Rheumatology II Flashcards

1
Q
A
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1
Q

In which of the following conditions can tendinitis/tenosynovitis present without being swollen and tender?

Systemic sclerosis
Gout
Rheumatoid arthritis
Disseminated gonococcal infection
Reactive arthritis

A

In which of the following conditions can tendinitis/tenosynovitis present without being swollen and tender?

Systemic sclerosis
Gout
Rheumatoid arthritis
Disseminated gonococcal infection
Reactive arthritis

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2
Q

Describe how you manage fibromyalgia [3]

A

Education; graded excerise programme

Psychological support
- CBT

Aerobic exercise - strong evidence

Analgesia
- amitriptyline; duloxetine; pregabalin

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3
Q

Describe the clinical features of PMR [+]

A

Patients may have a relatively rapid onset of symptoms over days to weeks:

Stiffness and pain in the
* Shoulders, potentially radiating to the upper arm and elbow
* Pelvic girdle (around the hips), potentially radiating to the thighs
* Neck
* NOT true weakness - only from the pain that’s occurring

Systemic symptoms (40-50%).
* Includes low grade fever
* fatigue
* anorexia
* weight loss
* depression.

Peripheral oligoarticular arthritis (50%).

The characteristic features of the pain and stiffness are:
* Worse in the morning
* Worse after rest or inactivity
* Interfere with sleep
* Take at least 45 minutes to ease in the morning
* Somewhat improve with activity

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4
Q

Tx for PMR? [1]

Tx for TA? [1]

A

PMR:
- Prednisolone 15-20mg/day

TA:
- Prednisolone 60 mg/day

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5
Q

RA presents similiarly in the early stages of the disease to PMR.
What specifically should you look for give a ddx? [2]

A

Rheumatoid arthritis can have an initial phase that presents similarly to PMR and so the presence of synovitis or clinical features suggestive of rheumatoid arthritis should prompt consideration of this as a potential diagnosis and referral to secondary care for confirmation.

RA patients also do not respond well to corticosteroids, unlike PMR

RA antibodies (anti-CCP; RF)

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6
Q

Which type of bursitis is associated with PMR? [1]

A

subacromial bursitis is associated with PMR.

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7
Q

What are the key clinical features of GCA? [4]

A

unilateral headache
jaw claudication
visual disturbance
tender or thickened temporal artery on palpation

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8
Q

Describe the treatment regime for PMR

A

Oral corticosteroids 15mg prednisolone daily, initially and gradually weaned off them with dose adjustments typically being every 4-8 weeks and reviews (telephone or face to face) scheduled for one week after each dose adjustment.

Treatment with steroids typically lasts 1-2 years. NICE suggest the following reducing regime of prednisolone:
* 15mg until the symptoms are fully controlled, then
* 12.5mg for 3 weeks, then
* 10mg for 4-6 weeks, then
* Reducing by 1mg every 4-8 weeks

In secondary care, patients may be considered for DMARD treatment as 2nd line therapy (e.g. methotrexate) or tocilizumab as 3rd line.

NB: Patients with PMR have a dramatic improvement in symptoms (at least 70%) within one week. Inflammatory markers return to normal within one month. A poor response to steroids suggests an alternative diagnosis.

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9
Q

Temporal arteritis, also known as giant cell arteritis, is a vasculitis predominantly affecting medium and large arteries, particularly the branches of the [] artery

A

Temporal arteritis, also known as giant cell arteritis, is a vasculitis predominantly affecting medium and large arteries, particularly the branches of the carotid artery.

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10
Q

Describe the presentation of temporal arteritis [5]

A

Unilateral headache is the primary presenting feature, typically severe and around the temple and forehead. It may be associated with:
* Scalp tenderness (e.g., noticed when brushing the hair)
* Jaw claudication
* Blurred or double vision
* Loss of vision if untreated

Anterior ischemic optic neuropathy accounts for the majority of ocular complications

Lethargy, depression, low-grade fever, anorexia, night sweats

NB patients are typically > 60; rapid onset (< 1month)

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11
Q

Describe why vision testing is key in CGA [1] (Describe the pathophysiology)

A

Anterior ischemic optic neuropathy:
- occlusion of the posterior ciliary artery (a branch of the ophthalmic artery)
- causing ischaemia of the optic nerve head
- may result in temporary visual loss - amaurosis fugax
- permanent visual loss is the most feared complication of temporal arteritis and may develop suddenly

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12
Q

Describe investigations would conduct for CGA [3]

A

raised inflammatory markers

temporal artery biopsy
- skip lesions may be present
- multinucleated giant cells present

Duplex ultrasound
- showing the hypoechoic “halosign and stenosis of the temporal artery

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13
Q

Management of CGA? [+]

A

Steroids are the mainstay of treatment. They are started immediately, before confirming the diagnosis, to reduce the risk of vision loss. There is usually a rapid and significant response to steroid treatment. Initial treatment is:

  • 40-60mg prednisolone daily with no visual symptoms or jaw claudication
  • 500mg-1000mg methylprednisolone daily with visual symptoms or jaw claudication

Once the diagnosis is confirmed and the condition is controlled, the steroid dose is slowly weaned over 1-2 years.

low-dose aspirin should be considered to reduce the risk of ischemic complications
Proton pump inhibitor (e.g., omeprazole) for gastroprotection while on steroids
Bisphosphonates and calcium and vitamin D for bone protection while on steroids

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14
Q

How do you differentiate CGA to central retinal artery occlusion? [2]

A

Similarities: sudden onset, loss of vision

Differences: fundoscopy may reveal presence of cherry red spot with retinal whitening, no muscle stiffness

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15
Q

How do you different AS from mechincal back pain?

A

AS:
- Young men
- Lower back pain that can be so intense that wakes people up
- > 3 months
- Exercise improves pain
- More insidious onset

MBP:
- > 40 yrs
- typically acute pain
- excerise worsens the pain
- Pain uncommon at night
- No morning stiffiness

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16
Q

Describe the test used to quantify AS [1]

A

Schober’s test:
- Patient stands straight; L5 vertabrae is located
- Point is marked at 10cm above and 5cm below L5
- Patient bends forward
- A length of less than 20cm indicates a restriction in lumbar movement a supports a dx.

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17
Q

Describe the medical management of AS [3]

A
  • Regular exercise like swimming
  • First line treatment: NSAIDS
  • Physiotherapy
  • Second line: Anti-TNF: for patients with persistently high disease activity; e.g. adalimumab
  • Third line: IL-17 antibodies: Secukinumab or ixekizumab

  • DMARDs only useful in peripheral joint involvement
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18
Q

Which neurological complications are associated with AS? [1]

Which GI complications are associated with AS? [1]

A

Neurological complications:
- Atlantoaxial subluxation and cauda equina syndrome are rare but serious neurological complications associated with AS.

GI:
- Increased risk of IBD

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19
Q

Describe diagnositic criteria for AS [5]

A

Limited lumber movement
Reduced chest expansion
Radiological changes:
- Progressive loss of joint space –> sclerosis –> fibrosis of joints
- CXR: apical fibrosis
- Syndesmophytes: formation of bony bridges that fuse - causes bamboo spine

Complete fusion of sacro-iliac joint on right photo

20
Q

What are syndesmophytes? [1]

A

Syndesmophytes formation of bony bridges that fuse
- Syndesmophytes are calcifications or heterotopic ossifications inside a spinal ligament or of the annulus fibrosus.

OA osteophytes DONT fuse

21
Q

Describe the basic pathophysiology of AS [2]

A

initial inflammatory stage:
- activation of the immune system leading to inflammation within the entheses.

reparative stage:
- ongoing inflammation leads to new bone formation in an attempt to repair the damage caused in the earlier phase
- However, this process is dysregulated in AS resulting in pathological bone formation.

22
Q

What are the 7As of AS EAM? [7]

A

Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
and cauda equina syndrome

23
Q

Which CV complications are AS patients at risk of? [4]

A

aortitis, aortic regurgitation, conduction abnormalities and ischemic heart disease.

24
Infection from which organisms typically causes reactive arthritis? [1]
The most common triggers of reactive arthritis are **gastroenteritis** or **sexually transmitted infections.** - **Chlamydia** may cause reactive arthritis. ## Footnote **NB**: Gonorrhoea typically causes septic arthritis rather than reactive arthritis.
25
Investigations for ReA? [+]
**Blood tests** - ongoing inflammation (raised **ESR** and **CRP**) - **ANA and RF**: to rule out other forms of arthritis - **HLA B27** - present in 40% patients **Urine test:** - **NAAT** to detect **Chlamydia trachomatis** **Stool test:** - Test for **Salmonella, Shigella, Campylobacter, and Yersinia** **Radiological changes:** - Early stages - no specific changes - chronic ReA - radiographic changes in 70% **Synovial fluid analysis:** - to **exclude** alternative diagnoses like **septic arthritis**; **gout and pseudogout** - Synovial cultures are always negative. ## Footnote **NB** - Stool cultures are usually negative by the time arthritis occurs, but should be considered if diarrhoea present or recently resolved.
26
Describe the treatment used for ReA [+]
**Treatment of arthritis** - first-line therapy for acute phase: **NSAIDs** - **Corticosteroids**: during an acute flare, or unresponsive to NSAIDs. - **DMARDs**: **Sulfasalazine** is effective in peripheral disease and has little or no effect on spinal disease; **Methotrexate** is effective in treating both acute and chronic ReA especially, in patients with spinal involvement. - **Anti-TNF-α therapy:** **Etanercept** is an emerging therapy, **Antibiotics** for acute Chlaymdia infection - **doxycycline or azithromycin** ## Footnote **NB** - Antibiotics may be given until septic arthritis is excluded.
27
How would you treat cicinate balanitis or keratoderma blennorrhagica in ReA? [1]
topical steroids.
28
Describe the pathophysiology of SLE [+]
Body produces **B cells**. Normally when they **react** to **self they get destroyed.** In SLE they **escape** from **peripheral circulation** - they **differentiate** into **plasma** **cells** and **produce** **antibodies** to **nuclear** parts of cells (thus ANA) **Complexes of antibody-antigen deposit** in the **body** and **cause** **damage** e.g. skin, joints and renal are most common When deposited the immune system causes complexes. But in SLE, have **complement** **dysfunction**. This **adds to the inflam cascade** and actives inflam cytokines Over the course of lifetime, get waxes and wanes of SLE
29
Describe the clinical presentation of SLE
**Joint pain & swelling** (without joint destruction) **Malar rash** - spares nasolabial folds. Feels inflammed **Mouth ulcers** **Reynauds** **Livedo reticularis** - broken lattice work in inflam. disease **Photosensitivity** **Lupus nephritis** **Alopecia** **Discoid erythematosus** **Lymphadenopathy**
30
Which antibodies need to know that are associated with SLE? [6]
**ANA** - **DS-DNA** - highly specific to SLE, meaning a positive result suggests SLE rather than other causes. But only half have ds-ANA **Anti-SM** - highly specific but not very sensitive Anti-RNP Anti-Ro Anti-La
31
Describe the general principles for treating SLE go over MORE
**Skin**: - **hydroxychloroquine** or azathriopine. **Joints** - hydroxychloroquine or methotrexate **Vital organs:** - Azathriopine - MMF - Cyclophosphamide - esp. if renal involvement **PassMed**: All patients - **hydroxychloroquine** **Mild Disease**: - hydroxychloroquine & low dose pred. - Methotrexate and NSAIDs can also be used **Moderate disease:** - hydroxychloroquine with short term pred. - Additional agents may include methotrexate, azathioprine, mycophenolate and ciclosporin. **Severe disease:** - induction therapy of intense immunosuppressants and then maintenence - DMARDs (methotrexate; MMF; cyclophoshamide) & Biologics (Rituximab; Belimumab) may also be used
32
Describe the clinical presentation of Sjogrens
**Sicca (dryness)** - eyes (keratoconjunctivitis sicca) - mouth - vagina **Arthralgia** **Raynaud's, myalgia** **Sensory polyneuropathy** **Gland swelling** (parotitis)
33
Describe how you diagnose Sjogrens
**Assess clinical features:** - **dry eyes** > 3 months; use of tear substitutes more than three times daily or presence of keratoconjunctivitis sicca with ocular staining score ≥3. - **dry mouth** consistently **Serology**: - anti-Ro; anti-La - Elevated IgG levels **Further tests:** - **Schirmer's test** - < 5 mm/5mins wetting and / or Rose Bengal scores of > 4 - **Sialometry** < 1.5ml/15mins **Histopathology** - Lip biopsy exhibiting focal lymphocytic sialadenitis with a focus score ≥1 per 4 mm² glandular tissue area.
34
What are the antibodies seen in APS? [3]
* **anticardiolipin antibodies** * **anti-beta2 glycoprotein I (anti-beta2GPI) antibodies** * **lupus anticoagulant**
35
# SLE symptoms Other symptoms can be organ-specific caused by active inflammation these tend to develop within 5 years of diagnosis, such as:
Renal - **lupus nephritis** **Pleuritis**; pneumonitis; PE **Pericarditis**; **Reynauds**; Atherosclerosis **Headache or migraine**; seizure; pyschosis
36
Which other autoimmune conditons is SLE associated with? [3]
**anti-phospholipid syndrome** (20-30%), **Sjogren**'s disease (17.5%) and autoimmune **thyroid disease** (7.5%).
37
Describe the diagnostic criteria for SLE
The European League Against Rheumatism (EULAR) / American College of Rheumatology (ACR) criteria (2019) can be used for diagnosis. This takes into account the clinical features and autoantibodies suggestive of SLE. **uses 11 criteria of which 4 or more are required for diagnosis**
38
Describe the complications of SLE
**Nephritis**: - Lupus nephritis: can lead to proteinuira; haematuria; end stage kidney disease **CV disease**: - CAD; myocarditis; pericarditis; heart failure **Pulmonary**: - ILD; pulmonary HTN; acute lupus pneumonitis **Neuropsyc:** - Head and mood to stroke or seizures **Hematologic abnormalities**: - leukopenia, lymphopenia, thrombocytopenia and autoimmune hemolytic anaemia.
39
**anti-Smith**: highly specific (> 99%), sensitivity (30%)
40
Describe the presentation of discoid LE [3]
Photosensitive lesions of the face Scarring alopecia Hyper & hypopigmentation
41
What is the management for DLE? [3]
* **topical steroid cream** * oral **antimalarials** may be used second-line e.g. **hydroxychloroquine** * **avoid sun exposure**
42
Define Sjogrens syndrome [1]
Sjögren’s syndrome is an autoimmune condition where you get **lymphocytic infiltration of exocrine glands** notably the **lacrimal and salivary glands**, causing symptoms of **dry mouth, eyes and vagina**. Dry eyes and dry mouth can be called sicca symptoms.
43
Describe the pathophysiology of Sjogren's syndrome
Aberrant immune response - **autoreactive T cells become activated and infiltrate exocrine glands.** Within the affected glands, there is a marked increase in pro-inflammatory cytokines including interleukin-1 (IL-1), tumour necrosis factor-alpha (TNF-α), and interferon-gamma (IFN-γ). The local inflammatory milieu results in glandular dysfunction through both direct cytotoxic effects on acinar epithelial cells and disruption of normal glandular architecture. **B cell hyperactivity** is another hallmark of Sjögren's syndrome. There is **increased production of autoantibodies such as anti-Ro/SSA and anti-La/SSB,** which are directed complexes within the nucleus of glandular epithelial cells. **These autoantibodies** may contribute to **tissue damage either directly or via immune complex formation and deposition within glandular tissues**. Chronic inflammation leads to **fibrosis and atrophy of the exocrine glands over time**. The resultant loss of functional **acinar cells impairs saliva and tear production**, manifesting clinically as dry mouth and dry eyes.
44
Describe the primary [5] and secondary [4] treatment options for Sjogrens
**Primary treatment options:** * **Artificial tears** (e.g., polyvinyl alcohol eye drops during the day and carbomer gel at night) * **Artificial saliva** * **Vaginal lubricants** * **Pilocarpine** (oral) can be used to stimulate tear and saliva production * **Hydroxychloroquine** may be considered, mainly in patients with associated joint pain **Secondary treatment options** are usually prescribed by an ophthalmologist and include: * **Topical NSAIDs or Corticosteroids**: usually short-term use. Associated with corneal complications long-term. * **Topical cyclosporin**: reserved for patients needing recurrent courses of topical steroid. * **Serum tear drops:** application of autologous or allogenic serum. Variable efficacy. ## Footnote **NB**: Pilocarpine stimulates muscarinic receptors, stimulating the parasympathetic nerves and promoting salivary and lacrimal gland secretion.
45
The major complication of SS is **[]**.
The major complication of SS is **lymphoma**.
46
Which skin condition is associated with APS? [1]
**livedo racemosa** *permanent Livedo reticularis*
47
Which cardiac complication is associated with APS? [1]
**Libmann-Sacks endocarditis** is a non-bacterial endocarditis with growths (vegetations) on the heart valves (most often the mitral and aortic valves). It is associated with SLE and antiphospholipid syndrome.
48
Describe the primary [1] and secondary thromboprophylaxis (initial VTE; recurrent VTE; arterial thrombosis [3]
**primary thromboprophylaxis** - low-dose aspirin **secondary thromboprophylaxis** * initial venous thromboembolic events: **lifelong warfarin with a target INR of 2-3** * recurrent venous thromboembolic events: **lifelong warfarin**; if occurred whilst taking warfarin then consider **adding low-dose aspirin, increase target INR to 3-4** * **arterial thrombosis** should be treated with lifelong warfarin with **target INR 2-3**