Rheumatology and Multisystem Disease Flashcards
1
Q
Give some examples of organ specific autoimmune connective tissue disorders
A
Hashimotos thyroiditis T1DM Anti-GBM disease Bullous phegmoid Myasthenia gravis Pernicious anaemia
2
Q
Give some examples or systemic autoimmune connective tissue disorders
A
SLE
Systemic sclerosis
Sjorgens syndrome
Polymyositis
Dermatomyositis
Mixed CTD
ANCA associated vasculitis
IBD
Psoriasis
3
Q
What investigations should be undertaken in a patient with an aCTD?
A
BEDSIDE: urinalysis, BP, temp, sats
BLOODS: FBC, CRP, ESR, specific autoantibodies
4
Q
Anti-La/Ro?
A
Sjorgens syndrome
5
Q
Anti-cardiolipin?
A
Antiphospholipid syndrome
6
Q
Anti-Jo1?
A
Polymyositis/dermatomyositis
7
Q
pANCA for MPO?
A
- Microscopic polyangitis
- Eosinophilic polyangitis with granulomas
- Also associated with ulcerative colitis
8
Q
cANCA for RP3?
A
Granulomatous polyangitis
9
Q
dsDNA?
A
SLE
10
Q
Anti-RNP?
A
Mixed CTD - usually associated with clinical manifestation of Raynaud’s, myositis and sclerodactyly
11
Q
Anti-Scl70?
A
Diffuse systemic sclerosis
12
Q
Anticentromere?
A
Limited systemic sclerosis
13
Q
Anti-Sm?
A
SLE
14
Q
What are the systemic inflammatory vasculitides?
A
A group of disorders characterised by widespread vasculitis leading to systemic symptoms requiring treatment with STEROIDS and IMMUNOSUPPRESSANTS.
These can be classified according to:
- Size of blood vessels involved
- Presence of ANCA (anti-neutrophil cytoplasmic antibodies)
15
Q
What are the large vessel vasculitides?
A
Refers to aorta and major tributaries:
- Giant cell arteritis/PMR (large cerebral arteries)
- Takayasus arteritis (aorta)
16
Q
What are the symptoms of GCA?
A
- Thickened temporal artery
- Severe headaches
- Scalp tenderness
- Jaw claudication when eating
- Sudden unilateral blindness (due to involvement of ophthalmic artery)
- Systemic (malaise, tiredness, fever, abdo pain, hon)
- Limb pain/PMR
17
Q
What do investigations show in GCA and how is it diagnosed?
A
INVESTIGATIONS:
- Raised ESR >50
- May have low albumin
- Normocytic anaemia
- TA USS shows black halo sign
- Fundoscopy
DIAGNOSIS:
- Temporal artery biopsy shows CD4+ T cells and giant cells, disrupted elastic lamina, granulomatous inflammation, skip lesions
18
Q
How is GCA managed?
A
- Oral prednisolone (higher doses need in GCA than PMR) for 12-18 months. START IMMEDIATELY IF SUSPECTED GCA
- Oral tocilizumab/methotrexate if long-term steroids contraindicated
If no rapid improvement, consider alternate diagnosis. Give gastric and osteoporosis protection.
19
Q
What are the complications of GCA?
A
- Irreversible blindness (AION)
- Aortic aneurysm
- Large vessel stenosis
20
Q
Describe the atypical presentation of GCA (no headache)
A
PUO, weight loss, arm pain, thoracic aneurysm, posterior stroke
21
Q
What is the most common GCA eye complication and how is it managed?
A
AAION (anterior arteritis ischaemic optic neuropathy)
Admit, IV methyprednisolone for 3 days prior to starting oral prednisolone
22
Q
What other eye complications do you get in GCA?
A
- PAION (no nerve head swelling)
- Amaourosis fugax (sudden visual loss due to carotid artery occlusion)
- Slow flow retinopathy (pixellation)
- Cilioretinal artery occlusion
- Central retinal artery occlusion (cherry red spot)
- Steroid side effects (cataracts, diabetic retinopathy)
23
Q
What are the symptoms of Takayasus arteritis?
