Week 6: Rheumatology (1) (conditions) Flashcards
(79 cards)
1
Q
pathophysiology of rheumatoid arthritis
A
Citrullination of self-antigens which are then recognised by T and B cells – producing autoantibodies (RF and anti-CCP)
- Stimulates macrophages and fibroblasts release TNFalpha
- Inflammatory cascade leads to proliferation of synoviocytes (bogg joint swelling), these grow over the cartilage and lead to restriction of nutrients and cartilage damage
- Activate macrophages stimulate osteoclast differentiation contributing to bone damage
2
Q
risk factors of RA
A
- Female 3:1
- 30-50 years old
3
Q
presentation of RA
A
- Progressive, peripheral and symmetrical polyarthritis
- Commonly affected joints: MCP/ PIP/ MTPs (typically spares DIP (OA)). May effect any joint inc hip/knees/shoulders/c-spine
- Hx .6 weeks
- Morning stiffness >30 min duration
- Fatigue/ malaise
4
Q
RA examination
A
- Soft tissue swelling and tenderness first
- Ulnar deviation/palmar subluxation of MCP
- Swan-neck and boutonniere deformity to digits
- Rheumatoid nodules- most common on elbows
- Median nerve- carpal tunnel association
5
Q
RA investigations
A
- Auto antibodies: RF and anti-CCP
- FBC- normocytic anaemia (chronic disease)
- WCC (septic arthritis)
- Inflammatory markers (CRP and ESR)- elevates
- X-ray changes apparent in established disease- USSS/MRI more sensitive in early disease
6
Q
treatment of RA
A
- Initially DMARD monotherapy- Methotrexate
- Consider combination DMARDs (leflunomide, hydroxychloroquine, sulfasalazine)
- Steroids (acutely)= prednisolone
- PO/IM or intra-articular
- Symptoms control with NSAID (PPI cover)
- If disease still severe add biologic- anti-TNFs – Infliximab/ Etanercepts
-
Non-drug
- OT/PT
- podiatry
- psychological
7
Q
x-ray features of RA
A
8
Q
extra-articular presentations of RA
A
9
Q
giant cell arteritis
A
- Chronic vasculitis of large- and medium-sized vessels that occurs among individuals over 50 yr. of age
- Often referred to as temporal arteritis (TA)
- Most commonly causes inflammation of arteries originating from the arch of the aorta
- Occlusive arteritis can result in anterior ischemic optic neuropathy (AION) and acute visual loss
- Visual symptoms are an ophthalmic emergency
- Inflammation of arteries supplying the muscles of mastication results in jaw claudication and tongue discomfort
- GCA may present as CVA
10
Q
risk factors for GCA
A
- Rare in patients <50 yr old (mean age 72)
- Increased prevalence in Northern latitude
- 2 to 4 times more common in women
- Rare in African American patients, common in Whites
- There is a strong association with polymyalgia rheumatica (PMR) ∼50%
- Genetic predisposition: HLA-DR4
11
Q
presentation of GCA
A
- Headache
- Localised, unilateral, boring or lancinating in quality ove the temple
- Tongue and jaw claudication upon mastication
- Constitutional symptoms common
- Visual findings may develop weeks to months after onset of other symptoms
- Fugax
- Blindness
- Dipoplia
- Blurring
- Scalp tenderness, esp over temporal after
12
Q
diagnosis of GCA
A
Presence of any 2 or more of the following in patients >50 years with:
- Raised ESR, CRP or PV
- New onset of localized headache
- Tenderness or decreased pulsation of temporal artery
- New visual symptoms
- 25-50% of patients who present with acute loss of vision in one eye who go untreated will develop bilateral blindness
- Biopsy revealing necrotizing arteritis
13
Q
management of GCA
A
DO NOT DELAY INITATION OF STEROID THERAPY IF VISUAL LOSS- do not delay for biopsy if strong clinical suspicion
- Prednisolone 60–100 mg PO per day for at least 2 weeks before considering tapering down slowly
- For acute onset visual symptoms, consider 1g methylprednisolone IV pulse therapy for the 1–3 days
- Low-dose aspirin therapy to reduce thrombotic risks
14
Q
polymyalgia rheumatica
A
- Characterized by pain and stiffness of the shoulder, hip girdles, and neck.
- Patients may use the term stiffness and pain interchangeably.
- Primarily impacts the elderly, associated with morning stiffness and elevated inflammatory markers.
15
Q
presentation of polymyalgia rheumatica
A
16
Q
risk factors for polymyalgia rheumatica
A
- Incidence increases with age.
- Average age of onset ~70 years
- Rare in people <50 years of age
- Peak incidence is between ages 70 and 80
- Is associated with GCA
17
Q
history of polymyalgia rheumatica
A
- Suspect PMR in elderly patients with new sudden onset of proximal limb pain and stiffness (neck, shoulders, hips).
- Difficulty rising from chair or combing hair (proximal muscle involvement)
- Night time pain
- Systemic symptoms in ~25% (fatigue, weight loss, low-grade fever)
18
Q
physical exam PR
A
- Decreased range of motion (ROM) of shoulders, neck, and hips
- Muscle strength is usually normal—may be limited by pain and/or stiffness.
- Muscle tenderness
19
Q
diagnosis of PR
A
- Decreased range of motion (ROM) of shoulders, neck, and hips
- Muscle strength is usually normal—may be limited by pain and/or stiffness.
- Muscle tenderness
20
Q
management of PR
A
- Dramatic diagnostic response within 5 days of starting prednisolone (15mg daily- then taper slowly)
- Rapid taper is associated with symptoms relapse
- Methotrexate can be steroid-sparing in relapsing patients
21
Q
Spondyloarthropathies
A
These are a group of conditions that affect the spine and peripheral joints and are associated with the presence of HLA-B27.
22
Q
examples of spondyloarthropathies
A
- Ankylosing spondylitis (most common)
- Enteropathic arthritis
- Psoriatic arthritis
- Reactive arthritis
23
Q
common clinical features of spondyloarthropathies
A
- Sacroiliac/axial disease (back/buttock pain)
- Inflammatory arthropathy of peripheral joints
- Enthesitis (inflammation at tendon insertions e.g. tennis elbow, achilles))
- Extra-articular features (skin/gut/eye)
24
Q
psoriatic arthritis
A
Psoriatic arthritis is a type of arthritis that affects some people with the skin condition psoriasis.
25
risk factor for psoriatic arthritis
10% of patients with psoriasis (Male=Female)
26
typical exam findings of psoriatic arthritis
* Oligo-arthritis with dactylitis or "sausage" digit.
* Can be symmetrical arthritis like RA, or mono-arthritis.
* Severe deformities (arthritis mutilans in 5%).
27
investigation of psoriatic arthritis
CRP often raised. Central joint erosions seen early on u/sd or MRI leading to ‘pencil in cup’ x-ray appearance.
28
management of psoriatic arthritis
NSAIDs, DMARDs, anti-TNF, IL-17 inhibitors, IL12/23 inhibitors.
29
anklosing spondyylitis
Ankylosing spondylitis is **an inflammatory disease** that, over time, can cause some of the bones in the spine (vertebrae) to fuse. This fusing makes the spine less flexible and can result in a hunched posture. If ribs are affected, it can be difficult to breathe deeply.
30
RF for ankylosing spondylitits
Usually in young men (teens – mid-thirties)
31
presentation of ankylosing spondylitis
with bilat buttock pain, chest wall and thoracic pain
32
examination of ankylosing sponylitis
* often normal.
* Later: loss of lumbar lordosis, exaggerated thoracic kyphosis,
* Schober’s test (mark skin 10cm above and 5cm below PSIS, bend forward with straight legs, distance increase to \>20cm is normal), reduced chest expansion
33
investigations for ankylosing spondylitits
* CRP may be raised but often normal.
* MRI spine and SI joints (more sensitive than X-ray).
*
34
management of AS
NSAIDs and physio, TNF inhibitors, IL-17 inhibitors.
35
**Reactive arthritis**
Sterile synovitis developing after a distant infection either post dysentery (Salmonella / Shigella / Campylobacter) or following urethritis/cervicitis (Chlamydia trachomatis)
36
presentation Reactive arthritis
* few days – 2 weeks post infection, acute asymmetrical lower limb arthritis develops.
