MSD 2.85-92 Flashcards
(215 cards)
1
Q
What type of AI disease are CTD?
A
Systemic! Because involve nuclear Ag found ubiquitously
2
Q
How does CTD start?
A
as uCTD, then usually becomes one of: SLE, DM/PM, Sjogrnes, SSc, MCTD.
3
Q
Symptoms of SLE?
A
- Lungs: tachypnoea, cough, pleural inflammation and effusion, PE
- Skin: photosensitive rash, butterfly rash over cheeks (sparing nasolabial folds), livedo, discoid, alopecia. SICCA, mucosal ulcers.
- Systemic inc. lymphadenopathy, anaemia, thrombocytopenia, leukopenia
- CVD: Raynaud’s, pericarditis, vasculitis, thrombosis (particularly if have APS)
- Renal: lupus nephritis, proteinuria, haematuria
- CNS: neuro disorders, migrainous headaches, seizures, aseptic meningitis
- MSK (arthralgia, arthritis, myositis)
- Reproductive: menorrhagia/amenorrhoea, prematurity, stillbith, recurrent miscarriage. Lupus more likely to flare during pregnancy.
4
Q
Risk factors for SLE?
A
F, child-bearing age, FHx, genes (HLA-DR2/3), Afro Caribbean>Asian>Caucasian, drug-induced
5
Q
Key pathophysiology?
A
Defective clearance of apoptotic cells exposes nuclear antigens to IS; following DNA damage, get autoantibodies to nuclear proteins, forming immune complexes and recruiting complement to damage tissue, releasing more Ag. Autoantibodies opsonise DS DNA.
6
Q
Natural history of SLE?
A
Preclinical; autoantibodies form. Clinical get inflammation and involvement of first organs. Then get flares, more organs involved, damage. Eventually to disease and treatment get irreversible organ damage, malignancy, atherosclerosis, diabetes, osteoporosis. Incurable.
7
Q
Joints affected in SLE?
A
Typically small joints of hands and feet. Non-erosive
8
Q
LE cells?
A
(Lupus erythematous cell) are PMNs or macrophages that have ingested another cells nuclear material; classically seen in SLE.
9
Q
VDRL test in SLE?
A
Get false positive
10
Q
ANA patterns?
A
Homogenous, nucleolar, speckled and centromere-B; associated with different ANAs and therefore different CTD.
11
Q
Use of ANA?
A
Autoantibodies; sensitive but not specific for SLE; umbrella term which can be differentiated into ENAs. Presence detected with ELISA. Increased frequency with age, so titre is important as not all ANA = AI. Get +ve ANA then do ENA panel.
12
Q
dsDNA? And its signifiance?
A
Fairly specific for SLE; low levels in RA/MCTD/AIH. Important in SLE because associated with risk of ESRD in SLE.
13
Q
Homogenous ANA?
A
Associated with dsDNA staining and therefore SLE
14
Q
Sm (smith)?
A
Very specific for SLE but no very sensitive
15
Q
Ro/La? And significance of Ro?
A
Associated with Sjogrens and SLE (particularly with skin disease); get speckled ANA. Ro can cross placenta and cause congenital heart block
16
Q
Anti-RNP?
A
Associated with SLE and MCTD. Nucleolar ANA.
17
Q
Antiphospholipid antibodies?
A
Anti-cardiolipin, lupus anticoagulant (DRVVT), anti-B2 glycoprotein. Prothrombotic. Need two +ves 3 months apart. Can be used to support SLE diagnosis too.
18
Q
APLS?
A
Livedo rash, CNS disease, obstetric problems, worse prognosis in LN.
19
Q
Antibodies for drug-induced SLE?
A
Anti-histone antibodies!
20
Q
Drugs causing drug-induced SLE?
A
Minocyclie, procainamide, sulfasalazine, AEDs, anti-TNFa (infliximab) for RA, Crohns, eye disease.
21
Q
Why are cell counts low in SLE?
A
Ab mediated; get haemolysis etc. (direct Coombes test) Platelets low due to this and APLS Abs.
22
Q
Differentiating SLE flare and infection?
A
Neutrophils normal in SLE! And discordant rise in ESR
23
Q
ESR and CRP in SLE?
A
CRP often slightly raised; ESR often very high.
24
Q
SLICC criteria SLE?
A
- Biopsy proven lupus nephritis and ANA/dsDNA
- Or 4 criteria, at least 1 immunological. Clinical = acute/chronic cutaneous LE, oral ulcer, alopecia, synovitis, serositis, renal, neurlogi, haemolytic anaemia, lecopenia/lymphopenia, thrombocytopenia.
Immune = ANA, dsDNA, Anti Sm, APLS Abs, Low complement, direct Coombs.
25
Why do you need low threshold for infection in SLE?
Drugs may mask immune response
26
Four types of epidermal rashes?
Eczematous, psoriasiform, lichenoid, vesiculobullous. Lichenoid seen in SLE. Barrier disrupted so either wet or dry
27
Eczematous rash?
Gaps in epidermis; spongiosis. Fluid comes up from dermis, get weeping, blisters and dry flakes. Variable blisters suggests eczema.
28
Psoriasiform rash?
Skin proliferates and thickens; get scale
29
Lichenoid rash?
Epidermal. More serious/AI link. May get in SLE with discoid rash. Get purple, interface dermatitis.
30
Vesiculobullous rash?
Destruction of proteins holding dermis and epidermis; epidermis lifts off. Can be due to external (burns) or internal (pemphigoid, AI).
31
Dermal rashes?
Get raised lesion but skin smooth. Granulomatous, vasculopathic, tissue deposition.
32
Granulomatous rash?
Ring-like; histiocytes involved. Purple-brown. Seen in Tb and sarcoid (lupus pernio), and granuloma annulare (reaction to collagen).
33
Vasculopathic rash?
Death/blockage or leakage of blood vessels (e.g. leakage could be urticaria. Linked to SLE/CT ie IC can block vessels; red cells leak into dermis. Palpable purpura.
34
What can infiltrate the skin?
Extracellular substances, or inflammatory cells (eosinophils, neutrophils, lymphocytes)
35
Anti-Scl70?
Diffuse systemic sclerosis. (aka anti-topoisomerase)
36
RhF?
RA
37
Anti-Ro/La?
Sjogrens, SLE.
38
HLA-B27?
Ank spond and other seronegative spondlyoarthropathies
39
Anti-Jo1?
Polymyositis
40
MPO/P-ANCA?
EGPA and MPA (AND PSC/UC)!!!
41
PR3/C-ANCA?
GPA
42
Jaccoud's arthropathy?
Deformity of hands post SLE arthritis
43
Contraception in SLE?
Progesterone only or barrier; COC pro-thrombotic.
44
Monitoring lupus activity?
DSDNA, ESR, C3 and C4, BP, urinalysis, basic bloods
45
Differentials for LRT problems in SLE patients?
Pneumonia (IC), PE (hypercoagulable), pleuritis, pulmonary oedema secondary to renal failure
46
Lupus nephritis?
Can cause ESRD.
47
Chronic pain syndromes?
Multiple hyperalgesic sites, often associated with other physical and psych symptoms, no identifiable organic cause. Fatigue prominent, poor sleep. Cognitive problems! Plethora of somatic symptoms
48
Conditions associated with fibromyalgia?
TMJ, Sjogrens, anxiety and depression, headaches and migraines, panic disoders, IBS
49
Risk factors for fibromyalgia?
Female, 30-50, previous physical trauma, previous viral infection, psychological stress
50
FIBRO?
Fatigue, insomnia, blues, rigidity, ow (widespread pain)
51
Common features in fibromyalgia?
Often waited years for diagnosis, may feel that have been passed around, frustration at negative investigation, underlying mental health and social issues, limited evidence based treatments. Good sleep hygeine key!
52
Yellow flags in FM?
Inappropriate treatment expections, sickness behaviour, belief that pain and activity are always harmful, withdrawal, emotional problems, problems related to time off work
53
Two causes of vasculitis?
Bacteria i.e. mycotic, or AI.
54
How can LVV give organ-specific disease?
If origin of visceral vessels is affected (i.e. where SMA joins aorta)
55
Most common vasculitis in UK?
HSP including paeds; GCA in adults
56
GCA basics?
>50, usually 70s. F:M 2:1. Sight loss in 20%. Northern European highest risk.
57
Takayasus basics?
Rare in UK, commoner in Japan. Younger women; 5-40, 9:1.
58
Cranial GCA symptoms?
Headache; severe, debilitating, there all day, analgesia not helpful. Scalp tenderness, jaw claudication, neck ache (not with movement) [vertebral artery involvement], PMR (limb girdle pain, no weakness, worse in morning), VISUAL SYMPTOMS. May have isolated occipital headache.
59
Systemic GCA symptoms?
Constituional, weight loss, fever, limb claudication (SCA), abdominal pain (SMA/IMA), HTN. More likely to be male with existing PVD.
60
Neuro exam in PMR?
Should be normal
61
Cranial or systemic GCA more problematic?
Systemic more likely to be insidious and then present with visual involvement, paradoxically.
62
Signs of GCA?
Thickened temporal artery, visual involvement
63
Managing GCA?
Any visual involvement = refer to opthalmology. Otherwise, urgent medical review and start oral steroids.
64
Optic presentations of GCA?
Amaurosis fugax, vision loss. Usually due to occlusion of posterior ciliary arteries (arteritic anterior ischaemic optic neuropathy). May see reduced VA, RAPD, reduced colour vision and visual field defects.
65
Cranial nerve palsies in GCA?
Compromised supply to extraocular muscles or CNs; get opthalmoplegia, + anisocoria and ptosis if 3rd nerve involved. Can just be transient diplopia!
66
Opthalmic complications of systemic steroids?
Raised IOP (significant if have/at risk for glaucoma, cataracts, worsening of infection if have existing keratitis, worsening of diabetic eye disease
67
GCA investigations?
ESR >50, CRP >20. Normocytic anaemia, raised platelets, neutrophilia, elevated ALP, U&E usually normal tho renal impairment possible. TA USS and biopsy.
68
Further useful IX in GCA?
CXR (occult malig), electrophoresis (myeloma)
69
TA USS and biopsy?
USS alone can be enough to diagnose if have history and inflammatory markers. However, steroids make signs disappear very quickly. Can be useful to find biopsy site!
70
GCA pathology?
Activated DCs present Ag to CD4 T cells; Th1/17 recruit macrophages which coalesce to form multinucleate giant cells (hallmark); granulomatous thickening. Get oedema, SM proliferation, occlusion. Elastic lamina disrupted, meaning damage is permanent. Giant cells not always seen!
71
Difficulties with steroid related diabetes?
Tend to get unusual peak in afternoon sugars; overtreating can lead to hypoglycaemia in the morning
72
GCA with no headache?
Incidental diagnosis when investigating anaemia, weight loss, raised inflammatory markers, arm pain/change in colour/claudication, PUO, thoracic aneurysm, stroke.
73
Investigations for non-cranial GCA symptoms?
e.g. arm claudication, aortic aneurysm. Do PET; patchy inflammation in atherosclerosis is consistent here.
74
Stages of Takayasu's arteritis?
1. Systemic (prevasculitic)
2. Vascular (inflammatory, get stenosis and aneurysms)
3. Burnout (ischaemia, pulseless phase).
Aim to pick up in one or two.
75
Takayasu's is?
A granulomatous pan-arteritis with two/three clinical phase. LVV. Pan means whole of arterial wall affected
76
Presentation of Takayasu?
Arthralgia, myalgia, chest wall pain. Fever, weight loss, dizzines &/or visual symptoms, SOB. Get upper limb claudication with BP R-L >20mmHg. HTN, CAROTIDYNIA, aortic root dilatation. May have coronary involvement. May just be unexplained acute phase response, PUO.
77
Investigating Takayasu?
CTA, MRA, Doppler USS, CT PET. See cuff of inflammatory tissue around aorta, and see stenosis explainign dizziness/visual/CN problems
78
Difference between vasculitic and embolic rash?
Vasculitic is palpable (painful) purpura!
79
Causes of purpura?
1. Blood: platelet or clotting problem (leakage)
| 2. Vessel wall: thombus/vasculitis/meningoccaemia (death) or steroids (leakage)
80
Infarction of whole finger?
Likely to be mid vessel.
81
HSP?
Small vessel vasculitis. Triad of petechial rash on buttocks and ankle, abdominal pain and renal involvement (IgA nephropathy). Often follows URTI/sore throat. Usually self limiting; bad prognosis if have severe abdo pain/bloody diarrhoea, renal involvement, persistent rash >1/12. May get chronic renal disease. M>F.
82
Mechanism of vessel death in vasculitis?
T3 HS; get IC lodging in small vessels, causing purpura as inflammatory cells extravasate. Also may get blistering as epidermis dies and lifts off, then dermis dies and get ulcers. Purple spots, then blisters, then black/necrotic.
83
Larger vasculitis lesions?
More worrying than multiple petechiae because suggests larger vessel involvement so could wipe out entire renal/coronary artery.
84
Mechanism of livedo?
Normal in cold; get constriction of ascending arterioles between superficial and deep plexi, with blood trapped by the skin. White areas are well-perfused (ish), purple rings are at dangerous watershed areas; a map of quite large vessels so worrying. Patchy livedo more worrying; uniform and bilateral physiological. Can lead to ulceration. Get pink-blue mottling. More prominent on legs and in cold.
85
APLS Abs cause?
CLOT: Coagulation defect, livedo, obstretic complications, thrombocytopenia.
86
Causes of livedo?
Vasculitis/CTD: SLE, PAN, ANCA, RA, dermatomyositis, PAN, infection, malignancy, drugs (amantadine), hypercoagulable states e.g. APLS, polycythaemia.
87
Three ways vessels can be damaged?
Atherosclerosis, fibromuscular dysplasia, vasculitis (l'cytes and neutrophils in surrounding tissue).
88
Characteristic histology of SVV/MVV?
Inflammation and fibrinoid necrosis of the blood vessel wall with impairment of flow and sometimes thrombosis. Inflammatory cells escape and cause inflammation.
89
Presentation of vasculitis?
Can be insidious or with organ failure.
90
Skin signs of vasculitis?
Livedo, purpura, digital infarction/nail fold involvement in SVV, digital ulceration and gangrene in MVV, nodules.
91
Mucous membrane signs of vasculitis?
Oral ulcers, nasal ulcers/crusting/polyps, genital ulcers. Large, persistent or hard palate ulcers particularly suspicious.
92
Eye signs of vasculitis?
Episcleritis (fairly benign), scleritis and scleromalacia (thinning of sclera allows pigmented choroid to be seen); serious as can perforate.
93
MSK signs of vasculitis?
(Mild) arthralgia and arthritis, myalgia, wasting muscle and weakness (if myositis).
94
ENT signs of vasculitis?
Epistaxis, crusting, sinusitis, hoarse voice, nasal collapse [late], stridor from laryngeal disease (if airway collapses). Flattened flow-volume loop (fixed UAO) but same TLC; if have unilateral bronchial obstruction get reduced.
95
Cardio-resp vasculitis symptoms?
Pain, SOB, haemoptysis, crackles, asthma/wheeze. Pain if near pleura. Haemoptysis suggests parenchymal involvement.
96
Neurological signs of vasculitis?
Peripheral neuropathy (classic glove and stocking), mononeuritis multiplex (can pick and choose certain nerves), transverse myelitis.
97
Abdominal signs in vasculitis?
Abdo pain, diarrhoea, blood loss/anaemia, weight loss, liver damage, intestinal (obstruction), peritonitis.
98
Primary vs secondary vasculitis?
Primary = ANCA, HSP, PAN, Behcet's. Secondary = cryoglobulinaemic vasculitis.
99
Vasculitis by size order?
Biggest = GCA and Takayasu's, then PAN and Kawasaki, then GPA/EGPA/MPA, then HSP and essential cryoglobulinaemic vasculitis, then cutaneous leukocytoclastic vasculitis.
100
Appearance of C-ANCA and P-ANCA?
C-ANCA is cytoplasm uniformly stained (in PMN); p-ANCA is most around nucleus. C-ANCA usually to PR3 but not always; the same with P-ANCA and MPO. i.e. can be ANCA+ve without MPO/PR3 staining. Measure ANCA with immunofluorescence; now just do PR3/MPO tests.
101
HLA-DP and DQ?
DP assoc with PR3; DQ with MPO
102
ANCA levels and disease activity?
Unlike RhF/CCP, ANCA is directly pathogenic, so retesting it is useful clinically
103
Aetiology of ANCA?
Varies between populations, and GPA has cyclical pattern so may be environmental trigger.
104
Features of GPA?
Prominent constitutional symptoms (rash, w/l, arthralgia etc.) and renal (GN), ocular (scleritis, retroorbital mass), chest (granuloma, bronchiolar and subglottic stenosis, ILD [UPPER AND LOWER AW]), ENT (sinusitis/crusting/bloody rhinorrhoea, bony erosion).
105
How can GPA present?
1. Localised/granulomatous. Not immediately organ threatening; get ENT/sinusitis, episcleritis, skin, URT. More often female or younger. However, can rapidly get invasive locally (disfiguring) or...
2. Systemic/vasculitic. Two sides of a coin. Immediately organ threatening with GN, alveolar haemorrhage, scleritis, mononeuritis multiplex, systemic symptoms. Needs more aggressive treatment!
106
Features of MPA?
Milder constitutional symptoms, more renal involvement. GN key. Skin get mac-pap purpura; mononueritis multiplex, pulmonary haemorrhage/infiltrates/effusions, GI (bleeding, pain, ischaemia, ulceration)
107
Difference in MPA and GPA lung involvement?
MPA is smaller, more diffuse so get haemorrhage, cough, SOB; GPA larger so get stridor, subglottal stenosis.
108
MPA basics?
Only 2/3 ANCA+ve; MPO more than PR3 (p more than c-ANCA). Raised acute phase response, with RAPIDLY DETERIORATING RENAL FUNCTION (red cell casts/dysmorphic RBC).
109
EGPA features?
Late onset "asthma" symptoms typical; get ENT (sinusitis/polyposis), mononeuritis, cutaneous (biopsy shows eosinophils and granulomas hence name), cardiac (25%) (get myocarditis; CCF rather than angina), GI get abdo pain/bleeding (poor prognosis). May need MRI to monitor
110
Special about EGPA biopsy?
Presence of eosinophils means biopsy is more meaningful than usual
111
PAN features?
MVV. Not usually ANCA+ve. Idiopathic or Hep B related. CNS & PNS, cutaneous, renal. Prominent GI and orchitis.
112
Causes of secondary vasculitis?
1. CTD (SLE, RA, Sjogrens)
2. Drugs (hydralazine, propylthiouracil, allopurinol, sulfasalazine, penecillamine)
3. Malignancy (haematological and para-neoplastic).
4. Infection (direct damage to vessel wall; strep, staph, syphilis, Rickettsia).
5. Infection (IC formation) HIV, Hep B/C.
113
Only primary vasculitis with a clear cause?
PAN and Hep B
114
Secondary vasculitis with Hep C?
Cryoglobulinaemia. Get IC formation; immunoglobulins precipitate below 37 degrees and dissolve. More prominent in cold peripheries.
115
Ix for SVV?
CXR, PFT (AW disease), urine dip, HRCT, CT sinus/orbits, MRI muscles, NCS/EMG, bloods inc. CRP/ESR, ANCA, ENA, dsDNA, GBM, blood cultures, infection screen (looking for secondary vasculitis)
116
Biopsy for SVV?
Useful if presentation isn't helpful. ENT not very useful, pulmonary can be done to exclude malignancy, renal good in MPA, muscle/skin/nerve.
117
Significance of -ve pulmonary biopsy in SVV?
Doesn't rule out SVV as not very sensitive
118
LCV?
Leukocytoclastic vasculitis; includes HSP.
119
SSA and SSB?
Other names for Ro/La (Sjogrens)
120
Which two ANA patterns are seen in SLE?
Homogenous and speckled
121
Risk factors for Raynaud's?
F, smoking, B-blockers.
122
Diffuse systemic sclerosis?
Scl-70, Rna polymerase III (predicts renal involvement). Get Raynaud's, digital ulceration, diffuse skin thickening, ILD, GI problems, renal, cardiac. Skin changes go BEYOND ELBOWS AND KNEES. Linked with PAH and ILD! Caused by excessive collagen deposition
123
Limited systemic sclerosis?
Anti-centromere Ab. CREST. Calcinosis of finger pulps. Raynaud's is severe and can get autoamputation. Telangectasia of face and hands. Get scleroderma of neck and face (small mouth, can't fully open). Does not extend beyond elbows and knees!
124
Concerns with ulcers in SScl?
Risk of osteomyelitis; need xrays.
125
Investigating systemic sclerosis?
ANA/ENA. Urine dip (renal crisis), BP. Nail fold capillaroscopy (useful diagnostically (as changes not seen in primary Raynauds. Get loss of capillary loops, then dilation and reduction). Do CXR and CT at diagnosis; then serial PFTs. If FVC drops, or TLCO drops, re-image for ILD. CXR may show reticul-nodular basal shadowing. Apical scarring may be recurrent aspiration. Annual echo for PAH, and pro-BNP. OGD and barium swallow for GI pathology. Barium will show dilated oesophagus and slow transit time.
126
GAVE/watermelon stomach?
Seen on OGD for SScl; prone to bleeding
127
Managing vascular problems in SScl?
CCBs, sildenafil, then iloprost etc. if refractory.
128
Renal crisis in SScl?
A feature of diffuse disease. More likely if RNA polymerase III +ve. Get rapidly deteriorating renal function, flash pulmonary oedema, seizures. Steroids may precipitate it. ACEI are protective!
129
SScl demographics?
Rare, F:M 10:1, 30-50.
130
Scleroderma?
Localised pathology seen in systemic sclerosis; different to limited. Get morphoea, linea morphoea and encoupe de sabre. Focal collagen deposition in dermis hence scleroderma.
131
SICCA symptoms?
Dryness or sensation of dryness/grittiness in any mucosal surface
132
Sjogrens?
MSD; chronic inflammatory CTD disorder characterised by lymphocytic infiltrations in exocrine glands. Can be primary or secondary to other AI CTD. Symptoms are SICCA, joint involvement, fatigue. May get Raynaud's, myalgia, resp/GI disease, renal tubular acidosis. May get parotid hypertophy.
133
Sjogrens demographics?
F:M 9:1, 40-50.
134
Cx of Sjogrens?
MALT lymphoma; 20* increased risk. 4.3% incidence.
135
Investigating Sjogrens?
ANA, ENA (Ro/La). Rf may be mildly elevated. Serial ESR/comp/Ig/electrophoresis for complications. Schirmer's tear test (<5mm), salivary flow, parotid and submandibular USS (diagnostically useful), exclude malignancy etc. Lip biopsy can support diagnosis and gives risk of progression to lymphoma.
136
Myalgia vs myositis?
Myalgia is common feature of AI disease; no weakness and enzymes (CK, LDH) normal. e.g. RA, PMR, hypothyroid. Inflammatory myositis (poly, dermato, overlap) get weakness, raised enzymes but MAY NOT BE PAINFUL. NB: can be hard to assess weakness in pain.
137
Why test for HCV in Sjogrens?
Apart from serology of each, clinical picture can be nearly identical
138
Diagnosing Sjogrens?
Ocular symptoms, oral symptoms, ocular signs (Schirmer's), oral signs (salivary flow), +ve biopsy and compatible serology
139
General features of inflammatory myopathies?
Muscle weakness, proximal > distal, problems rising from chair etc. Involvement of resp and pharyngeal muscles can be life-threatening (ask about hoarseness/dysphonia/problems breathing). Peak 50-60. DD includes inherited muscle disease.
140
Polymyositis?
Idiopathic, proximal, symmetrical. Rarely have myalgia! Distal power may be normal, often spares ocular muscle. May be systemically unwell. Few CTD sx.
Bloods show raised CK, LDH, AST, troponin. ANA +ve, Anti-Jo. ESR and CRP may be elevated. KEY RISK OF ILD ESPECIALLY IF HAVE JO
141
Why troponin done in polymyositis?
Often raised; means if have ACS symptoms can have baseline.
142
Diagnosing polymyositis?
Symmetrical proximal muscle weakness, elevated muscle enzymes, EMG findings, +ve biopsy. Anti-Jo1 not needed for diagnosis.
143
Anti-Jo syndrome?
Associated with synthetase syndrome; dermato/polymyositis, ILD, mechanics hands, Raynauds. I.e. found in these but will likely have ILD too.
144
Investigating polymyositis?
Enzymes, serology, ESR, MRI/EMG for findings and tell you where to biopsy. Only biopsy can be definite diagnosis. MRI just shows non-specific muscle oedema EMG painful and not routinely done.
145
Monitoring polymyositis?
Serial PFTs (thoracic involvement)
146
Features of dermatomyositis?
Gottron's papules, heliotrope rash, periorbital oedema, shawl sign (photosensitive). Anti-Mi-2, biopsy and MALIGNANCY SCREEN. Otherwise the same as polymyositis. Anti-Jo may be positive. Can affect lungs, joints, oesophagus. Do skin biopsy.
147
Advantages of IV Ig in myopathies?
Can be safely used in infection
148
MCTD?
Mix of RA, SLE, myositis, scleroderma. Anti-RNP (U1RNP). Get Raynauld's fatigue, ulceration, skin, arthritis, ILD and PAH involvement, puffy hands, muscle involvement. EROSIVE ARTHRITIS, PAH!
149
Anti-RNP?
Umbrella term. Associated with MCTD. Anti-smith in SLE, Ro/La in Sjogrens, U1RNP in MCTD.
150
APLS?
Acquired AI disease. Recurrent A or V thrombosis +/- fetal loss. Can affect any system. Skin = livedo, digital infarctions, purpura. CNS = CVA, chorea, seizures, VST, headache. Peripheral veins = DVT/PE.
151
APLS Ab?
Lupus anticoagulant, IgG/M anti beta-2-glycoprotein, anticardiolipin, on +2 occasions, +3 months apart!. Can be used in SLE diagnosis.
152
Diagnosing APLS?
At least one clinical and one lab criteria.
1. Thrombosis (imaging shown), or obstetric (1+ late term spont. abortion, 1+ premature birth of normal neonate at/before 34 weeks due to PET etc, 3+ unexplained, consecutive spontaneous early miscarriages (<10 weeks).
153
Primary and secondary APLS?
1. Primary
| 2. Secondary to SLE etc.
154
Maternal Ro/La?
High risk of foetal heart block so get extra echos if +ve.
155
Strongest predictor of adverse pregnancy outcomes?
Lupus anticoagulant! Test for it in early pregnancy
156
Managing flares in pregnancy?
Lowest possible dose of steroid
157
DMARDs in pregnancy?
Methotrexate and mycophenolate bad. Cyclophosphamide quite bad. Hydroxychloroquine good, as is azathioprine. Rituximab not teratogenic, but chance of neonatal B cell depletion in 2nd/2rd. Steroids used pulsed, at minimum dose.
158
Neonatal lupus?
Transmission occurs in 1-2%.
159
Secondary Raynauld's?
Hypercoagulable states e.g. polycythaemia, systemic sclerosis, SLE.
160
Features of PMR?
Proximal muscle pain/stiffness, over 50, ESR>40, rapid response to prednisolone. Weakness should not be a feature, CK always normal
161
CK in PMR?
Always normal!
162
Causes of raised ESR?
Malig, pregnancy, PMR, SLE, haematological, Tb, hepatitis, bacterial infection, GCA, RA, SSc.
163
Key use of nailfold capillaroscopy?
Differentiating between primary and secondary RP; should be normal in primary. Particularly relevant to the secondary causes of systemic sclerosis and DM/PM.
164
Causes of muscle weakness and raised CK?
DM/PM, muscular dystrophy, rhabdomyolysis, MND, iatrogenic e.g. statin-induced myositis
165
Where else can dermatomyositis affect?
Joints, oesophagus, lungs, rarely heart.
166
Monitoring lung involvement in PM/DM?
Serial FVCs (get restrictive pattern). Life-threatening. Can be ILD or can be muscle weakness.
167
Steroids and muscles?
Treat myositis, can cause myopathy (chronically).
168
Receptor hit in MG?
nACh
169
DM and ILD?
Possible; linked with Jo-1.
170
Follow up of DM?
CT C/A/P, OGD/colonoscopy, bronchoscopy. HRCT to monitor ILD.
171
DM and malig?
25% have or will develop an associated malignancy;
172
Nailfold changes seen in CTD?
Dilated capillary loops, drop-out of capillaries, nailfold infarcts/telangectasia, ragged cuticles, cuticular hypertrophy.
173
Neurofibromatosis T1 cutaneous features?
Neurofibromas, multiple cafe-au-lait macules, axillary freckles, Lisch nodules in iris
174
Key cutaneous feature of Cushing's?
Acne!
175
Skin changes in adrenal insufficiency?
If primary, get hyperpigmented buccal mucosa and palmar creases (high ACTH); if secondary than have pallor.
176
Hypothyroid skin changes?
Loss of outer 1/3 of eyebrow, periorbital oedema, dry skin.
177
Graves skin changes?
Thyroid acropachy, pretibial myxoedema, exopthalmos
178
Tendon xanthomas seen in?
FHC
179
Eruptive xanthoma seen in?
Hypertriglyceridaemia
180
Diabetes skin changes?
Acanthosis nigricans in insulin resistance in obese, T2DM; hyperpigmented neck/axilla/skin folds. Rarely seen in malignancy.
Necrobiosis lipoidica; granulomatous inflammation of the skin. Means bad glycaemic control!
Infections e.g. candida
181
Gout skin?
Periarticular tophi (urate)
182
Pyoderma gangrenosum?
Neutrophils in skin; haem malignancy and IBD, RA. Mistaken for nec fasc; steroids will help this and make NF worse. Looks infectious
183
Sweet's syndrome?
Haem. malig; PMNs in skin i.e. neutrophilic dermatoses. Bright purple plaques, high fever. Myeloma, leukaemia etc. Give steroids.
184
Cutaneous T cell lymphoma?
Rash looks like psoraisis; treatment resistant. Formerly called mycosis fungoides. Patch and plaque stage (5-30 years so long prodrome) then tumour stage; poor prognosis.
185
Umbilicated blisters?
= viral infection.
186
Cutaneous features of syphilis?
1. Primary chancre. Painless, on genitals.
2. Rash on palms and soles.
3. Tertiary gives gummae etc.
187
Lyme disease?
Get erythema migrans; may get second ring inside ('bullseye'). Borrelia. New forest.
188
Purpura fulminans?
Necrotic, pre-terminal stage of meningococcaemia. Large areas of dying skin.
189
Erythema nodosum associated with?
Sarcoid, IBD, post-streptococcal, Tb.
190
IBD skin?
PG, EN.
191
Behcets features?
Oral and genital ulcers, uveitis, +/- arthritis. Pyoderma. Get pathergy (eruptions at skin prick sites). thrombophlebitis etc. AI, inflammatory.
192
Sarcoid skin?
"Naked" granulomas, lupus pernio.
193
RA skin?
Rheumatoid nodules (must be RhF +ve), nail fold infarcts, vasculitic leg ulcers.
194
Lupus skin?
Malar rash, photosensitivity, discoid lesions (DLE), livedo.
195
Microstomia?
Seen in SSc
196
Scurvy?
Vit C def; needed for collagen synthesis. Get purpura (as with steroids, blood vessels left vulnerable). Corkscrew hairs.
197
Pellagra?
Niacin/B3 def. Get 3 'd's: photosensitive dermatitis, diarrhoea, dementia
198
Paraneoplastic skin?
Myriad, e.g. dermatomyositis, acanthosis nigricans.
199
Titre levels?
Higher = 1:640 rather than 1:40
200
Lymphopenia in SLE?
= more active
201
Lofgren's syndrome?
BHL + EN + arthralgia. Acute form of sarcoidosis. Treat with NSAIDs etc (conservative)
202
Is EN tender?
Yes!
203
Key Ix in lupus flare?
Urine dip; lupus nephritis can be very dangerous and very insidious
204
Which ANCA has most prominent sinus/UA symptoms?
GPA
205
Signs of SVC obstruction?
Diffusely warm hands, dizzy, facial plethora, (oedema of face and arms), prominent neck and chest veins, cough, headache, difficulty breathing, Pemberton's sign
206
Sjogren's patient with night sweats and lymphadenopathy?
(MALT) lymphoma; nodes are likely to be firm and fixed (soft in reactive).
207
Erythematous annular rash could be?
Borrelia (Lyme disease)
208
When to do malignant screen in DM?
If have pulmonary/airway involvement, need to immunosuppress aggressively. If not, then look for malignancy urgently.
209
Skin changes in limited SScl?
Do not go beyond elbows!
210
Skin changes in PAN?
MVV, so will be digital infarcts and large lesions rather than petechiae.
211
Arthritis in reactive?
Oligoarticular, asymmetrical.
212
HSP abdo cx in children?
Can get oedema, obstruction or intussuception
213
Investigating RP?
Want to find out if primary or likely to progress. ANA, ENA, symptom screen, nailfold capillaroscopy!
214
Treating paraneoplastic dermatomyositis?
Removing cancer usually helps it
215
When are NSAIDs okay in pregnancy?
Up to the third TM (associated with closure of ductus arteriosus in utero), as is rituximab (risk of B cell depletion)