A
- Joint pain
- Systemic (fever, dizziness, claudication, PUO, hypertension)
- Aneurysms
- Absent peripheral pulses
SUSPECT IF JAPANESE, FEMALE, <55
24
Q
How is TA managed?
A
- Oral prednisolone
- Oral cyclophosphamide, tocilizumab
- Endovascular surgery, bypass
25
What are the complications of TA?
- Aortic valve regurgitation
- Aortic aneurysm
- Ischaemic stroke
- Heart failure
26
What are the medium vessel vasculitides?
Vasculitis of the medial and small-sized arteries and arterioles
1. Polyarteritis nodosa (middle aged men, hepatitis B)
2. Kawasakis disease (children, japanese)
27
What investigations should be done in small/medium vessel vasculitis?
Bedside - Urine dip, pulmonary function tests
Bloods - ANCA, FBC, CRP, ESR, infection screen
Imaging - CXR, HRCT, CT sinus, MRI muscles, EMG
Biopsy - if unsure about diagnosis
28
What is the pathophysiology of polyarteritis nodosa?
- Necrotising vasculitis secondary to deposition of immune complexes (hep B?)
- Microaneurysm formation with thrombosis and infarction
- Systemic features tend to precede dramatic acute features due to organ infarction
29
What are the symptoms of polyarteritis nodosa?
- Systemic features
- Mononeuritis multiplex (painful, asymmetrical peripheral neuropathy)
- Abdo pain and GI haemorrhage
- Kidney disease
- MI, pericarditis and heart failure
- Skin bruising and gangrene, livedo reticularis
ONLY ORGAN THAT ISNT COMMONLY AFFECTED IS LUNG
30
What is livedo reticularis?
Mottled purplish discolouration of the skin found in autoimmune vasculitis
31
What are the characteristic investigation findings in PAN?
- High WCC,CRP, ESR
- May have Hep B virology as this can be a driver
- ANCA negative
32
How is PAN managed?
Oral prednisolone and azathioprine
Must control BP meticulously
33
What is the pathophysiology of Kawasakis disease?
Acute systemic vasculitis following infection
34
What are the symptoms of Kawasakis disease?
- Fever > 5 days
- Bilateral conjunctival congestion
- Dry/red lips and strawberry tongue
- Polymorphic rash
- Red, swollen palms and soles of feet
- Coronary aneurysms
35
How is Kawasakis disease managed?
1. Single dose IV immunoglobulin to prevent coronary aneurysms and aspirin
36
What are the small vessel vasculitides?
Vasculitis in small arteries, arterioles, venues and capillaries.
ANCA associated (30-50%):
1. Microscopic polyangitis
2. Granulomatous polyangitis (Wegeners)
3. Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
ANCA negative:
1. Immune complex related (lupus, HSP, RA, good pastures, sjorgens, behcets)
2. Paraneoplastic
3. IBD vasculitis
(Goodpastures anti GBM can be positive or negative!)
37
What are the features of microscopic polyangitis?
- pANCA for MPO
- Kidney and lung involvement
- Recurrent haemoptysis
- Palpable purpura
- Treat with cyclophosphamide
38
What are the features of granulomatous polyangitis?
- Systemic vasculitis in small and medium vessels
- cANCA against PR3
- Classical triad of upper and lower lung involvement and pauci-immune glomerulonephritis (renal)
- Treat with cyclophosphamide/rituximab
39
What are the features of eosinophilic granulomatosis with polyangitis?
- pANCA MPO positive
- Patient typically diagnosed with asthma and develop systemic vasculitis months/years later
- Treat with steroids
- Cardiac involvement causes significant morbidity and mortality
40
What are the features of HSP?
- ANCA negative, IGA raised
- Tetrad of palpable purpuric rash, abdo pain, arthralgia and glomerulonephritis
- Urinalysis shows protein/blood/casts
- Treat with steroids
41
What is the epidemiology of Behcets disease?
- Common in Turkey, Iran, Japan
- Link to HLA-B51 allele
- Young men (20-30)
42
What are the symptoms of Behcets disease?
- ORAL ULCERATION
- Genital ulceration
- Uveitis
- (May have arthritis and GI sx)
43
How is Behcets disease diagnosed?