* Other features: skin (circinate balanitis, keratoderma blennorrhagica), eye (conjunctivitis, uveitis), enthesitis.
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investigations for reactive arthritis
serology/microbiology, inflammatory markers raised, may need joint aspirate to rule out septic/crystal arthritis
38
treatment for reactive arthritis
* treat infection (this may not improve arthritis). NSAIDs and joint injections. Most will resolve within 2 years. Those that do not (esp if HLA-B27+) may need DMARDs.
39
enteropathic arthritis
**a form of chronic, inflammatory arthritis associated with the occurrence of an inflammatory bowel disease** (IBD), the two best-known types of which are ulcerative colitis and Crohn's disease.
40
rf for enteric arthritis
10 - 20% of those with IBD develop arthropathy. Of these 2/3 = peripheral arthritis and 1/3 develop axial disease.
41
enteropathic- how many types of peripheral diseas
* Type 1 = oligoarticular, asymmetric and has a correlation with IBD flares
* Type 2 = polyarticular symmetrical and less correlation with IBD flares
42
management of enterpathic arthritis
* Remember NSAIDs may flare IBD.
* Consider DMARDs.
* TNF inhibitors will treat both the bowel disease and the arthritis.
43
systemic lupus erythematous
* autoimmune disease
* inadequate T cell suppressor activity with increased B cell activity
* most patients have antibodies to certain cell nucleus components
* complex multisystem disease with variable presentations
* extremely variable presentations, disease courses, and prognosis
* characterized by remissions and flares.
* can be familial
44
risk factors for SLE
* 9:1 women (oestrogen)
* Early adulthood
* Black people have worse prognosis
* High CVD risk
45
common presentation of SLE
* **S erositis -** Pleurisy, pericarditis
* **O ral ulcers** - usually painless; palate is most specific
* **A rthritis** – small joints nonerosive
* **P hotosensitivity** – or malar or discoid rash
* **B lood disorders** – low WCC, lymphopenia, thrombocytopenia, hemolytic anemia
* **R enal involvement -**glomerulonephritis
* **A utoantibodies** (ANA positive in \>90% cases)
* **I mmunologic tests** e.g. low complements
* **N eurologic disorder -** Seizures or psychosis
46
rinvestigations for SLE
* Raised ESR or plasma viscosity
* Anaemia and leukopenia common
* 95% are antinuclear antibody positive
* Anti-Ro and Anti-La are common
* Anti-dsDNA titre increase with disease activity
* Antiphospholipid antibodies increase risk of pregnancy loss and thrombosis
* C3 and C4 fall with disease activity
* Urinalysis for detecting renal disease
* Skin biopsy
* Renal biopsy
47
treatment for SLE
**First line treatments** are:
* NSAIDs
* Steroids (prednisolone)
* ***Hydroxychloroquine*** (first line for mild SLE)
* Suncream and sun avoidance for the photosensitive malar rash
**Other commonly used immunosuppressants** in resistant or more severe lupus:
* Methotrexate
* Mycophenolate mofetil
48
raynauds phenomenon (disease)
This is a condition due to **vasospasm** of the digits- primary cause. It is painful and is characterized by a typical sequence of colour changes in response to a cold stimulus. It is often also precipitated by stress.
* white- inadequate blood flow
* blue -venous stasis
* red -re-warming hyperaemia.
49
**Raynauds syndrome**
* when cause is idiopathic
* young women and may improve when they get older
* may be familial
* avoid smoking
50
**Diseases associated with Raynaud’s phenomenon (secondary)**
* RP symptoms developing over age 30 should alert you to underlying disease
* Abnormal nail-fold capillaries or puffy fingers or photosensitive rash suggest rheumatic cause
* Associated with
* Scleroderma
* SLE
* Dermatomyositis and polymyositis
* Sjogrens syndrome
* Physical causes
* Uses of heavy vibrating tools
* Sticky blood e.g. cryoglobulinemia
* Drug induced: b blockers
51
**Treatment of raynauds phenomenon**
* Keep warm and don’t smoke
* Nifedipine- CCB first line
* Sildenafil -phosphodiesterase-5 inhibitors (VIAGRA)
52
complication of raynauds
**Complications:** digital ulcers, severe digital ischaemia and infection
53
vasculitis
* Inflammatory blood vessel disorder
* Clinical features result from the damage of blood vessel walls with subsequent thrombosis, ischemia, bleeding, and/or aneurysm formation.
