanching on pressure)

Crucially skin involvement is limited to the hands, face, feet and forearms. No chest or neck involvement

15% cases = pul HTN

73
Q

Investigations limited systemic sclerosis

A

Anti-centromere Abx
Dilated nail fold capillary loops

Barium swallow shows duck neck.

74
Q

Diffuse systemic sclerosis PC

A

Usually begins with oedema and Raynauds and progresses rapidly. More severe involving multiple organs systems. Skin involvement above the elbows on the neck and trunk

Lung = 40% develop ILD - restrictive lung disease with basal reticular nodular shadowing on X-ray
20 % also develop pulmonary HTN

Cardiac = rare involvement - conduction defects

GI = Very common to have oesophageal dysmotility - dysphagia, reflux, heartburn, occasionally anal incontinence. Atony of small bowel, GAVE, malabsorption due to bacterial overgrowth.

Renal - acute hypertensive crisis

75
Q

Inv for diffuse systemic sclerosis

A

Antitopisomerase - scl70
RNA polymerase III - increased risk of renal involvement

Enlarged capillary loops with significant loss

76
Q

Systemic sclerosis renal crisis

A

PC malignant HTN, high renin, microangiopathic haemolytic anaemia. Protein and haematuria

Affects 10% with dSS. Can be precipitated by steriods

Mx = aggressive ACEi. Prognosis linked to level of skin involvement, RNA III polymerase levels and tendon friction rub

77
Q

MAHA (microangiopathic haemolytic anaemia)

A

red cells are ripped apart by physical trauma. Often in the small vessels. SLE and SS are also includes. Crucially schisocytes on blood film

  1. Disseminated intravascular coagulation (DIC) – a nasty condition in which there is bleeding and clotting at the same time in the patient. Lots of things can cause DIC (like malignancy, obstetric complications, trauma, and sepsis) – and it’s complicated enough that we’ll get into it in a future post.
  2. Thrombotic thrombocytopenic purpura (TTP) – a syndrome in which the patient gets little thrombi within the microvasculature anywhere in the body, but especially the CNS and kidneys. We’ve talked a little about TTP before.
  3. Hemolytic-uremic syndrome (HUS) – a disorder often related to ingestion of food (especially raw hamburger, but also spinach, other vegetables, you name it) containing E. coli 0157:H7. The bug makes a toxin that damages endothelial cells, and for some reason, the kidneys are hit the hardest.
78
Q

GI complications of SS

A

GORD - Barretts oesophegus (squamous - glandular)
Duodenum - dilation can lead to atony

GAVE - gastric central vascular ectasia 25% of pt with high RNA polyermase III. Dilated submucosal vessels on antrum may precipitate GI bleed.

79
Q

Investigations in systemic sclerosis

A

FBC - normocytic anaemia
Urine dip - proteinuria, A:CR
Serial PFT’s, echo and BNP to monitor
Barium swallow may show beak shaped deformity

ILD usually manifests with reticulonodular shadowing @ the bases. However can develop apical if due to recurrent aspiration. HRCT to diagnose

80
Q

Mx of SS

A

Avoid the cold, wear gloves
Rarely any management options for oesophageal problems - PPI if symptomatic
Malabsorption - Abx for bacterial overgrowth, nutritional substitutes

Monitor for renal involvement - ACEi stat!

Pulmonary fibrosis = aggressive steroid therapy IV cyclophosphamide and azathioprine

81
Q

Localised scleroderma

A

Morphea - localised subcutaneous confined to the skin in young adults and children.Seen in bluish patches on the trunk can progress to central white atrophy.

82
Q

Sjogrens syndrome PC

A

SICCA symptoms = dryness of eyes, mouth, vaginal, larynx. These can = nutritional defects, tooth decay, dyspareunia, salivary gland enlargement and chronic eye infections - dry gritty eyes

Fatigue

83
Q

Systemic signs of Sjogrens

A

Arthralgia - non progressive polyarthritis
Raynauds
TIN leading to renal tubular acidosis

20x increase of MALT - non hodgkin B cell lymphoma

Rare - oesophageal dysmotility, pulmonary fibrosis

84
Q

Primary Sjogrens

A

9:1 Female:males
Onset 40-50y/o

Absence of RA, SLE,

85
Q

Secondary Sjogrens

A

Linked to HLA DR3 - AIH, PBC, RA, SLE, SS, MCTD, thyroid disease

86
Q

Investigations into Sjogrens

A

100 % for ANA and RF
- Specific = antiRo and La

High ESR, low C3, hypergabbaglobulinemia

Labial and parotid biopsy shows focal infiltration of B cells and lymphocytes.