Pathergy test - needle prick leads to pustule formation within 24-48h
Raised ESR/CRP, negative autoantibodies
44
How is Behcets disease managed?
Prednisolone + azathioprine
| treat mild oral ulcers with topical corticosteroids such as triamcinolone
45
What is the pathophysiology of SLE?
- Polyclonal B-cell secretion of pathogenic autoantibodies against a range of antignes
- Immune complex formation and deposition
- Complement activation
- TISSUE DAMAGE
46
Describe a typical history of a patient with SLE
- Young, female, afrocarribbean/asian
- Relapsing remitting
- Non specific features - malaise, weight loss, fever, sweats
- Specific features - malar/discoid rash, photosensitivity, alopecia, oral ulcer, non-erosive ARTHRITIS, raynauds, serositis, renal dysfunction, seizures, myalgia, vasculitis, livedo reticularis, non-scarring alopecia, proteinura, neuropsychaitrc
47
What would blood tests of an SLE patient look like?
FBC - anaemia, neutropenia, thrombocytopenia, raised ESR, low complement C3/4, NORMAL CRP
Autoantibodies - ANA (95%), dsDNA (60%), anti-Sm/anti-Ro/La (but RoLA more sjorgens)
Urine - casts/protein for lupus nephritis
```
Also might have:
HLAb8/DR2/3
Antihistone antibody - drug-induced lupus
EBV - precipitant
Bchrom - antihistone antibodies (drug induced)
CXR - infiltrates, effusion
```
48
Which other autoimmune conditions is SLE associated with?
- Sjorgens syndrome
- Autoimmune thyroid disease
- Antiphospholipid syndrome
49
How is SLE definitively diagnosed?
Need >4 out of 11 criteria (8 symptoms, abnormal FBC, abnormal antibodies, ANA positive)
Biopsy shows LE cells lupus erythematous cells - (macrophage that has engulfed another cell)
50
What test does SLE cause a false positive for?
VDRL (venereal disease research lab test), used to diagnose syphillis
51
Which drugs can cause SLE?
>50 different drugs, including isoniazid, hydralazine, procainamide, phenytoin, sulfonamides
This is due to production of antihistone antibodies!
MOST COMMON ARE PROCAINAMIDE AND HYDRALAZINE
52
How is SLE managed?
a) flares
b) maintenance
c) cutaneous
d) lupus nephritis
e) b cell depletion
FLARES - IV cyclophosphamide, steroids
MAINTENANCE - NSAIDS, hydroxychlorquine (DMARD), low dose steroids, immunosuppressants eg. methotrexate
CUTANEOUS - suncream, topical steroids
LUPUS NEPHRITIS - cyclophosphamide/mycophenolate, steroids, control BP rigorously!
B CELL DEPLETION - biologics (rituximab, belimumab)
53
What is the pathophysiology of antiphospholipid syndrome?
Production of antiphospholipid antibodies (anticardiolipin, lupus anticoagulant, anti-beta2-glycoprotein 1) leading to recurrent thrombosis and pregnancy-related morbidity
54
What are the symptoms of antiphospholipid syndrome?
CLOT
Coagulation defect
Livedo reticularis
Obstetric (recurrent miscarriage)
Thrombocytopenia
May also have valvulopathy, MI, amaurosis fugax, retinal thrombosis, adrenal infarction, renal complications
55
How is APL syndrome diagnosed?
Need at least 1 clinical and 1 lab:
CLINICAL - history of vascular thrombosis OR pregnancy morbidity
LAB - elevated IgG/IgM/anticardiolipin/antib2GP1 OR lupus anticoagulant on 2 occasions, 12 weeks apart
(nb - ANA, dsDNA are also elevated in underlying SLE but not specific to APL)
Tend to have a paradoxical prolonged APTT and thrombocytopenia
56
How is APL syndrome managed?
Primary thromboprophylaxis - low dose aspirin
Secondary thromboprophylaxis:
Initial VTE- lifelong warfarin target INR 2-3
VTE on warfarin - add in aspirin, aim INR 3-4
Arterial thrombosis - lifelong wararin target INR 2-3
57
What is discoid lupus?
