Rheumatology Flashcards
1
Q
What are the symptoms of rheumatoid arthritis?
A
Pain Stiffness Early morning stiffness >1 hour Joint swelling Small joints > large joints Symmetrical Persistent - generally accumulates over time
2
Q
What are the signs of rheumatoid arthritis?
A
Synovitis Swan neck deformity boutonniere Z-thumb Ulnar deviation Rheumatoid nodules
3
Q
What are the ocular features of rheumatoid arthritis?
A
Scleritis
Episcleritis
Sicca syndrome
4
Q
What are the cardiorespiratory features of rheumatoid arthritis?
A
Pleural effusions Pulmonary nodules Pulmonary fibrosis Pericarditis Serositis
5
Q
What are the extra-articular features of rheumatoid arthritis?
A
Ocular features Cardiorespiratory features Splenomegaly Peripheral neuropathy Anaemia Amyloidosis
6
Q
What are the differential diagnoses for rheumatoid arthritis?
A
Polyarticular gout
Psoriatic arthritis
Osteoarthritis
SLE
7
Q
What are the investigations for rheumatoid arthritis?
A
CRP/ESR FBC Bone/urate Immunology Radiograph Ultrasound MRI
8
Q
What is rheumatoid factor?
A
IgM antibody
Directed against Fc portion of IgG Ab
Sensitivity around 70%
Specificity around 85%
9
Q
What is CCP antibody?
A
Inflammation leads to cellular damage. Enzymatic process leads to the conversion of arginine residues to citrulline. Alteration of shape creates a foreign antigen from self - anti citrullinated cyclic peptide antibodies.
Sensitivity 66%
Specificity 90%
10
Q
What are the x-ray changes in rheumatoid arthritis?
A
Peri-articular osteopenia Soft tissue swelling Erosion Joint destruction Subluxation
11
Q
What can small joint ultrasound detect in rheumatoid arthritis?
A
Subclinical synovitis.
Allows for distinction between active disease and chronic inactive synovial thickening found in long-standing inactive disease.
12
Q
What are the functional assessments of rheumatoid arthritis?
A
HAQ-DI - health assessment questionnaire disability index.
SF-36 - Short form 36
13
Q
What score on the EULAR classification criteria would fulfil the classification criteria for RA?
A
6 or more
14
Q
What is the system used for disease monitoring in rheumatoid arthritis?
A
DAS-28
15
Q
What is the step-up approach in initiating therapy for rheumatoid arthritis?
A
Introduce and escalate single drug to max tolerated dose, if ongoing disease activity add a further drug.
16
Q
What is the step-down approach to initiating therapy for rheumatoid arthritis?
A
Several drugs started at once and then gradually withdrawn.
17
Q
What is the parallel approach to initiating therapy for rheumatoid arthritis?
A
Combination introduced at same time and maintained.
18
Q
What is the initial therapy in rheumatoid arthritis?
A
NSAIDs
COX-2 inhibitors e.g. etoricoxib
Steroids e.g. oral prednisolone
19
Q
When should DMARDs be offered in rheumatoid arthritis?
A
First line and within 3 months of symptom onset.
20
Q
Which DMARDs can be offered in rheumatoid arthritis?
A
Methotrexate
Lefluonomide
Sulfasalazine
Hydroxychloroquine if mild or palindromic disease.
21
Q
How often is methotrexate taken in rheumatoid arthritis?
A
Once weekly
22
Q
What is methotrexate an antagonist of?
A
Folate
23
Q
What are the side effects of methotrexate?
A
Usually mucosal or GI side effects.
24
Q
What are the monitoring requirements for patients taking methotrexate?
A
FBC
LFTs
25
When is methotrexate contraindicated?
Pregnancy
26
what are the actions of sulfasalazine?
Immunomodulatory. Several actions, including against folate, T and b cells.
27
How often should sulfasalazine been taken for rheumatoid arthritis?
Daily pill
28
What are the side effects of sulfasalazine?
GI
Headache
Rash
29
Is sulfasalazine safe in pregnancy?
Yes - but folic acid 10mg daily, started 3 months pre-conception.
30
What is the criteria for commencing biologic therapy?
Must have failed on at least 2 csDMARDs, one of which must be methotrexate unless contraindicated.
Must have severe disease ie. DAS-28 >5.1 on at least 2 occasions one month apart.
Treatment to be continued only if adequate response within first six months, defined as a reduction in DAS-28 of >1.2.
31
What screening should take place before commencement of DMARDs?
Viral hepatitis and HIV
Varicella
CXR and IGRA (TB)
Vaccination - influenza, pneumococcal
32
What are the contraindications for starting biologics?
```
Active infection
Active or latent TB
Pregnancy
Malignancy
Diverticular disease (IL-6)
```
33
What should be monitored in a patient on biologics?
Infections
Malignancy
Bloods (FBC, LFTs)
Awareness with vaccination
34
What is an example of a novel synthetic DMARD?
JAK inhibitors
35
What are the side effects of JAK inhibitors?
Side effects - viral infection including troublesome HSV infection
DVT risk in over 50s.
36
Who is involved in the MDT of a patient with rheumatoid arthritis?
Occupational therapists
Physiotherapists
Nurses
Podiatry
37
What are sernogeative spondyloarthropathies?
A group of diseases involving the axial skeleton and express a variety of extra-skeletal signs and symptoms. They are RF negative and associated with HLA-B27.
38
Give 6 examples of diseases that are seronegative spondyloarthropathies.
```
Psoriatic arthritis
Ankylosing spondylitis
Reactive arthritis
Enteropathic arthropathy
Juvenile idiopathic arthritis
Behcet's disease
```
39
What are the symptoms of ankylosing spondylitis?
```
Inflammatory back pain
Early morning stiffness
Improves with exercise.
Associated with peripheral arthritis.
Enthesitis common.
```
40
Who gets ankylosing spondylitis?
M:F 5:1
| Mean age on onset 3rd decade.
41
Which imaging is used to investigate ankylosing spondylitis?
X-ray
| MRI (most helpful)
42
How should ankylosing spondylitis be treated?
NSAIDs and anti-TNF.
43
What is reactive arthritis?
Asymmetrical inflammatory arthritis that usually occurs after a diarrhoeal illness or urethritis.
44
What are the differential diagnosis for reactive arthritis?
```
Post-viral arthritis
Reactive arthritis
SLE
Psoriatic arthritis
Polyarticuclar gout
Polyarticular OA
```
45
How many joints does reactive arthritis usually affect?
Mean = 3
Monoarticular in 10%
46
Which joints does reactive arthritis most commonly affect?
Knees
Ankles
Feet
47
What is the management of reactive arthritis?
```
Treat underlying infection
Culture stool if diarrhoea
Sexual health review
X-ray of affected joint may show enthesitis with periosteal reaction
Rest
NSAIDs
Intra-articular steroid
DMARDs
```
48
What is the prognosis of reactive arthritis?
Most initial attacks resolve within 6 months.
50% relapse
20% chronic relapsing course
30% establishes, often with lower limb involvement.
49
What are the investigations for a suspected spondyloarthropathy?
```
Bloods:
FBC, U&Es, LFTs
ESR/CRP
RF/Anti CCP antibodies
HLA-B27
X-rays (hands, spine, pelvis)
MRI
```
50
What might hand x-rays show in spondyloarthropathies?
DIP erosion
Lysis of terminal phalanges
Asymmetry
Pencil in cup deformity
51
What might spinal x-rays show in spondyloarthropathies?
Erosions
Vertebral squaring
Syndesmophytes
Fused spine (bamboo spine)
52
What might a pelvic x-ray show in spondyloarthropathies?
Sacroiliitis
53
What might and MRI show in spondyloarthropathies?
Enthesitis
54
What are the common extra-articular features of spondyloarthropathies?
```
Skin - psoriasis, pyoderma gangrenosum, erythema nodosum.
Inflammatory bowel disease
Urethritis and cervicitis
Renal involvement - amyloidosis
Thrombophilia
Respiratory - pulmonary fibrosis
```
55
Which seronegative spondyloarthropathies is uveitis most common in?
Ankylosing spondylitis
| Inflammatory bowel disease
56
What type of uveitis is most common in ankylosing spondylitis?
Anterior, unilateral and acute
57
Which type of uveitis is most common in psoriatic arthritis and inflammatory bowel disease?
Posterior, bilateral and chronic
58
Which type of cardiovascular changes are often seen associated with seronegative spondyloarthropathies?
Aortitis
Mitral valve insufficiency
Fibrosis of the conduction system with varying degrees of AV block.
59
Which type of respiratory involvement is often seen associated with seronegative spondyloarthropathies?
Bilateral apical pulmonary fibrosis (relatively rare)
| Restrictive lung disease.
60
What are the disease assessment tools for ankylosing spondylitis?
BASDAI - Bath ankylosing spondylitis disease activity index.
BASFI - Bath ankylosing spondylitis functional index
BASMI - Bath ankylosing spondylitis metrology index.
61
Describe the BASDAI.
```
Gold standard for measuring and evaluating disease activity in ankylosing spondylitis.
6 questions measuring and evaluating 5 major symptoms:
Fatigue
Spinal pain
Arthralgia or swelling
Areas of localised tenderness
Morning stiffness duration
Morning stiffness severity.
```
Visual analogue scale from 1-10 with average of 2 stiffness scores.
Resultant score 0-50 divided by 5 to give BASDAI score.
62
Which BASDAI score would indicate suboptimal control of disease?
4 or greater.
63
What does the BASFI assess?
Function of a person with ankylosing spondylitis.
64
What is the treatment for spondyloarthropathies?
NSAIDs
Non-pharmacological (education, exercise, physio, self help groups)
Local steroids
DMARDs
Second line: TNF alpha blocker or IL-17 blocker
Additional therapy: analgesics, surgery.
65
What is vasculitis?
Inflammation of blood vessels.
66
How is vasculitis classified?
Primary - categorised by size of vessels:
- large, medium, small
Secondary - groups of diseases thought to be caused by underlying health conditions e.g. SLE, RA, Hep B/C, HIV
67
Give some examples of large vessel vasculitides.
Aortitis
Giant cell arterritis
Polymyalgia rheumatica
Takayasu arteritis
68
Give some examples of medium vessel vasculitides.
Kawasaki disease
| Polyarteritis nodosa
69
Give some examples of small vessel vasculitides.
Anti-glomerular basement membrane disease.
Cryoglobulinemia
Cutaneous leukocytoclastic vasulitis
Drug induced vasculitis
Eosinophilic granulomatosis with polyangiitis
70
What is the clinical presentation of giant cell arteritis?
```
Headache:
- temporal headache with tenderness
- subacute onset
- constant
- little relief with analgesics
Visual symptoms
Jaw claudication occurs in 50%
Polymyalgia rheumatica symptoms in up to 50%
Constitutional upset
```
71
What are the complications of giant cell arteritis?
Visual loss - acute ischaemic optic neuropathy due to vasculitis and interruption of blood flow int eh posterior ciliary arteries. Sudden painless loss of vision, occasionally preceded by amaurosis fugax.
Large vessel vasculitis - vascular stenosis and aneurysms
Cerebrovascular accident - obstruction or occlusion of internal carotid artery or vertebral arteries.
72
How is giant cell arteritis diagnosed?
Clinical presentation
Examination findings - temporal artery asymmetry, thickening, loss of pulsatility, tenderness.
Acute phase response - ESR/CRP, often elevated but not specific.
Temporal artery biopsy - gold standard.
73
What would be found on a temporal artery biopsy in giant cell arteritis?
Interruption of internal elastic laminae with mononuclear inflammatory cell infiltrate within vessel wall. Multinucleated giatn cells are typical but their absence does not exclude a diagnosis.
74
What could be found on ultrasound scan in giant cell arteritis?
Sign of halo, which is hypoechogenic mural thickening.
75
What is the treatment for giant cell arteritis?
If strong clinical suspicion, treat. 1mg/kg/day prednisolone - max 60mg.
IV methylprednisolone if visual symptoms.
Aspirin 75mg daily to reduce ischaemic complications.
76
How long should treatment for giant cell arteritis continue for?
Maintain prednisolone 60mg for 1 month.
Taper to 15mg by 12 weeks.
Aim to discontinue corticosteroids for 12-18 months.
Corticosteroid sparing therapy for patients with disease relapse on steroid sparing: methotrexate, tocilizumab.
77
What is the classic presentation of Henoch Schonlein Purpura?
Classic purpuric rash: buttocks, thighs
Urticarial rash, confluent petchiae, ecchymoses, ulcers.
Arthralgia, arthritis
78
What are the complications of Henoch Schonlein Purpua?
GI: pain, bleeding, diarrhoea
Renal: IgA nephropathy
Urology: orchitis
CNS: very rare
79
What is the management of Henoch Schonlein purpura?
Exclude secondary causes (e.g. multisystem disease, recent infections, recent drugs)
Often no treatment required.
Corticosteroids for certain complications.
80
What is granulomatosis with polyangiitis characterised by?
Granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract and often a pauci-immune glomerulonephritis.
81
Which immunological blood tests should be carried out when there is suspicion of vasculitis?
ANCA
MPO
PR3
82
What does ANCA test for?
Autoantibodies directed against the cytoplasmic constituents of neutrophils and monocytes.
83
What are common treatments for vasculitis?
Prednisolone plus either cyclophosphamide or rituximab.
84
What are the risk factors for septic arthritis?
```
Primary joint disease
Prosthetic joints
Recent joint surgery
Leg ulceration
Intravenous drug abuse
Low socioeconomic status
Immunosuppression
```
85
What is the pathogenesis of septic arthritis?
Direct inoculation
Contigious spread from adjacent bone.
Haematogenous seeding from a distant site
86
What are the causative organisms of septic arthritis?
```
Staph. aureus
MRSA
Streptococci
Gram negative bacteria
Neisseria gonorrhoeae
```
87
Which virulence factors are produced by staph. aureus?
Diverse extra-cellular toxins
Enzymes
Components of bacterial cell wall.
88
What are the clinical features of septic arthritis?
```
Pain
Swelling
Rubor
Restricted movement in a single joint
Fever
```
89
What are the differential diagnosis of acute monoarthritis?
```
Septic arthritis
Crystal arthritis (gout or pseudogout)
Reactive arthritis
Seronegative arthritis
Haemarthrosis
```
90
Which laboratory investigations should be carried out to investigate septic arthritis?
```
FBC and white cell differential
ESR and CRP
U&Es, LFTs
Serum urate
ANA and RF
Reactive arthritis screen
```
91
What should be done in the investigation of septic arthritis?
Joint aspiration before antibiotics - gram stain, microscopy, crystals.
Blood cultures even if apyrexial.
Urinalysis
MSSU
Sputum
Wound swab
Chest x-ray
X-ray of affected joint as baseline
Ultrasound to facilitate diagnosis and guide aspiration.
MRI if osteomyeltis is suspected or deep joint.
92
What is the management of septic arthritis?
Aspirate to dryness
| Antibiotic therapy
93
Which antibiotics should be used in septic arthritis?
Cover staphylococci and streptococci.
Flucloxacillin + gentamicin. Clindamycin if penicillin allergy.
Usual practice to use combination therapy.
2 weeks IV and 4 weeks oral therapy.
6 weeks IV if prosthetic joint.
94
When should methotrexate be withheld?
Any infection
Cytopenia
Overdose
95
What are the common side effects of sulfasalazine?
Headaches
GI disturbance
Blood abnormalities
96
What does sulfasalazine interact with?
Digoxin
97
What are the features of DRESS syndrome?
Rash and fever
Lymphadenopathy
Hepatitis
Haematological abnormaliteis
98
What are possible side effects of hydroxychloroquine?
```
Mild headache
Nausea
Hair bleaching
Increased skin pigmentation
Maculopathy
```
99
List three TNF inhibitors.
Infliximab
Adalimumab
Etanercept
100
What are the possible side effects of TNF inhibitors?
Local skin reactions
Infections
Drug-induced lupus
TNF induced demyelination
101
What happens in an IL-6 blockade?
```
Flu-like illness
Hypercholesterolaemia
Abnormal LFTs
Increased incidence of herpes virus, pneumonia dn URTIs
Skin reactions
```
102
Name 2 JAK inhibitors.
Baricitinib
| Tofacitinib
103
Name 5 different types of psoriasis.
```
Plaque psoriasis
Flexural psoriasis
Erythrodermic psoriasis
Pustular psoriasis
Guttate psoriasis
```
104
Describe plaque psoriasis.
Well-circumscribed, erythematous, flat-topped plaques with adherent silvery scale on extensor srufaces, scalp, trunk, or buttocks.
105
Describe flexural psoriasis.
Characterised by erythematous plaques with minimal scale in skin folds or flexural surfaces.
106
Describe erythrodermic psoriasis.
Generalised erythema covering nearly the entire body surface with varying degrees of scaling.
107
Describe pustular psoriasis.
Localised pustules involving soles and palms occurring with or without plaque-type disease.
108
Describe guttate psoriasis.
Characterised by 1-10mm pink papules with fine scale primarily on trunk and proximal extremities.
109
What happens in psoriatic nail dystrophy?
```
Discoloured nails
Onycholysis
Nail pitting
Ridging
Subungual hyperkeratosis
```
110
What are the clinical features of psoriatic arthritis?
Women more often present with a symmetric polyarticular arthritis, while men tend to have more spinal involvement.
Peripheral joint disease occurs in most patients, usually as a polyarthritis (>5 joints affected).
Assymetric
Distal interphalangeal joint involvement is common.
Severity of arthritis and skin symptoms usually do not correlate.
Axial disease in about 5% of patients.
111
What are the 5 clinical forms of psoriatic arthritis?
```
Oligoarticular
Asymmetric DIP form
Symmetrical polyarthritis
Psoriatic spondyloarthropathy
Arthritis mutilans
```
112
What are the risk factors for psoriatic arthritis?
```
Genetic risk factors - first degree relatives, HLA-Cw6 or HLA-B subtypes
Recent trauma
Psoriasis
Obesity
Alcohol excess
Smoking
```
113
What is the pathogenesis of psoriatic arthritis?
T cell activation and proliferation in skin. T cells amplify chronic inflammatory disease process.
CD4+ T cells more frequent in dermal papillae and joints.
CD8+ T cells more frequent in epidermis and inflamed enthesis.
Multiple cytokines released, causing further destruction.
Elevated TNF levels increase frequency of circulating osteoclast precursors, which may result in osteolysis.
114
Which criteria must be met to diagnose psoriatic arthritis?
Caspar criteria
115
Which investigations would be carried out in cases of suspected psoriatic arthritis?
```
Bloods:
- ESR/CRP
- FBC
- Rheumatoid factor
- Synovial fluid aspirate to rule out crystals
Radiology:
- Plain x-rays
- US
- MRI
```
116
What are the signs of psoriatic arthritis on x-ray?
Mild bony erosion at edge of cartilage with bone proliferation.
Asymmetric erosive changes in small joints of the hands and feet.
DIP/PIP involvement.
Erosion of distal tuft of distal phalanx
Periostitis.
117
When should biologics be considered in psoriatic arthritis?
If patient has inadequate response to at least one DMARD.
Active enthesitis, dactylitis and insufficient response to NSAIDs.
Active axial disease.
>3 tender/swollen joints.
Two other DMARDs have not worked.
118
Which tool is used to express the severity of psoriasis?
PASI
119
What is polymyalgia rheumatica?
A chronic, inflammatory rheumatic disease characterised by muscle pain and stiffness, particularly affecting the neck, shoulders and hips.
120
What are the clinical features of polymyalgia rheumatica?
Muscle pain in neck, shoulder or pelvic girdle for >2 weeks.
Neck stiffness, especially in morning.
Eleveated ESR or CRP.
121
Which systemic symptoms occur in about half of patients with polymyalgia rheumatica?
Fatigue
Anorexia
Low-grade fever
122
Which criteria would help you make a diagnosis of polymyalgia rheumatica?
Age over 50
Duration more than 2 weeks
Bilateral shoulder and pelvic girdle aching.
Morning stiffness lasting more than 45 minutes.
Evidence of acute phase response.
123
Which investigations should be carried out in suspected polymyalgia rheumatica?
```
FBC, biochem, ESR, CRP
TFTs, CK, RF, CCP
Igs and PEP
Vit D
Chest x-ray
Echo
CT CAP
```
124
What is the differential diagnosis of polymyalgia rheumatica?
```
Giant cell arteritis
Osteoarthritis
Rheumatoid arthritis
Polymyositis
Fibromyalgia
Active infection or malignancy
Myalgia due to statins
Hyperparathyroid
Hypothyroid
```
125
What is paraneoplastic syndrome?
A group of rare disorders triggered by an abnormal tumour response to a cancerous tumour.
126
What is the treatment for polymyalgia rheumatica?
Start prednisolong 15mg/day for 3 weeks, taper over 1-2 years.
127
What are the side effects of steroid therapy?
```
Weight gain
Osteoporosis
Adrenal suppression
Cushing's syndrome
Peptic ulceration
Pancreatitis
Candidiasis
Cataracts
Increased susceptibility of infection
Hypertension
Diabetes
Depression
Psychosis
```
128
What is Reiter's syndrome?
Arthritis, conjunctivitis, urethritis
129
Which joints are most likely to be affected by pseudogout?
Wrists
Knees
Elbows
130
Which type of crystals are found in pseudogout?
Weaky positive, bi-refringent, rhomboid shaped crystals
131
What is the treatment for pseudogout?
```
NSAIDs
Colchicine
Analgesia
Joint aspiration and injection - local anaesthetic +/- steroids
Systemic steroids
```
132
What are the predisposing factors to gout?
```
Male>Female
Family history
Previous attack
Body size
Alcohol
Purine rich diet
Diuretics
Increased turnover (psoriasis/chemo)
Renal diseases
```
133
Which kind of crystals are found in gout?
Negatively, bi-refringent, needle shape crystals
134
What is the treatment for gout?
NSAIDs
Steroids
Colchicine
135
What can be taken as prophylaxis for gout?
Allopurinol
136
What is lupus?
A multi-system autoimmune disease characterised by vasculopathy, vasculitis, inflammation.
137
What is the pathogenesis of lupus?
Autoantibodies target nuclear antigens.
138
What are the signs and symptoms of lupus?
```
Malar rash
Raynaud's
Cytopenia
Nephritis
Alopecia
Sicca
Photosensitivity
Arthralgia
```
139
What are the investigations of lupus?
```
FBC
ESR
CRP
Serum albumin
Serum creatinine
Urinalysis
Urine protein/creatinine ratio
Renal biopsy (if lupus nephritis suspected)
Plain x-rays of hands and feet
Autoantibodies
```
140
Which autoantibodies should be measured in suspected lupus?
```
Antinuclear antibody (ANA)
Anti-double stranded DNA antibody (dsDNA)
Anti-Ro antibody
Anti-La antibody
Anti-RNP antibody
Anti-Sm antibody
Anti-phospholipid antibody
C3, C4
```
141
Which laboratory abnormalities might be found in lupus?
```
Haemolytic anaemia
Leukopaenia
Thrombocytopenia
ANA>1:80
dsDNA positive
```
142
How many of the ACR criteria are required for a diagnosis of lupus?
4 of 11 serially or simultaneously.
143
What is on the ACR criteria for lupus?
```
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Renal disorder
Neurologic disorder
Haematologic disorder
Immunologic disorder
ANA
```
144
How many of the SLICC criteria are needed for a diagnosis of lupus?
4 criteria, at least 1 clinical and 1 immunological or lupus nephritis in presence of ANA or anti-dsDNA.
145
How is SLE treated generally?
```
NSAIDs
Smoking cessation
Sunblock
Exercise
Assess comorbidities
```
146
How is mild SLE treated?
NSAIDs
| Hydroxychloroquine
147
How is moderate SLE treated?
```
Corticosteroids
Methotrexate
Mycophenylate
Azathioprine
Cyclosporine A
```
148
How is severe SLE treated?