Crucial to palpate for lymphadenopathy

87
Q

Schirmer test

A

Tests for tear production strip of filter paper placed on inside of lower eyelid for 5 minutes

>15mm = normal 
<5mm = Sjogrens
88
Q

Mx Sjogrens

A

Artifical tears and saliva replacements
NSAIDS and hydroxchloroquine for arthralgia

Immunosuppres for systemic disease

Check for lymphoma!!!

89
Q

Myalgia vs myositis

A

Myalgia - common feature of many AI, no weakness normal muscle enzymes

Myositis - Inflammatory myopathies characterised by proximal > distal weakness often symmetrical in pt 50+

90
Q

Polymyositis

A

Idiopathic inflammation of striated muscle, 3x more common in female

91
Q

PC polymyositis

A

Progressive symmetrical proximal weakness often affecting the shoulders and the pelvic girdle. Spares distal and ocular muscles.

Systemic features of wt loss, fever, anorexia

Can lead to dysphonia, dysphagia and involvement of laryngeal muscles

92
Q

Invx of polymyositis

A

Biopsy - cytotoxic CD8 T cells in endomysium.
High CK and LDH (1000s in active disease)

Crucial to take trop T for baseline
Anti-Jo

93
Q

Livedo reticularis

A

Anti phospholipid, RA, TB, SLE, RCC, dermatomyositis

Polyarteritis nodosa, cryoglobulinemia, myeloma

94
Q

Bechets

A

Oral and genital ulceration, anterior and posterior uveitis, arthralgia

95
Q

Dermatomyositis

A

Polymyositis + skin involvement

  • Heliotrope rash on eyelids +/- oedema
  • Gottrans papules lichenoid changes over knuckles
  • Macular rash (Shawls sign) photosensitive over back and shoulders
  • Mechanics hands painful rough cracked skin without vesicles

Always hunt for Cancer approx 40% linked to.

96
Q

Inv dermatomyositis

A

High CK, LDH, ALT
Raised ESR and CRP

Anti Mi 2

EMG = triad of spontaneous fibrillation @ rest, polyphasic potentials on voluntary contraction, repetitive potentials on mechanical stimulation

Biopsy - CD4 infiltrate, lowest possible steroid dose, screen for malignancy

97
Q

Cancer linked to dermatomyositis

A

Ovary, lung, Non-hodgkin lymphoma, pancreatic and stomach

98
Q

Complication of inflammatory myopathies

A

High incidence of ILD and oesophageal dysmotility

99
Q

Anti Jo +ve (anti trna synthase)

A

Mechanics hands are highly suggestive, increased risk of ILD and raynauds

100
Q

Anti Mi

A

Better prognosis. low incidence of ILD, more cutaneous signs

101
Q

MCTD

A

PC = ILD, Raynauds, skin and muscle involvement, fatigue and arthritis

Anti U1 RNP

102
Q

Antiphospholipid syndrome diagnosis

A

1 x clinical = vascular thrombosis (FHx),
pregnancy >3 unexplained miscarriages >1 late abortion

1 x lab = Lupus anticoagulant (2 x 12 weeks apart)
anticardiolipin / anti B2 glycoprotein

103
Q

DMARDs in pregnancy

A

Safe = azathioprine and hydroxychlorquine

Stop known teratogens i.e. MMF, methotrexate and cyclophosphamide. Rituxamib linked to 2nd/3rd trimester B cell depletion.

104
Q

Pre-pregnancy counselling

A

Achieve disease quiscense 6 months prior to conception.
Measure C3, dsDNA, BP and urine @ baseline

Maternal Ro/La = 2% risk of fetal HB at birth, Low dose aspirin for all with SLE,

Manage flares with lowest dose possible methylpred. Pregnancy makes rheumatic conditions much worse! High dose folic acid. Consultant led care

CSIM (12 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions