Rheumatology Master Deck Flashcards
(168 cards)
1
Q
Differences between arthropathy, arthritis and arthralgia?
A
Arthropathy - disease of a joint
Arthritis - inflammation of a joint
Arthralgia - pain in a joint
2
Q
2 main categories of arthritis?
A
- Non-inflammatory arthritis (commonly osteoarthritis)
* Inflammatory arthritis
3
Q
Sub-divide inflammatory arthritis?
A
Seropositive arthritis - antibodies present in serum, inc. RA and connective tissue disease
Seronegative inflammatory arthropathy
4
Q
Overall classifications of inflammatory arthritis?
A
PICTURE 1 IN UNI
5
Q
Which antibodies are assoc. with which auto-immune condition?
A
PICTURE 2 IN UNI
6
Q
Describe osteoarthritis (OA)
A
Most common form of arthritis; primarily, a degenerative disorder, where, over time, normal structure of the bone is subject to wear and tear
There may be assoc. inflammation, leading to periodic flares
7
Q
Pathophysiology of osteoarthritis?
A
Imbalance between wear and repair of cartilage within joints
8
Q
Types of OA?
A
Primary - no causative factor found
Secondary - causative factor is found, e.g: previous injury, crystal arthropathy, genu varum/valgum
9
Q
4 signs of OA on X-ray?
A
LOSS:
- Loss of joint space
- Osteophytes
- Subchondral sclerosis
- Subchondral cysts
PICTURE 3 IN UNI
10
Q
Management of OA?
A
Simple analgesia for pain control; mild opiates may be helpful
Physiotherapy to strengthen surrounding structures
Weight loss and exercise are also important
Sometimes, surgery is an option
11
Q
4 groups of inflammatory arthropathies?
A
- Seropositive
- Seronegative
- Infectious
- Crystal deposition disorders
12
Q
Typical patterns of joint involvement in: A - Rheumatoid arthritis B - Psoriatic arthritis C - Inflammatory spondylitis D - Osteoarthritis
?
A
PICTURE 4 IN UNI
13
Q
Features suggestive of joint inflammation and inflammatory arthritis?
A
Joint pain with associated swelling • Morning stiffness • Improvement in symptoms with exercise • Synovitis on examination • Raised inflammatory markers (CRP and PV) • Extra-articular symptoms
14
Q
Describe rheumatoid arthritis (RA)
A
Most common seropositive inflammatory arthropathy
It is an autoimmune inflammatory SYMMETRIC polyarthropathy that most commonly affects small joints of the hands and feet; as the disease progresses, large joints can become inv.
15
Q
Occurrence of RA?
A
WOMEN are affected more than men
Peaks between the ages of 35 and 50 years
First-degree relatives are at higher risk
16
Q
Pathogenesis of RA?
A
Immune response is initiated against synovium, leading to synovitis and tenosynovitis
Inflammatory pannus forms which then attacks and denudes articular cartilage, leading to joint destruction
Tendon ruptures and soft tissue damage can occur leading to joint instability and subluxation
17
Q
Triggers of RA?
A
Smoking, infection, trauma, etc
18
Q
Classification criteria for RA in newly presenting patients?
A
A score of 6/10 is required for diagnosis
PICTURE 5 IN UNI
19
Q
Clinical features of RA?
A
Symmetrical synovitis (doughy swelling) with pain and morning stiffness
Hands and feet tend to be inv. early
MCP and PIPs joints are affected, as well as wrists, but DIP joints are NOT AFFECTED
20
Q
What can aggressive/untreated RA lead to?
A
Deformities
21
Q
Spine in RA?
A
With longstanding disease, atlanto-axial subluxation of the cervical spine can develop, leading to cervical spinal cord compression
PICTURE 6 IN UNI
22
Q
Extra-articular manifestations of RA?
A
- Rheumatoid nodule can occur in some RA patients; they are most commonly found on EXTENSOR surfaces or sites of frequent mechanical irritation
- Lung inv. includes pleural effusion, interstitial fibrosis and pulmonary nodules
- CV morbidity and mortality increased
- Ocular inv. is common and inc. keratoconjunctivitis sicca, episcleritis, uveitis, nodular scleritis (may lead to scleromalacia)
23
Q
Ix for RA?
A
Measure rheumatoid factor and anti-CCP antibody (more specific)
CRP, ESR and PV raised
X-ray
US may be helpful in detecting synovial inflammation, if uncertain
24
Q
X-ray features of RA?
A
May show no joint abnormality in early disease; may show:
• Peri-articular osteopenia
• Soft tissue swelling
25
Aim of RA treatment?
Early and aggressive treatment can improve outcomes
Goal is to commence DMARD therapy within 3 months of symptoms onset
26
Treatment of RA?
Simple analgesia, NSAIDs and IM/intra-articular/oral ateroids; these are used short-term, for symptoms relief, as bridging therapy, until DMARDs begin working
DMARD:
1st line is methotrexate
Others - sulfasalazine, hydroxychloroquine, etc
If no response to DMARDs, biologic therapy (anti-TNF drugs)
27
Side effects of DMARDs?
Increased risk of infection
Bone marrow suppression
Reactivation of TB (with anti-TNF drugs)
28
How is disease activity measured?
DAS 28 score, which is a composite score of 4 domains:
• Tender joint count • Swollen joint count
• CRP/ESR
• Visual analogue score (patients own assessment of their disease activity)
29
Values of DAS 28 score and their meaning?
PICTURE 7 IN UNI
30
When are patients eligible for biologic therapy?
DAS 28 score > 5.1
31
Other therapies available for RA?
Physiotherapy and occupational therapy
Podiatrists and orthotists
Surgery for resistant disease (uncommon now)
32
4 types of seronegative arthritis?
1. Ankylosing spondylitis
2. Psoriatic arthritis
3. Enteropathic arthritis
4. Reactive arthritis
33
Characteristics of seronegative arthritis?
Inflammation and/or arthritic disease of the spine, known as spondylarthropathy, and an ASYMMETRIC oligoarthritis
Sacroiliitis, uveitis, dactylitis (inflammation of a digit) and entesopathies are common
34
Two types of entesopathies that are very common in seronegative arthritis?
Achilles insertional tendonitis
Plantar fasciitis
35
Testing for spondyloarthritis?
Patients are often HLA-B27 +ve, although only a minority of these people develop one (it is not a useful diagnostic test)
CRP and ESR usually raised
36
What is ankylosing spondylitis?
Chronic inflammatory disease of the spine and sacroiliac joints, which can lead to eventual fusion of the intervertebral and SI joints
37
Occurrence of ankylosing spondylitis?
MALES are more commonly affected
Age of onset typically between 20-40 years
38
Symptoms of ankylosing spondylitis?
Spinal pain and stiffness; may also develop knee/hip arthritis
Spinal morning stiffness that IMPROVES with EXERCISE
Eventually, loss of spinal movement and ? mark spine develops (loss of lumbar lordosis and increased thoracic kyphosis)
39
Test for lumbar spinal flexion?
Schobers test inv. measuring 5cm below PSIS and 10cm above, whilst the patient is upright
Ask them to bend forward and remeasure the distance
Normally, it should extend beyond 20 cm
40
Conditions assoc. with ankylosing spondylitis?
Anterior uveitis
Aortitis,
Pulmonary fibrosis Amyloidosis
41
X-ray signs of ankylosing spondylitis?
Commonly normal at the time of presentation.
* Sclerosis and fusion of the sacroiliac joints
* Bony spurs from the vertebral bodies, known as syndesmophytes, which can bridge the intervertebral disc
Result is fusion, producing a “bamboo spine”
42
HLA-B27 in ankylosing spondylitis?
90% of patients are +ve
43
Treatment of ankylosing spondylitis?
* Physiotherapy, exercise, NSAIDs and anti-TNF inhibitors for more aggressive disease
* DMARDs do not have any impact on spinal disease but may be used if there is peripheral joint inflammation
* Surgery is mainly reserved for hip and knee arthritis
44
Describe psoriatic arthritis
Occurs in 30% of psoriasis patients
Arthritis is usually an ASYMMETRICAL oligoarthritis; can affect the DIP joints (only inflammatory arthritis to do this)
Patients usually have nail changes, inc. pitting and onycholysis
45
Other features of psoriatic arthritis?
Spondylitis, dactylitis and enthesitis commonly occur
46
Treatment of psoriatic arthritis?
DMARDs, usually methotrexate; anti-TNF therapy is available for those who do not respond
Joint replacement can be considered in severely affected larger joints and DIP joint fusion can occasionally help
47
What is enteropathic arthritis?
Inflammatory arthritis involving the peripheral joints and sometimes spine, occurring in patients with IBD (Crohn’s or UC)
48
Presentation of enteropathic arthritis?
Large joint ASYMMETRICAL oligoarthritis
49
Treatment of enteropathic arthritis?
Medication to manage both the IBD and arthritis
50
What is reactive arthritis?
Tends to occur after an infection, commonly genitourinary (Chlamydia, Neisseria) or GI infections (Salmonella, Campylobacter); this triggers an autoimmune arthropathy
51
Presentation of arthritis?
Large joints, e.g: the knee become inflamed 1‐3 weeks following the infection
52
What is Reiter's syndrome?
Triad of symptoms of urethritis, uveitis/conjunctivitis and arthritis
53
Treatment of Reiter's syndrome?
Most cases are self‐limiting; others have a chronic form OR frequent relapses
Treatment is aimed at the underlying infectious cause and symptomatic relief, inc. IA/IM steroid injections; occasionally DMARDs are required in chronic cases
54
Different types of connective tissue disease?
* Systemic lupus erythematosus (SLE)
* Sjogrens syndrome
* Systemic sclerosis
Mixed connective tissue disease
• Anti-phospholipid syndrome.
55
What is SLE?
Prototype connective tissue disease; it is chronic and autoimmune and inv. skin, joints kidneys, blood cells and NS (can affect almost any system)
56
Classification criteria of SLE?
≥ 4 criteria in total but at least 1 clinical and 1 laboratory
OR
Biopsy proven lupus nephritis with +ve ANA or anti-DNA
PICTURE 8 IN UNI
57
Pathogenesis of SLE?
Defective apoptosis causes increased cell death and disturbed immune tolerance
Defective clearance of apoptotic cell debris allows for antigen persistence and immune complex production
58
Occurrence of SLE?
High in Black people of the US and UK, but not in African Blacks
WOMEN (90%) are affected more, frequently starting at child-bearing age
59
Constitutional symptoms of SLE?
Fever, fatigue and weight loss
60
MSK symptoms of SLE?
Arthralgia, myalgia and inflammatory arthritis
Avascular necrosis of the femoral head may be related to steroid use
61
Mucocutaneous symptoms of SLE?
Malar rash (SPARES THE NASO-LABIAL FOLDS) and photsensitivity
Discoid lupus
Subacute cutaneous lupus
Oral/nasal ulceration
Raynaud's phenomenon
Alopecia
62
Renal symptoms of SLE?
LUPUS NEPHRITIS (must screen for this)
63
Respiratory symptoms of SLE?
```
Pleurisy
Pleural effusion
Pneumonitis
PE
Pulmonary hypertension
Interstitial lung disease (ILD)
```
64
Haematological symptoms of SLE?
Leukopenia, lymphopenia, anaemia (may be haemolytic), and thrombocytopenia
65
Neuropsychiatric symptoms of SLE?
Seizures, psychosis, headache, aseptic meningitis
66
Cardiac symptoms of SLE?
Pericarditis (most common cardiac sign), pericardial effusion, pulmonary hypertension, sterile endocarditis (Libman-Sacks endocarditis) and accelerated ischaemic heart disease
67
GI symptoms of SLE?
Uncommon but inc. autoimmune hepatitis, pancreatitis and mesenteric vasculitis
68
Ix for SLE?
FBC (for anaemia, leucopaenia and thrombocytopaenia)
```
Antibody testing:
Anti-ANA (not specific)
Anti-dsDNA (specific)
Anti-Sm (specific by low sensitivity)
Anti-Ro, anti-La and anti-RNP (non-specific)
```
Urinalysis and RENAL BIOPSY to look for lupus nephritis
Imaging for damage to other organs
69
How to monitor SLE disease activity?
Anti-dsDNA Ab varies with disease activity
C3/4 levels are low when the disease is active, esp. renal disease, as complement is consumed
Urinalysis to check for renal involvement
Manage CV risk factors
70
Management of SLE?
Skin disease and arthralgia:
Hydroxychloroquine, topical steroids and NSAIDs
Inflammatory arthritis/some organ involvement, e.g: pericardial disease - immunosuppression (azathioprine) with corticosteroids at moderate doses and for short periods
Severe organ disease, e.g: lupus nephritis or CNS lupus then treatment tend to involve IV steroids and cyclophosphamide
In unresponsive cases, IV Ig and Rituximab may be used
71
Monitoring of SLE?
Check anti-dsDNA antibodies and complement levels
Check urinalysis for blood or protein (may indicate glomerulonephritis)
Evaluate and manage CV risk factors, like BP and cholesterol (one of the major causes of mortality)
72
What is Sjogren's syndrome?
Autoimmune condition characterised by lymphocytic infiltrates in EXOCRINE organs
It can be a primary conditions OR can occur secondary to RA or SLE, etc
73
Symptoms of Sjogren's?
Typically, DRY EYES (keratoconjunctivitis sicca) AND MOUTH (xerostomia); due to dry mouth, dental problems can occur (e.g: high risk of caries)
```
Other symptoms:
• Arthralgia
• Fatigue
• Vaginal dryness
• Parotid gland swelling
```
74
Complications of Sjogren's?
Peripheral neuropathy
ILD
Increased risk of lymphoma
75
Diagnosis of Sjogren's?
Confirmation of ocular dryness *using Schirmer's test*
+ve anti-Ro and anti-La antibodies
Typical features of a lip? gland biopsy
76
Classification criteria for diagnosis of Sjogren's?
Diagnosis requires 4 of:
1. Ocular symptoms - dry eyes for > 3 months, foreign-body sensation, use of tear substitutes > 3 times daily
2. Oral symptoms - feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
3. Ocular signs - Schirmer test performed without anesthesia (< 5 mm in 5 min), +ve vital dye staining results
4. Oral signs - abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings
5. Positive minor salivary gland biopsy findings
6. Positive anti–Ro or La antibodies
77
Management of Sjogren's?
Symptomatic management:
• Lubricating eyedrops
* Saliva replacement products and regular dental care
* Pilocarpine can stimulate saliva production but side effects inc. flushing
* Hydroxychloroquine can help with arthralgia and fatigue
* Other immunosuppression would only be used for organ inv. like ILD
78
What is systemic sclerosis?
Systemic CTD where excess collagen deposition leads to skin and internal organ changes
79
Presentation of systemic sclerosis?
CREST:
Calcinosis (subcutaneous deposits of Ca may be seen on digits)
Raynaud's phenomenon (tends to be the presenting feature)
Esophageal dysmotility
Sclerodatyly (skin becomes thick, tight and shiny)
Telangiectasia
80
Major and minor cutaneous features of systemic sclerosis?
Major:
Centrally located skin sclerosis affecting the arms, face and/or neck
Minor:
Sclerodactyly and atrophy o the fingertips and bilateral lung fibrosis
Diagnosis is made when the patient has 1 major 2 minor features
81
Facial signs of systemic sclerosis?
Pinching of the skin of the nose ("BEAKING") and tightening of the skin around the mouth
Telangiectasia can also be seen
82
Organ involvement in systemic sclerosis?
Pulmonary hypertension, fibrosis and accelerated hypertension leading to renal crisis
Gut inv. can lead to dyaphagia, malabsorption and small bowel bacterial overgrowth
Inflammatory arthritis and myositis may occur
83
2 classifications of systemic sclerosis?
Limited - skin inv. tends to be confined tot he face, hands, forearms and feet; organ inv. occur latera
Diffuse - skin changes occur more rapidly and can inv. the trunk; there is early signification organ inv.
84
Which antibodies are assoc. with which types of systemic sclerosis?
Limited: anti-centromere antibody
Diffuse: anti-Scl-70 antibody
85
Ix for systemic sclerosis?
Anti-centromere Ab and anti-Scl-70 Ab
86
Monitoring of systemic sclerosis?
Organ screening regularly occurs, inc. PFTs, echo and monitoring of renal function
87
Management of systemic sclerosis?
Raynaud's/digital ulcers: CCBs, iloprost, bosentan
Renal involvement: ACEIs
GI involvement: PPIs for reflux
ILD: immunosuppression (usually cyclophosphamide)
88
What is Mixed Connective Tissue Disease (MCTD)?
```
Defined conditions which features symptoms also seen in other CTDs, inc. :
• Raynaud's phenomenon
• Arthralgia/arthritis
• Myositis
• Sclerodactyly
• Pulmonary hypertension
• ILD
```
89
Antibodies assoc. with MCTD?
Anti-RNP antibodies
90
Monitoring of MCTD?
Due to the risk of pulmonary hypertension, regular echocardiograms (anually) are done
Screen for ILD with PFTs
91
Management of MCTD?
CCBs may be helpful for Raynaud's
Immunosuprpession if there is significant muscle/lung disease
92
What is anti-phospholipid syndrome (APS)?
Autoimmune, hypercoagulable state manifesting as recurrent venous/arterial thrombosis and/or fetal loss
It can be assoc. with other disease, e.g: SLE or other autoimmune disorders, OR it can occur by itself
93
Presentation of APS?
Increased frequency of stroke/MI (esp. in younger individuals)
Libman-Sacks enodcarditis (sterile); this can also lead to embolisation and stroke
Recurrent PE or thrombosis can lead to pulmonary hypertension
Catastrophic APS is rare and often a fatal manifestation, where there are multi-organ infarction over a period of days to week s
Late spontaneous fetal loss is common and recurrent early fetal loss can occur
Some patient have a migraine
Livedo reticularis (cutaneous finding)
94
Ix for APS?
May find thrombocytopaenia
Prolongation of APTT
Lupus anticoagulant, anti-cardiolipin antibodies and anti-β2 glycoprotein may be +ve
95
Management of APS?
Anticoagulation for those who have had an episode of thrombosis
LMWH is used during pregnancy for those who have had recurrent pregnancy loss (warfarin is teratogenic)
Patients who are found to have +ve antibodies but who have never had had an episode of thrombosis do not require anticoagulation
96
What is gout?
A CRYSTAL ARHTROPATHY caused by urate crystal deposition within a joint; it tends to occur due to hyperuricaemia
97
Describe why hyperuricaemia can occur
Uric acid is the final breakdown product of purines (adenine and guanine) in DNA metabolism
It can occur due to renal underexcretion (diuretics or renal failure) or due to excessive intake of alcohol, red meat or seafood
98
How can gout be triggered?
Uric acid crystals precipitating into joints can be triggered by dehydration, trauma or surgery
99
Joints affected by gout?
Any joint can be affected but it tends to be the 1st MTP joint (AKA Podagra)
Ankle and knee are also commonly affected
100
Presentation of gout?
Intensely painful, red, hot and swollen joint; symptoms tend to last for 7-10 days, if untreated, before spontaneously resolving
(MIMICS septic arthritis)
Gouty tophi can be seen (painless, white accumulation of uric acid which can occur in soft tissue and occasionally erupt through skin)
101
Consequences of chronic gout?
Destructive, erosive arthritis
102
How can a definitive diagnosis of gout be made?
Joint synovial fluid aspirate with polarised microscopy shows NEEDLE-shaped uric acid crystals which display -ve birefringence (change from yellow to blue when lined across the direction of polarisation)
Fluid is also analysed with gram stain and culture to exclude infection
103
Treatment of acute gout?
NSAIDs, corticosteroids and opioid analgesics
| Colchicine for those who cannot tolerate NSAIDs
104
Prophylaxis of gout?
For recurrent attacks, OR joint destruction OR tophy:
Allopurinol/other urate-lowering therapies can prevent attack
They should not be started until as acute attack has settled as they can potentiate a further flare
105
What is pseudogout?
Crystal arthropathy causing an acute arthritis but, in contrast to gout, it is caused by calcium pyrophosphate crystals
106
Diagnosis of pseudogout?
Joint aspirate shows +vely birefringent crystals
107
What is chrondrocalcinosis?
Calcium pyrophosphate deposition occurs in cartilage and other soft tissues in the absence of acute inflammation
Both come under the umbrella of Calcium Pyrophosphate Deposition disease (CPPD), of which the exact cause is unknown
108
Joints affected by CPPD?
Tends to affect the knee, wrist and ankle
109
Which conditions can coexist with CPPD?
Hyperparathyroidism, hypothyroidism
Renal osteodystrophy
Haemochromatosis
Wilson's disease
Can also occur in some OA cases; however, chronic CPPD can also cause osteoarthritic change
110
Treatment of acute pseudogout?
NSAIDs, corticosteroids (systemic and intra-articular) and occasionally colchicine
No medications can be used as prophylaxis
111
What is polymyalgia rheumatica?
Fairly common chronic inflammatory condition of unknown aetiology that affects elderly individuals (rare <50 years)
112
Symptoms of polymyalgia rheumatica?
PROXIMAL myalgia of the hip and shoulder girdles with accompanying morning stiffness of > 1 hour
Symptoms tend to IMPROVE as the day goes on and with MOVEMENT
113
Assoc. conditions with polymyalgia rheumatica?
~15% of patient develop giant cell arteritis (GCA)
40-50% of GCA patients have assoc. PMR
114
Diagnosis of PMR?
No specific diagnostic test for PMR but it tends to be assoc. with a raised CRP and PV/ESR
Symptoms respond dramatically to low-dose steroids (prednisolone 15 mg daily) and this is used as a diagnostic tool
115
Treatment of PMR?
Prednisolone 15mg daily; dose is gradually reduced over 18 months
After this, conditions tends to have resolved
116
What is Giant Cell Arteritis (GCA)?
Most common form of systemic vasculitis in adults; it has an unknown aetiology and occurs in ELDERLY patients
117
Histopathology of GCA?
Transmural inflammation of the intima, media and adventitia of affected arteries
Patchy inflammation by lymphocytes, macrophages and multinucleated giant cells
Vessel wall thickening can result in arterial lumen narrowing, resulting in distal ischaemia
118
Presentation of GCA?
Visual disturbances, headache, jaw claudication and scalp tenderness (with hair combing)
Constitutional symptoms, such as fatigue, malaise and fever may also be present
119
When should GCA be considered?
Always consider it in the DD of a new-onset headache in patients >50 years, with an elevated ESR/CRP/PV
120
Visual symptoms of GCA?
Visual loss is one of the most significant causes of morbidity, e.g: unilateral vision blurring/loss, often painless, and sometimes diplopia (double vision)
Permanent visual impairment can occur
121
Describe the headache of GCA
Headache is usually continuous and located in the temporal or occipital areas
Focal tenderness on direct palpation is typically present, with a thickened, prominent and tender to touch temporal artery
122
Describe jaw claudication in GCA
Fatigue/discomfort of jaw muscles during chewing of firm foods
It is caused by ischaemia of the maxillary artery
123
Diagnosis of GCA?
Raised inflammatory markers
Temporal artery biopsy (most definitive test), which has 100% specificity for GCA but low sensitivity
124
Why does a temporal artery biopsy have low specificity for GCA?