Benign variant of lupus involving only skin - characteristic facial rash and erythematous plaques
58
What is the pathophysiology of Sjogrens?
Lymphocytic infiltration and destruction of salivary and lacrimal glands resulting in dry mucosal surfaces
PRIMARY - occurs alone
SECONDARY - assoc with SLE, RA, systemic sclerosis
59
What are the symptoms of Sjogrens?
Dry eyes (keratoconjunctivitis sicca)
Dry mouth (xerostomia)
Vaginal dryness
Dry cough
Arthralgia/fatigue
Raynauds
Sensory polyneuropathy
Recurrent episodes of parotitis
Renal tubular acidosis
60
How is Sjogrens diagnosed?
AUTOANTIBODIES - Positive anti-Ro (SS-A), anti-La (SS-B) and ANA/RF, low C4
SCHIRMERS TEST - filter paper placed in lower eyelid, wetting of <5mm in 5 min is positive for sjogrens
Can do sialometry, biopsy, MRI/US of salivary glands if unsure
61
What does anti-60 kD Ro antibody indicate?
Indicates skin involvement
.This antibody can cross the placenta to the baby and cause fetal heart block
62
What is the most worrying complication of Sjogrens syndrome?
Increased risk of non-hodgkins lymphoma (particularly MALT)
63
How is Sjogrens managed?
SYMPTOMATIC - artificial tears/saliva, pilocarpine (cholinergic)
SYSTEMIC - hydroxychloroquine (DMARD - reduces inflammatory process)
64
What are the three patterns of disease in systemic sclerosis?
Limited cutaneous SS:
- Raynauds initially
- Affects face and distal limbs
- Positive anti-centromere antibodies
- CREST syndrome is a subtype!
Diffuse cutaneous SS:
- Affects trunk and proximal limbs
- Positive anti scl-70 antibodies
- Death due to respiratory involvement (ILD, PAH)
Scleroderma:
- Tightening and fibrosis of ski
- May manifest as plaques (morphoea) or linear
- No internal organ involvement
65
What is the pathophysiology of systemic sclerosis?
Autoimmune and vascular dysfunction, leading to fibrosis and small vessel occlusion.
ANA positive in 90%
Highest mortality of any autoimmune rheumatic disease!
66
What is CREST syndrome?
Calcinosis
Raynauds
Esophageal (GI involvement)
Sclerodactly
Telangectasia
67
What investigations should be done in a patient with SS and why?
```
Urine dip/BP - renal
Nail fold capillaroscopy - nail changes
CXR - fibrosis, ILD
PFTs - restrictive in ILD
HRCT - pulmonary htn
Serial echo/BNP - diffuse cardiac involvement
OGD - GI involvement
```
68
What is the most worrying complication of SS?
Scleroderma renal crisis - abrupt onset hypertension, renal failure, hypertensive encephalopathy and heart failure.
Precipitated by high dose steroids! Protect with ACEis
69
What are the 3 stages of Raynauds?
White - vasoconstriction
Blue - cyanosis
Red - rapid blood reflow, painful
70
Which factors exacerbate raynauds?
Cold, female, smoking, polycythemia, hypercholesterolaemia
71
How is Raynauds managed?
- Hand warmers
- Vasodilators (Nifedipine, ACEis, ARBs
- 2nd line IV epoprostenol (prostacyclin)
- If ulcers give sildenafil and wound care
Refer to secondary care if suspected secondary Raynaud's phenomenon
72
How is SS managed?
Manage each organ separately to improve function - no cure for SS itself
73
What is the mechanism behind polymyositis/dermatomyositis
Disorder of unknown cause, involves inflammation of striated muscle causing insidious proximal muscle weakness (and skin involvement)
74
What are the features of an inflammatory myositis?
Muscle weakness, symmetrical and affecting proximal limbs first
Insidious onset but may progress rapidly.
May cause dysphonia/resp failure as condition progresses
Often spares ocular muscles
Often there are few other CTD features except ILD, but systemic features include fever, weight loss, morning stiffness
75
What are the characteristic cutaneous manifestations of someone with dermatomyositis?
