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Medicine (Year 5) - Moriom > Rheumatology (conditions continued) > Flashcards

Rheumatology (conditions continued) Flashcards

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1
Q

Systemic lupus erythematosus (SLE)

A
  • is a multisystem autoimmune disease of unknown aetiology that can present at any age.
  • SLE is prone to relapses and remissions and can result in morbidity due to flares of disease activity and accumulating damage over time, along with an increased risk of premature death due to infection or cardiovascular disease.

Lupus erythematosus describes the typical rash seen in SLE. The “systemic” part indicates the potential for multi-organ involvement.

Antiphospholipid syndrome can happen secondary to SLE, which carries an increased risk of venous thromboembolism.

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2
Q

SLE pathophysiology

A

Pathophysiology

SLE is thought to be a type III hypersensitivity reaction where impaired clearance of dead cells and failure of processes giving immune tolerance to nuclear antigens may lead to the development of antibodies against double-stranded DNA and nuclear proteins.

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3
Q

RF for SLE

A

* female
* >30
* African-caribbean descent
* Drugs e.g. sulfasalazine, phenytoin
* family history
* smoking

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4
Q

SLE is associated with

A
  • Antiphospholipid syndrome
  • RA
  • Other autoimmune e.g. thyroiditis
  • Atherosclerosis/HTN/dyslipidaemia, diabetes, osteo
  • Non-hodgkins lymphoma
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5
Q

presentation of SLE

A

General

  • Fever
  • Fatigue
  • Lymphadenopathy
  • Mouth ulcers

Skin and hair

  • Butterfly rash
  • Photosensitive rash
  • DIscoid rashes
  • Raynauds
  • Alopecia
  • Vasculitis rashes

MSK

  • Myalgia
  • Arthritis

Pulmonary

  • Pleurisy
  • alveolitis
  • PE (antiphospholipid association)

Cardiovascular

  • Pericarditis
  • HTN
  • Myocardiits

Renal

  • Protien uria, haematuira, HTN

Neuropsychiarric symptoms

  • Anxiety
  • Depression
  • Psychosis
  • Seizures
  • Strokes secondary to vascultiis
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6
Q

Antibodies for SLE

A

Common

  • ANA (high sensitivity)- all patients with SLE will have this, but some without will also have it
  • Anti-dsDNA (high specificity) - patients with SLE will defo have this

Others

Anti-Smith, anti-Ro (SS-A), and anti-La (SS-B)

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7
Q

Monitoring disease activity: SLE

A
  • Erythrocyte sedimentation rate (ESR) is used
  • C-reactive protein (CRP) may be normal during active disease
  • If CRP is raised, this may indicate an underlying infection
  • C3 and C4 may be low in active disease
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8
Q

investigations for SLE

A

Autoantibodies
- ANA
- anti-dsDNA

FBC
- anaemia, leukopeia, thrombocytopenia

APTT
- prolonged if antiphospholipid sydnrome - think VTE

UE
- renal manifeststions e.g. glomerulonephritis
- calcium can be high

Urinalysis
- haematuria
- casts
- proteinuria

CXR
- pleural effusion
- inflitrates
- cardiomegaly

ECG
- chest pain - rule out cardiopulmonary symptoms

Antiphospholipid antibodies
- venous thromboembolism
- recurrent miscarriages
- prolonged APTT

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9
Q

management of SLE

A

First line
- NSAIDS for MSK pain and headaches e.g. naproxen (PPI)
- Sunscreen

Second line
- Hydroxychloroquine

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10
Q

advice to patients with SLE

A
  • Patients should avoid excessive sun exposure and use effective sun screen
  • Patients should be aware that some drugs can exacerbate SLE
  • Patients should have smoking cessation help and aim to stop
  • Oestrogen-containing hormonal contraception can worsen SLE, but the lowest dose can cautiously be used if there are no contraindications and no anticardiolipin antibodies present
  • Fertility is normal and pregnancy is safe in stable/mild SLE
  • If SLE is severe, pregnancy should be delayed until the disease is better controlled:
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11
Q

drug-induced lupus vs SLE

A
  • Drug-induced lupus (DIL) is an autoimmune condition where a patient develops signs and symptoms similar to systemic lupus erythematosus after exposure to certain drugs.
  • DIL carries a more favourable prognosis compared to SLE and resolves within a few weeks after discontinuing the offending drug.
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12
Q

causes of drug induced lupus

A

Drug Causes

  • Procainamide
  • Hydralazine
  • Minocycline
  • Terbinafine
  • Sulfasalazine
  • Isoniazid
  • Phenytoin
  • Carbamazepine
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13
Q

investigations for drug induced lupus

A

Anti-nuclear antibodies (ANA):
* Nearly positive in all patients

Anti-double-stranded DNA (anti-dsDNA):
* Absent in most patients, used to screen for SLE

Anti-histone antibodies:
* Present in up to 90% of patients

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14
Q

antiphospholipid syndrome

A

an autoimmune disorder characterised by:
* arterial and venous thrombosis,
* adverse pregnancy outcomes
* raised levels of antiphospholipid (aPL) antibodies.

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15
Q

antiphospholipid syndrome can occur secondary to

A

SLE or other autoimmune conditions

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16
Q

antiphospholipids associations

A
  • 30% of people who have systemic lupus erythematosus have aPL antibodies
  • Higher prevalence in black people
  • Most seen in young women of childbearing age with a male: female ratio of 1:3.5
  • Accounts for 20% of recurrent thrombosis in young people
  • Accounts for 15% of cases of recurrent foetal loss
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17
Q

presentation of antiphospholipid syndrome

A
  1. Recurrent foetal loss
  2. Features of thrombocytopenia:
  • Petechial rashes
  • Mucosal bleeding
  1. Arthralgia
  • Due to possible underlying systemic lupus erythematosus
  1. Livedo reticularis
  2. Coagulation assays show a prolonged APTT
  3. Pulmonary hypertension
  4. Pregnancy morbidity:
  • Pre-eclampsia
  • Placental abruption
  • Intrauterine growth restriction
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18
Q

Investigations for antiphospholipid syndrome

A

Autoantibodies
1) If associated with SLE: ANA and anti-dsDNA antibodies

2) Any of these positive/elevated on 2 occasions 12 weeks apart:
- Lupud anticoagulant
- Anticardiolipin antibodies
- Anti-beta2-glycoprotein I antibodies

Coagulation assays
- Prolonged APTT

Others
- Full blood count - thrombocytopenia
- Venous doppler US - if DVT suspected
- CTPAor V/Q scan - if PE suspected

-

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19
Q

diagnosing antiphospholipid syndrome

A

The diagnosis of APS requires at least 1 clinical criterionand 1 laboratory criterion.

Clinical criteria are:

  • 1 or more episodes of vascular thrombosis
  • 1 or more unexplained losses of morphologically normal foetuses at or beyond the 10th week of gestation

Laboratory criteria are (must be positive on 2 or more occasions at least 12 weeks apart):

  • Lupus anticoagulant present
  • Anticardiolipin antibody present
  • Anti-beta2-glycoprotein I antibody
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20
Q

management of antiphospholipid syndrome: Non-pregnant patients

A

2 scenarios

1) No previous VTE: low dose aspirin

2) Single VTE episode: life long warfarin

  • initially aim for an INR of 2-3
  • if recurrent: increase INR to 3-4 and consider adding low-dose aspirin
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21
Q

management of antiphospholipid syndrome: pregnant patients

A

Different stages:
Stage 1
* Low-dose aspirin once the pregnancy is confirmed via urinary beta-hCG testing

Stage 2
* Low molecular weight heparin (LMWH) once the foetal heart is seen on ultrasound until 34 weeks gestation when it is then stopped

Stage 3
* Return to warfarin (teratogen) post-delivery

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22
Q

Systemic sclerosis

A

a multisystem autoimmune disease leading to increased fibroblast activity causing abnormal growth of connective tissue -> this leads to vascular damage and fibrosis:
- Skin
- GI tract
- Heart
- Lungs
-

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23
Q

There are three main patterns of SSc:

A
  • Limited cutaneous SSc (lcSSc)
  • Diffuse cutaneous SSc (dcSSc)
  • Scleroderma
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24
Q

presentation of limited cutaneous systemic sclerosis (lcSSc) (limited systemic scleroderma)

A

most common type - commonly affecting face and distal limbs

CREST syndrome features

  • Calcinosis
  • Raynaud’s disease
  • (O)Esophageal dysmotility and gastro-oesophageal reflux (GORD)
  • Sclerodactyly
  • Telangiectasia
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25
Diffuse cutaneous systemic sclerosis (dcSSC) (diffuse scleroderma)
Less common- commonly affecting the trunk and proximal limbs * Usually more rapid onset with skin thickening and Raynaud’s phenomenon happening together or with a short interval * Internal organ involvement more common such as interstitial lung disease
26
Presentation of diffuse cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis includes the CREST features and also affects internal organs, causing: * Cardiovascular problems (e.g., hypertension and coronary artery disease) * Lung problems (e.g., pulmonary hypertension and pulmonary fibrosis) * Kidney problems (e.g., glomerulonephritis and scleroderma renal crisis)
27
general features of systemic sclerosis
- Raynauds - Calcinosis - Swelling of fingers and toes - Sclerodactyly - Prayer sign - Digital ulcers - tightening of skin around the face and mouth - Telangiectasia - Salt and pepper skin
28
features of systemic sclerosis: skin
* Raynaud’s phenomenon is common in around 90% of cases * Calcinosis * Symmetrical swelling of fingers and toes * Sclerodactyly – hard and thickened skin * Prayer sign – swelling and sclerosis cause patients to be unable to make a fist or place the palmar surfaces together * Digital ulcers * Tightening of skin around the face and mouth * Telangiectasia * “Salt and pepper” appearance of skin due to areas of hypopigmentation and hyperpigmentation
29
Scleroderma vs sclerodactyly
**Scleroderma** refers to the hardening of the skin, giving the appearance of shiny, tight skin without the normal skin folds. These changes are most notable on the hands and face. **Sclerodactyly** describes the skin changes in the hands. Skin tightening around the joints restricts the range of motion and reduces function. The fat pads on the fingers are lost. The skin can break and ulcerate.
30
telangiectasia
refers to dilated blood vessels in the skin measuring less than 1mm in diameter.
31
Calcinosis
refers to calcium deposits under the skin, most commonly found on the fingertips.
32
Systemic sclerosis: Musculoskeletal symptoms
* Joint pain and swelling * Myalgia * Restriction of joint movement
33
systemic sclerosis : GI
- GORD - Dysphagia Oesophageal dysmotility is caused by atrophy and dysfunction of the smooth muscle, as well as fibrosis of the oesophagus. It causes swallowing difficulties, chest pain, acid reflux and oesophagitis.
34
Systemic sclerosis: respiratory
1. Pulmonary fibrosis 2. Pulmonary hypertension * Leading cause of death in SSc
35
# . Systemic sclerosis: Cardiovascular features
* Microvascular coronary artery disease and subsequent MI * Pericarditis * Arrhythmias * Left ventricular dysfunction
36
systemic sclerosis: Renal features
Scleroderma renal crisis Presents with accelerated hypertension with: * Oliguria * Headache * Fatigue * Oedema * Rapidly rising serum creatinine * Proteinuria * Microscopic haematuria
37
Systemic sclerosis: Genitourinary features
* Erectile dysfunction in 80-90% of men due to vascular changes * Women may have dyspareunia
38
autoantibodies for systemic sclerosis
**Antinuclear antibodies (ANA):** * In 90% of patients **Anti-centromere antibodies:** * Associated with **limited** cutaneous systemic sclerosis **Anti-scl-70 antibodies:** * Associated with **diffuse** cutaneous systemic sclerosis
39
management of systemic sclerosis
**First line**: smoking cessation, exercise, and physiotherapy **Second line** * Immunosuppression is considered in patients with lung involvement, myositis, or inflammatory arthritis: * Inflammatory arthritis/myositis present: **methotrexate** * nterstitial lung disease present: **mycophenolate, cyclophosphamide, azathioprine, rituximab**
40
management of renal crisis caused by systemic sclerosis
* 1st line: Angiotensin-converting enzyme (ACE) inhibitors + IV 0.9% saline * This is to control hypertension in renal crises due to systemic sclerosis
41
Raynaud’s phenomenon
is where the fingertips change colour in response to even mildly cold triggers (e.g., opening the fridge). It is caused by vasoconstriction of the vessels supplying the fingers. The typical pattern is: * First white, due to vasoconstriction * Then blue, due to cyanosis * Then red, due to reperfusion and hyperaemia
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43
Raynaud’s disease
is where Raynaud’s phenomenon occurs without an associated systemic disease. It is idiopathic and makes up 80-90% of patients with Raynaud’s phenomenon
44
Treatment options for Raynaud’s include:
* Keeping the hands warm (e.g., gloves and hand warmers) * Calcium channel blockers (e.g., nifedipine) * Other specialist drugs include losartan, ACE inhibitors, sildenafil and fluoxetine
45
sjogrens syndrome
is an autoimmune condition of unknown aetiology affecting the **exocrine** glands leading to the main symptoms of: * dry eyes (xerophthalmia) * dry mouth (xerostomia) * enlargement of the parotid glands. It can happen in isolation or be secondary to another autoimmune condition such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or systemic sclerosis (SSc).
46
having sjogrens syndrome put you at an increased risk of which diseases
* lymphoid malignancy * Patients may develop primary biliary cirrhosis or chronic hepatitis
47
risk factors for Sjogrens
* Female * History of rheumatological disease: RA, SLE, or SSc
48
presentation of Sjogrens
- Dry eyes - Dry mouth - Vaginal dryness - Recurrent parotitis - Enlarged parotid glands (bilateral0 - Fatigue - Raynauds phenomemnon - Chronic pain
49
investigations for Sjogrens
* Schirmer's test * Anti- Ro and and anti-La (SS-B) antibodies (ORAL backwards) * Salivary gland biopsy if patient has unilaterally enlarged salivary glands to rule out malignancy
50
Schirmers test
* Filter paper is placed in the lower eyelid * The test is positive if <5mm of paper is wet after 5 minutes
51
management of Sjogrens
Management primarily involves treating the symptoms: * Artificial saliva for dry mouth * Artificial tears for dry eyes * NSAIDs for musculoskeletal symptoms
52
Behçet’s disease
is a multi-system disorder of unknown aetiology characterised by recurrent oral ulcers and is presumed to be an autoimmune disease. It involves the mucocutaneous, ocular, cardiovascular, renal, gastrointestinal, pulmonary, vascular, musculoskeletal, urological, and central nervous systems
53
risk factors for Behcets
* Family history * Age 20-40 years * HLA-B51
54
presentation of Behcets
classic triad of: - oral ulcers - genital ulcers - anterior uveitis
55
other symptoms of Behcets
**Skin manifestations:** * Acne in atypical areas e.g. the arms and legs * Erythema nodosum **Musculoskeletal manifestations:** * Arthritis **Vascular manifestations:** * Superficial thrombophlebitis * Deep vein thrombosis **Gastrointestinal manifestations:** * Abdominal pain * Diarrhoea * Gastrointestinal ulceration – may present with features similar to inflammatory bowel syndrome **Respiratory manifestations** – may suggest pulmonary aneurysm: * Shortness of breath * Cough * Chest pain * Haemoptysis **Neurological manifestations:** * Meningeal inflammation and signs of meningitis * Memory loss * Dysphasia * Ataxia
56
investigations for Behcets
**Pathergy testing** - Subcutaneous skin prick with a sterile needle shows induration with or without a pustule within 48 hours **Autoantibdies** - ANA and ANCA **Other tests are used depending on the presentation:** **Lumbar puncture:** * Indicated when neurological involvement suspected **MRI brain with contrast:** * When large phlebitis or CNS involvement suspected **Colonoscopy:** * When gastrointestinal symptoms are present **Upper GI endoscopy:** * When gastrointestinal symptoms are present **CT chest, CT angiography of the chest, and pulmonary angiography:** * If a pulmonary aneurysm suspected
57
diagnosis of Behcets
Recurrent ulcers + 2 of the following in the absence of other systemic disease * Recurrent genital ulceration * Skin lesions are seen by a physician in an adult not receiving corticosteroids * Eye involvement (uveitis/retinitis) observed by an ophthalmologist * Positive pathergy test interpreted by a physician at 48 hours
58
manageemnt of Behcets
Ulcers * 1st line: topical or oral corticosteroids e.g. triamcinolone paste * 2nd line: colchicine or oral corticosteroid or immunosuppression therapy * 3rd line: TNF inhibitors Eyes, GI, CNS, Vascular - Prednisolone - ANticoagulation in vasc involvement
59
Polymyositis and dermatomyositis
are autoimmune disorders causing muscle inflammation (myositis) Dermatomyositis just means there is involvement of the skin too e.g. **Gottrens** papules and **Heliotrope** rash affecting the eyelids
60
Polymyositis or dermatomyositis can be caused by
* underlying cancer, making them paraneoplastic syndromes. * A viral infection may be the trigger (e.g., Coxsackie virus or HIV). * Certain HLA genes are risk factors.
61
presetation of polymyositis and dermatomyositis
key: **symmetrical proximal muscle weakness** leading to difficulties standing froma chair, climbing stairs or lifting overhead - gradual onset - distal muscles usually spared util late disease **Polymyositis** occurs without skin features **Dermatomyositis** occurs with skin changes - Gottrons - Heliotrope rash - Periorbital oedema - Photosensitive erythematous rash
62
investigations for Polymyositis or dermatomyositis
KEY: **Creatine Kinase**- raised **Serum aldolase** - Raised **Muscle biopsy** - Perivascular or inter-fasciular inflammation - Endothelial hyperplasia in intramuscular blood vessels **Anti-Mi-2 antibodies** - posiitve
63
Other causes of a raised creatine kinase include:
* Rhabdomyolysis * Acute kidney injury * Myocardial infarction * Statins * Strenuous exercise
64
most common myositis-specific antibodies
anti-jo-1 antibodies
65
management of polymyositis
*Patients should be screened for malignancy* **In combined muscle and skin disease** * **1st line: **oral corticosteroid initial therapy + photoprotection + treatment of skin disease + treatment of underlying malignancy * **2nd line:** methotrexate or azathioprine + photoprotection + treatment of skin disease + treatment of underlying malignancy * **3rd line**: other immunosuppressants e.g. ciclosporin **In skin disease alone** * **1st line:** topical corticosteroids e.g. hydrocortisone/betamethasone/clobetasol * Combined with moisturisers and photoprotection * **2nd line:** hydroxychloroquine * **3rd line:** immunosuppressants e.g. methotrexate

Medicine (Year 5) - Moriom (34 decks)

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