Skip lesions mean that some segments of the artery are normal
125
Typical temporal artery biopsy findings in GCA?
Mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells
126
Treatment of GCA?
NO VISUAL IMPAIRMENT:
Prednisolone 40 mg gradually reduced over 18 months to 2 years
WITH VISUAL SYMPTOMS:
Prednisolone 60 mg gradually reduced over 18 months to 2 years
Treatment is started as soon as the diagnosis is suspected and should not be delayed for the biopsy results
127
What are polymyositis and dermatomyositis?
Polymyositis - idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness
Dermatomyositis - clinically similar but also has typical cutaneous manifestations
128
Occurrence of dermato/polymyositis?
More common in women than men (2:1) and usually affects adults >20 years, esp. those 45-50 years
129
Pathogenesis of polymyositis?
T-cell mediated cytotoxic process directed against unidentified muscle antigens (nuclear and cytoplasmic auto-antigens)
CD8+ T cells, along with macrophages, initially surround healthy non-necrotic muscle fibers and eventually invade and destroy them
130
Antibodies found in polymyositis?
ANA and anti-RNP antibodies (shared with other autoimmune conditions)
Anti-Jo-1 and anti-SRP antibodies are unique to myositis
131
Presentation of polymyositis?
SYMMETRICAL, proximal muscle WEAKNESS in the upper and lower extremities that is of insidious onset and tends to be noticed with, e.g: climbing stairs
Some patients have myalgia
Dysphagia can occur secondary to oropharyngeal and esophageal involvement occurs in about 1/3rd of patients with polymyositis and is a poor prognostic sign
ILD can occur, esp. in those +ve for anti-Jo-1 antibody
132
Ix for polymyositis?
Raised inflammatory markers
Serum creatine kinase is usually raised
ANA, anti-Jo-1 and anti-SRP +ve
MRI for extent of muscle inv.
EMG findings are abnormal in most patients
MUSCLE BIOPSY
133
Muscle biopsy findings in polymyositis?
Muscle fibres in varying stages of inflammation, necrosis and regeneration
There is a chronic inflammatory infiltrate of T cells, esp. CD8+
134
Management of polymyositis?
Prednisolone (initially 40 mg)
COMBINED WITH
Immunosuppressive drugs, such as methotrexate or azathioprine
135
Cutaneous features of dermatomyositis?
V-shaped rash over chest
Gottron's papules, e.g; over fingers
Heliotrope rash
136
Malignant assoc. with dermatomyositis?
```
Common cancers inc:
Breast
Ovarian
Lung
Colon
Oesophagus
Bladder
```
Screen for malignancy at the time of diagnosis
137
What is fibromyalgia?
Unexplained conditions causing widespread muscle pain and fatigue, commonly in young and middle-aged women
Can occur as a primary condition but can also be seen in patients with RA or SLE
138
Potential causes of fibromyalgia?
Disorder of central pain processing or a syndrome of central sensitivity; patients tend to have a lower threshold for pain/other stimuli
139
Signs and symptoms of fibromyalgia?
Persistent (≥ 3 months) widespread pain on both sides of the body, above and below the waist
Fatigue (disrupted and unrefreshing sleep)
Cognitive difficulties
Multiple other unexplained symptoms, anxiety and/or depression, and functional impairment ADLs
140
Conditions assoc. with fibromyalgia?
Depression, IBS and migraines
141
Ix for fibromyalgia?
CLINICAL DIAGNOSIS
Rule out other conditions, e.g: hypothyroidism, RA, SLE, PMR, etc
142
Management of fibromyalgia?
Self-management techniques and education
Graded exercise
Atypical analgesia including tricyclics (e.g. amitriptyline), gabapentin and pregabalin may be of benefit
Cognitive behavioural therapy may help some patients
143
What is vasculitis?
Inflammation of blood vessels that results in thickening, stenosis and occlusion with ischaemia
144
Classifications of vasculitis?
ADD CLASSIFICATION PICTURE
145
Describe large vessel vasculitis?
Primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches
146
Types of large vessel vasculitis?
Temporal (giant cell) arteritis - tends to be in >50 years and is far more common in east Asian countries
Takayasu arteritis - tends to be in young patients, rarely above 50 years
147
Features of large vessel vasculitis?
Non-specific, e.g: low-grade fever, malaise, night sweats, weight loss, arthralgia and fatigue
Following this, claudication symptoms occur in upper and lower limbs
148
Consequences of untreated large vessel vasculitis?
Vascular stenosis and aneurysms can occur, causing pulses and bruits
149
Ix for large vessel vasculitis?
ESR/PV/CRP raised
MR angiography shows thickened vessel walls and stenosis
PET CT shows increased metabolic activity in the large vessels
150
Treatment of large vessel vasculitis?
40-60 mg of prednisolone gradually reduced
Steroid-sparing agents, e.g: methotrexate and azathioprine
151
How to differentiate between different types of small to medium vessel vasculitis?
ADD VASCULITIS PICTURE
152
Similar presenting features of small to medium vessel vasculitis?
* Fever and weight loss
* Raised, non blanching purpuric rash (vasculitic rash)
* Arthralgia/arthritis
* Mononeuritis multiplex
* Glomerulonephritis
* Lung opacities on X-ray
153
4 types of ANCA assoc. vasculitis?
Granulomatosis with polyangiitis (GPA), AKA Wegener's
Microscopic polyangiitis (MPA)
Renal limited vasculitis (RLV)
Churg-Strauss syndrome (CSS)
154
Describe GPA
Granulomatous inflammation inc. the resp tract and necrotising vasulitis affecting small to medium sized vessels, e.g: capillaries, venules, arterioles and arteries
Necrotising glomerulonephritis is common
155
Describe MPA
Necrotising vasculitis, with few/no immune deposits, affecting small vessel, i.e: capillaries, venules or arterioles
Necrotising arteritis involving small and medium sized arteries may be present; necrotising glomerulonephritis is very common
Pulmonary capillaritis often occurs
156
Describe RLV
Pauci-immune necrotising glomerulonephritis with no apparent extra-renal features at presentation
157
Describe CSS
EOSINOPHIL-rich granulomatous inflammation, inv. the resp tract, necrotising vasculitis affecting the small to medium-sized vessel and assoc. with asthma and eosinophilia
158
Symptoms of GPA?
ENT symptoms are common, inc. nosebleeds, deafness, recurrent sinusitis and nasal crusting (over time, there may be collapse of nasal cartilage)
Resp symptoms such as haemoptysis and cavitating lesions on CXR are seen
159
Antibodies assoc. with GPA?
cANCA and anti-proteinase 3 (anti-PR3) antibodies
160
Characteristics of EGPA?
Late onset asthma
Rhinitis
Raised eosinophil count
Neurological symptoms, such as mononeuritis multiplex are common
161
Most important complication of MPA?
Glomerulonephritis is common
162
How to differentiate between ANCA-assoc. vasculitides?
ADD ANCA TABLE
163
Ix for small to medium vessel vasculitides?
ESR/PV/CRP raised
Anaemia of chronic disease
U&Es for renal inv.
cANCA antibodies
Urinalysis
CXR
Biopsy of affected area, e.g: renal, skin, etc
164
Management of ANCA-assoc vasculitis?
IV steroids and cyclophosphamide
165
What is Henoch-Schonlein purpura?
Acute IgA-mediated disorder characterized by a generalised vasculitis inv. the small vessels of the skin, GI tract, kidneys, joints and, rarely, lungs and CNS
166
Occurrence of Henoch-Schonlein purpura?
Commonly affects children
Tends to be a history of upper resp tract infection (a few weeks prior to symptoms)
167
Symptoms of Henoch-Schonlein purpura?
Purpuric rash over buttocks and lower limbs, abdominal pain and vomiting and joint pain
168
Treatment of Henoch-Schonelin purpura?
Self-limiting conditions not requiring specific treatment
Usually settles over a course of weeks to months