- Gottrons papules (lichenoid roughened papules on knuckles)
- Flagelate
- Macular shawl sign
- Purple heliotrope rash on eyelids with oedema
- Dilated capillary nail fold loops
- Subcutaneous calcifications
76
What blood tests should be done in a patient with suspected polymyositis/dermatomyositis?
Antibodies - Anti-Jo1 (poly), AntiMi2 (dermo), antisynthetase syndrome, nucleolar ANA
Bloods - CK, LDH, AST, ALT raised (muscle enzymes)
77
What would EMG, MRI and muscle biopsy show for a patient with PM/DM?
EMG - characteristic fibrillation potentials
MRI - oedema in acute myositis
Muscle biopsy - endomysial infiltrates (DIAGNOSTIC)
78
What is the most worrying complication of dermatomyositis?
Malignancy - DM can be a paraneplastic disease (ovarian, breast and lung most common)
79
What could cause elevated CK?
Statins
- Long lie (rhabdomyolysis)
- DM/PM
- Hypothyroidism
- MI
80
In which cases would an elevated CK prompt you to do a muscle biopsy?
1. Abnormal EMG
3. CK 3x the upper limit of normal
4. Age<25
5. Exercise intolerance
81
How are PM/DM managed?
1. Prednisolone and azathioprine
2. Hydroxychloroquinine or topical tacrolimus for skin
3. Screen for malignancy
82
What is a mixed CTD?
Mixture of RA, SLE, myositis and SS (may not be a distinct disease entity!)
Often have pulmonary HTN and erosive arthritis
83
What investigation confirms mixed CTD?
Anti-RNP
84
How are a CTDs managed generally?
Severe flares - cyclophosphamide
Rash - topical steroids
Joint pains - hydroxychloroquinine
Renal - immunosuppression and BP control
New treatment - interferons/interleukins
85
What pre pregnancy planning do people with a CTDs require?
Obstetrician + rheumatologist led
- Screening for Ro/La and foetal cardiac scan if present
- Antibody testing in those with lupus
- Need low dose aspiring and prednisolone for flare ups
- Advise against if active disease, stage 4/5 CKD, pulmonary HTN
86
Which a) DMARDs and b) biologics are safe in pregnancy?
a) Azathioprine, IV immunoglobulin
| b) Rituximab ok in 1st trim
87
What are the principles of treatment of vasculitis?
Induce remission - cyclophosphamide, steroids, rituximab
Maintenance - methotrexate, azathioprine
Treat relapses - rituximab
Treat severe cases - plasma exchange
Also need to keep BP under control to avoid renal complications
88
How would HepC small vessel vasculitis present?
Vasculitis that only comes on in cold conditions
89
What are the signs of large vessel vasculitis?
Claudication
Absent pulses
Bruit
Assymetric BP
90
What are the signs of medium vessel vasculitis?
Livedo reticularis
Gangrene/ulceration
Mononeuritis multiplex
Microaneurysms
91
What are the signs of small vessel vasculitis?
Purpura
Glomerulonephritis
Alveolar haemorrhage
Eye/ENT involvement
92
What is pyoderma gangrenonsum?
Deep ulcerating lesion with violet border on leg, abdomen or face, associated with vasculitis (wengers) and IBD.
Give oral steroids/ciclosporin
93
What is eytherma nodosum
Painful, purple red lesions on shins due to infection, sarcoidosis, IBD
94
What is fibromyalgia and how is it managed?
Chronic pain syndrome defined by:
1. Presence of widespread body pain for at least 3 months AND
2. At least 11/18 tender points
Diagnosis is CLINICAL - often comorbid psychiatric disorder. Exclude other aCTDs.
Manage with TCA (amitriptyline, cyclobenzaprine) or SSRI (duloxetine) and CBT
95
What is CFS and how is it managed?
Diagnosis:
- 3 month hx of disabling fatigue >50% of the time in absence of other disease
Management:
- Refer to CFS specialist
- Energy management
- CBT
96
What is Ehlers-Danlos syndrome?