Cyclophosphamide
| IV pulsed 6 x 3 weekly
149
What is Sjogren's syndrome?
A chronic inflammatory disorder characterised by dysfunction of the lacrimal and salivary glands.
150
What are are the classification criteria for Sjogren's syndrome?
• Ocular symptoms of inadequate tear production
• Ocular signs of inadequate tear production
• Oral symptoms of decreased saliva production
• Tests indicating decreased salivary gland function
• Salivary gland histopathology
• Presence of characteristic autoantibodies (anti
ro/SSA and/or anti la/SSB)
151
How can salivary and lacrimal gland function be tested?
Shirmer's test
| Unstimulated salivary flow test.
152
What is the pathophysiology of Sjorgens syndrome?
Immune-mediate inflammation.
Targets exocrine glands of eyes and mouth.
Extensive lymphocytic infiltration in germ-centre type foci.
Glandular and ductal atrophy.
153
Which antibodies are found in patients with sjorgen's syndrome?
Anti-Ro
| Anti-La
154
What are the clinical symptoms of primary sjorgen's syndrome?
Dry eyes - grittiness, foreign body, irritation
Dry mouth - difficulty swallowing dry food, dental caries, decreased taste sensation, change in oral flora.
Vaginal dryness
155
What are the other features of sjorgen's syndrome?
```
Arthralgia
Interstitial lung disease
GI tract problems
Interstitial nephritis
Neuropathy
```
156
What is a long term complication of Sjorgen's syndrome?
Lymphoma
157
What are the risk factors for lymphoma in people with Sjorgen's?
Parotid englargement
Lymphadenopathy
Cryoglobulins/positive rheumatoid factor/low C4
Palpable purpura
158
What is the treatment for sjorgan's syndrome?
Treat symptoms
Sprays and gels for eyes and mouth.
Hydroxychloroquine for systemic disease.
Methotrexate if significant inflammatory arthritis.
Intermittent low dose prednisolone for systemic flares.
159
What is scleroderma characterised by?
Thickening of the skin and involvement of the visceral organs, including lungs, heart, GI tract and kidneys.
160
Which antibody is associated with scleroderma?
ANA
161
What are the classifications of scleroderma?
Diffuse cutaneous
Limited cutaneous
Localised
162
What are the features of diffuse cutaneous scleroderma?
Distal and proximal skin thickening, often trunk and face.
Rapid progression of skin change.
Rapid onset after Raynaud's phenomenon.
Early appearance of visceral involvement.
Poor prognosis.
163
What are the featurs of limited cutaneous scleroderma?
```
CREST
Calcinosis
Raynaud's
Oesophaegeal involvment
Sclerodactly
Telangiectasia
```
164
What are the clinical features of scleroderma?
Lungs - 2/3s of patients affected by interstitial fibrosis or pulmonary hypertension.
Cardiac - pericarditis, heart failure, arrythmias, myocardial fibrosis, myocarditis
Kidneys -
165
What are the investigations in scleroderma?
```
FBC, ESR
U&Es
Urinalysis
Immunology: ANA, anti-Scl 70 antibody
anticentromere antibody
```
166
What are the clinical features of the inflammatory myopathies?
Proximal muscle weakness
Muscle atrophy
Dysphagia
Dyspnoea
167
What happens in polymyositis?
Symmetrical proximal muscle weakness
168
What will be elevated in polymyositis?
Creatinine kinase.
169
What are the investigations of myositis?
Elevated muscle enzymes including CK, LDH, aldolase
ESR elevated
ANA positive
MRI shows inflammatory changes and muscle oedema
Muscle biopsy - definitive diagnosis
170
What are the clinical features of dermatomyositis?
Heliotrope rash
| Gottron's papules
171
What is the treatment of inflammatory myopathies?
Prednisolone 1mg/kg for 4-8 weeks.
Methorexate, azathioprine, mycophenolate as steroid sparing agents.
Patients with Anti-Jo-1 antibodies require long term immunosuppresion.
172
What can anti-ro and anti-la antibodies cause in pregnancy?
Neonatal lupus syndrome
173
What are the safest DMARDs in pregnancy?
Sulfasalazine and antimalarials.