* Vasculitis is a large, heterogeneous group of diseases classified by the predominant size, type, and location of involved blood vessels
54
history for vasculitis
* Consider age, gender, and ethnicity.
* Comprehensive drug history
* Family history of vasculitis
* Constitutional symptoms: fever, weight loss, malaise, fatigue, diminished appetite, sweats
* Then use the “Glove and sweater” approach
55
glove and sweater approach
* (Series of questions from hands to head and down to trunk)
* Ask about Raynaud's
* Musculoskeletal: arthralgia, myalgia, proximal muscle weakness
* CNS/PNS: headaches, visual loss, tinnitus, stroke, seizure, encephalopathy
* Nose bleeds, crusts, ulcers and oral ulcers
* Heart/lung: pericarditis, cough, chest pain, hemoptysis, dyspnea
* GI: abdominal pain
* Renal: Haematuria
* Limbs: Neuropathy, digital ulcers/ischaemia
56
physical exam vascultitis
* Vital signs: blood pressure (hypertension) and pulse (regularity and rate)
* Skin: palpable purpura, livedo reticularis, nodules, digital ulcers, gangrene, nail bed capillary changes
* Neurologic: cranial nerve exam, sensorimotor exam
* Ocular exam: visual fields, scleritis, uveitis episcleritis
* Cardiopulmonary exam: Crackles, pleural rubs murmurs, arrhythmias
* Abdominal exam: tenderness, organomegaly
57
vasculitis mimics
Vasculitis occurs as a primary disorder or secondary to infections, drugs, malignancy, or connective tissue diseases.
Secondary vasculitis is much more common than primary.
58
diagnostic tests for vasculitis
* Initial tests exclude alternate diagnoses and guide therapy FBC, U&Es, LFTs, CRP, PV, ESR,
* Specific serology: ANA, ANCA, RF
* Complement levels C3, C4 Hepatitis screen for B and C, HIV Cryoglobulins
* Serum and urine protein electrophoresis
* Miscellaneous: CK, Blood cultures, ECG
* CXR, CT scan, MRI, arteriography and CT-PET may be required to delineate extent of organ involvement.
59
treatment of vasculitis
* General Measures: Rule out infection, stop offending drug in secondary causes
* 1st line: Corticosteroids
* 2nd Line: Cytotoxic medications, immunomodulatory, or biologic agents (e.g., cyclophosphamide methotrexate, azathioprine, leflunomide mycophenolate mofetil, rituximab, IVIG)
60
small vessel vasculitis
* Microscopic polyangiitis (MPA)
* Granulomatosis with polyangiitis (Wegener's granulomatosis)
* Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
61
**Medium-vessel vasculitis**
* Polyarteritis nodosa (PAN)
* Kawasaki disease (KD)
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**Large-vessel vasculitis**
* Takayasu arteritis (TAK)
* Giant cell arteritis (GCA)
63
**Systemic sclerosis (SSc)**
* Multisystem autoimmune disease
* Previously known as *scleroderma*
* **Pathophysiology:** increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis
* 2 subsets
* Limited SSc
* Diffuse SSc3
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limited scleroderma
* Diffuse skin tightness below knees and below elbows
* Usually years of raynauds before scleroderma
* Development of Pulmonary arterial (PA) hypertension after a mean of 10 years of symptoms
* CREST syndrome
Calcinosis Cutis
Raynaud’s phenomenon
* Ulcers
* White/blue skin
Oesophageal dysmotility
Sclerodactyly
* Very tight skin
Telangiectasia
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**Diffuse scleroderma**
* Less common
* High risk of mortality
* Sudden onset of skin involvement and proximal to the elbow sand knees (can effect anywhere)
66
investigations for scleroderma
**Investigations**
* Inflammatory markers usually normal
* X-ray hands- calcinosis
* CXR, HRCT, PFT- pulmonary disease
* ECG and echo- PA hypertension, HF, myocarditis and arrhythmias
* Antibodies
* Positive ANA in 90% of pts
* Anti-centromere antibody strongly associated with limited SSc