Connective tissue disorder characterised by:
1. Joint hypermobility
2. Skin hyper extensibility
3. Tissue fragility
There are 13 subtypes - hypermobile is most common
97
What causes EDS?
Mutations in COL5A1/2, COL1A1 for classical type
Mutations in TNXB for hypermobile type (otherwise cause unknown for this type)
Mutations in COL3A1 for vascular type
Affects genes encoding for collagen/fibrillin and other matrix proteins.
98
How do patients with EDS present?
JOST GAPES
```
Joints and Other Soft Tissues
Gut
Allergy/atopy/autoimmune
Postural symptoms
Exhaustion
Skin (soft and silky, semi-transparent, poor wound healing, bruising)
```
99
How is EDS diagnosed?
PRIMARILY CLINICAL DIAGNOSIS
- Can use Beighton score to judge joint hyper mobility
- Genetic testing
- Tilt-table to test CVS
- Spinal x-ray may show kyphoscloiosis
100
How is EDS managed?
CONSERVATIVE: Genetic counselling/education, pain management/PT/OT, orthotics
MEDICAL: antidepressants/analgesia
SURGICAL: joint reduction and immobilisation (when dislocated)
101
What are the complications of EDS?
- Valvular heart disease (mitral valve prolapse)
- Aneurysms (in vascular EDS)
- Organ rupture incl uterine rupture (vEDS)
- GI bleed
102
What is Marfan's syndrome? How is it diagnosed?
AD connective tissue disorder involving decreased extracellular microfibril formation and poor elastic fibres. FBN1 gene.
Major diagnostic criteria (>2 of):
1. Lens dislocation
2. Aortic dissection/dilatation
3. Dural ectasia
4. Skeletal features (arachnodactyly, long arm span, pacts deformity, scoliosis, pes planus = flat foot)
Minor:
1. Mitral valve prolapse
2. High arched palate
3. Joint hypermobility
103
What is dural ectasia?
Widening of ballooning of the dural sac surrounding the spinal cord - found in Marfan's, neurofibromatosis, vertebral fracture, incomplete spinal anaesthesia
Diagnose on MRI
104
What investigations should be ordered in Marfan's?
Echocardiogram, CT, slit-lamp eye exam, abdo USS, FBN1 gene mutation
105
How is Marfan's syndrome managed?
1. B-blockers (metoprolol) to slow aortic dilatation
2. Annual echogram and elective surgical repair when aortic diameter >5cm (need life long anticoagulant post op)
3. Corrective lens
4. Orthopaedic bracing for spinal deformity
106
What is sarcoidosis? What is the pathology?
Chronic granulomatous disorder affecting LUNG, SKIN and EYES.
Accumulation of lymphocytes and macrophages and formation of non-caveating granulomas. Aetology unknown but associated with HLA-DRB1 and DQB1 alleles.
107
What are the clinical features of sarcoidosis?
Acute - erythema nodosum and polyarthralgia (lofgrens)
Chronic - asymptomatic, pulmonary disease (dry cough, dyspnoea, chest pain), skin (nodules, lupus pernio), organomegaly, liver disease, heart failure, kidney stones (hypercalcemia), bells palsy
PEAKS IN WOMEN AGED 20S AND 50S WITH UNPREDICTABLE CLINICAL COURSE
108
What do investigations show in sarcoidosis?
BEDSIDE:
- Raised urinary calcium
- Abnormal PFTs
BLOODS:
- Raised ESR, ACE, calcium, immunoglobulins, LFTs
- Lymphopenia
IMAGING:
- US shows nephrocalcinosis/hepatosplenomegaly
- Bone x-rays show punched out lesions
- CT/MRI to stage
- PET shows active areas of inflammation
SPECIAL:
- Biopsy shows non-caseating granulomas (DIAGNOSTIC!!)
- Broncheolar lavage shows increased lymphocytes and neutrophils
109
What indicates poorer prognosis in sarcoidosis?
- Afro-caribbean
- Lupus pernio
- Chronic hypercalcaemia
- Chronic pulmonary involvement
110
Describe the staging of sarcoidosis on CXR
Stage 0 - normal
Stage 1 - bilateral hilarity lymphadenopathy (BHL)
Stage 2 - BHL plus pulmonary infiltrates
Stage 3 - Pulmonary infiltrates alone
Stage 4 - Extensive fibrosis with distortion
111
What is broncheolar lavage?