* Scl-70 (topoisomerase) and anti RNA polymerase III antibodies strongly associated with diffuse SSc
67
scleroderma treatment
**Treatment**
* No cure
* Psychological support
* Calcium antagonist /sildenafil/iloprost infusion for Raynaud’s
* Methotrexate and mycophenolate mofetil may reduce skin thickening
* ACEi prevent hypertensive crisis and reduce mortality from renal failures
* Short courses of prednisolone for flares
* PPI for GI symptoms
68
**Sjogrens syndrome**
* Chronic autoimmune inflammatory disorder
* Immune system e.g. lymphocytes target glands that make tears and saliva (maybe triggered by particular virus or bacteria)
* Characterised by diminished lacrimal and salivary gland secretion
* Primary form- not associated with other diseases
* Secondary form- associated with another underlying rheumatic disease
* 80% female
69
sjogrens presentation
**(MADFRED!)**
Most pts present with **sicca symptoms** – xerophthalmia (dry eyes), xerostomia (dry mouth) and fatigue
* Myalgia
* Arthralgia
* Dry mouth
* Fatigue
* Raynauds phenomenon
* Enlarged parotids
* Dry eyes
70
investigations for sjogrens
* Anti RO and anti La antibodies (90%)
* RF and anti ds-DNA antibodies
* Schirmer’s test- measures tear volume
* Salivary gland biopsy may be needed
71
treatment for sjogrens
* Symptomatic treatment
* Avoid dry or smoky atmosphere
* Dry eyes- artificial tears
* Dry mouth- artificial saliva, sugar free gum, pastilles
* Skin emollients, vaginal lubricants
* Immunosuppressants/steroids rarely needed
72
**Gout**
* Inflammatory arthritis related to a hyperuricemia.
* Acute gout can affect ≥1 joint but the 1st metatarsophalangeal joint is most commonly involved at presentation (podagra).
73
pathophysiology of gout
* Deposition of monosodium urate (MSU) crystals that accumulate in joints and soft tissues, result in acute and chronic arthritis, soft-tissue masses called tophi, urate nephropathy, and uric acid nephrolithiasis
74
prognosis of gout
* After an initial flare, a second flare occurs in ~60% of patients within 1 year and 78% within 2 years of the initial attack.
75
management of gout
* Management involves treating acute attacks and preventing recurrent disease by long-term reduction of SUA levels through medication and lifestyle adjustments.
* Gout is associated with a high risk of CVD.
76
investigations for gout
* Joint aspiration (MSU crystals- negatively bifringement)
* Blood tests for uric acid
* X-ray
* Urate crystals are negatively birefringent through polarised light (microscope)
77
risk factors
* Non modifiable: \>40 and male
* Modifiable
* Increased purine (meats and seafood)
* Alcohol intake (esp beer)
* High fructose intake
* Obesity
* CHF
* CAD
* Dyslipidaemia
* Renal disease
* Organ transplant
* Hypertension
* Smoking DM
* Urate-elevating medications e.g. diuretics
78
treatment of acute gout
* General prevention: lose weight, exercise, reduced purine in diet, reduce alcohol consumption, smoking cessation, avoid dehydration
**During the acute flare:**
* NSAIDs (e.g. ibuprofen) are first-line
* Colchicine second-line
* ***Colchicine*** is used in patients that are inappropriate for NSAIDs, such as those with renal impairment or significant heart disease.
* diarrhoea common side effect
* Steroids can be considered third-line
**Prophylaxis**
***Allopurinol*** is a ***xanthine oxidase inhibitor*** used for the prophylaxis of gout. It reduces the uric acid level.
***Lifestyle changes*** can reduce the risk of developing gout. This involves losing weight, staying hydrated and minimising the consumption of alcohol and purine-based food (such as meat and seafood).
79
treatment of chronic gout
* **Chronic cases**: commence urate lowering therapy (ULT) after acute attack
* **E**.g. allopurinal and febuxostat (xanthine oxidase inhibitors and reduce urate formation)
* Aim to reduce SUA to \<360 micromol/L