1. Bronchoscope passed through the mouth/nose into lungs
2. Fluid squirted into lung and collected to examine
Shows increased lymphocytes in active disease and increased neutrophils in pulmonary infiltrate
112
How is acute sarcoidosis managed?
Bed rest and NSAIDS, self limiting!
113
How is chronic sarcoidosis managed?
1. Steroids - oral or IV depending on severity, IV if respiratory failure
2. Immunosuppresants (methotrexate, hydorxychloroquinine, cyclosporin)
3. Lung transplant
114
What is the prognosis for sarcoidosis?
```
Rates of spontaneous remission:
Stage 1 (55-90%)
Stage 2 (40-70%)
Stage 3 (20%)
Stage 4 (no remission)
```
115
What is the typical patient with sarcoidosis?
20-40 year old black female patient presenting with dry cough and SOB, may have nodules on shins (erythema nodosum)
116
What is Lofgrens syndrome?
Specific presentation in sarcoidosis, triad of:
1. Erythema nodosum
2. Polyarthragia
3. BHL
117
What are some DDS for sarcoidosis?
- TB
- Lymphoma
- Hypersensitivity pneumonitis
- HIV
- Toxoplasmosis/histoplasmosis
- Malignancy
118
What is the key blood test in sarcoidosis?
Raised serum ACE
119
What are the 4 types of hypersensitivity?
Type 1 - Anaphylactic (IgE)
Type 2 - Cell bound (IgG/IgM)
Type 3 - Complex (IgG, IgA)
Type 4 - Delayed (T cell mediated)
Type 5 - Antibodies
Anaphylaxis
antiBody
Complex
Delayed
ABCD
120
What considerations should be taken into account when prescribing azathiprine?
1. Check TPMT levels first - if low levels there is high risk of adverse effects including bone marrow suppression
2. Prescribe lower dose if also using allopurinol
3. Risk of non-melanoma skin cancer
4. Safe in pregnancy
121
What considerations should be taken into account when prescribing sulfsalazine?
- Caution in G6PD deficiency or allergy to aspirin/sulphonamide (cross-sensitivity)
- Can cause oligospermia, steven johnson, pneumonitis, myelosuppression
- May colour tears
- Safe in pregnancy and breastfeeding
121
What considerations should be taken into account when prescribing hydroxychloroquine?
- Patients need a baseline ophthalmologic examination and annual screening due to risk of bull's eye retinopathy
- Safe in pregnancy
122
What considerations should be taken into account when prescribing methotrexate?
- Taken weekly
- Monitor FBC, U&Es and LFTs before treatment and weekly until stabilised
- After this monitor every 2-3 months
- Folic acid OW co-prescribed, take >24h after methotrexate
- Avoid with trimethoprim or co-trimoxazole
- Not safe in pregnancy - contraception for at least 6m after treatment has stopped
Adverse effects:
- Mucositis
- Myelosuppression
- Pneumonitis (1y after)
- Pulmonary fibrosis
- Liver fibrosis
123
What are the side effects of etanercrept?
- Demyelination
- Reactivation of TB
124
What are the side effects of leflunomide?
- Liver impairment
- Interstitial lung disease
- Hypertension
125
What are the features of Berger's disease?
- aka IgA nephropathy
- Most common primary glomerulonephriti
- Affects men in 2nd-3rd decade of life
- Presents as visible haematuria and flank pain (nephritic syndrome) 48h after URTI/GI infection
- Treat with high dose prednisolone or immunosuppression
- High risk of end-stage renal failure
THIS IS DIFFERENT TO BUERGERS DISEASE WHICH IS PAD SYMPTOMSI N YOUNG SMOKERS
126
What are the features of Alport syndrome?
- Genetic disorder characterised by glomerulonephritis, sensorineural hearing loss and eye abnormalities
- Caused by a genetic defect of type 4 collagen, usually X-linked
- Risk of renal failure, definitive treatment is kidney translant
