Rheumatology: From ABCs to SLE

Question 1

Patient presents with joint pain and stiffness. They state it occurs in the knuckle and its closest finger joint of their right index and left ring finger. The stiffness is worst in the morning but improves over the course of the day.

1) What is the suspected diagnosis?
2) What investigations should be done?
3) What scoring systems aid diagnosis?

Answer 1

Rheumatoid arthritis: Symmetrical, multiple joint pain and stiffness that improves throughout the day. May also experience systemic upset.

XR for bone status

Antibodies:

1. Anti-Rf
2. Anti-CCP
   3) DAS28 >2.6

ELAR >=6

Question 2

In rheumatoid arthritis, what is the…

1) X ray findings
2) Treatment algorithm

Answer 2

1) Periarticular erosions, loss of joint space, evidence of osteoporosis
2) Steroid to reduce flare then…
1. HCQ (mild)/MTX/LFM/SFZ
2. Combo of 2 above
3. MTX + TNFi
4. MTX + rituximab

Question 3

Which of the following DMARDs are NOT teratogenic?

Methotrexate

Hydroxychloroquine

Sulfasalzine

Leflunomide

Answer 3

Sulfasalzine and HCQ safe in pregnancy

Question 4

Which DMARDs causes …

Bone marrow suppression

leukopenia

teratogenicity

Answer 4

Methotrexate

Question 5

Which DMARDs causes …

Hypertension and peripheral neuropathy

Answer 5

Leflunomide

Question 6

Which DMARD causes…

Male infertility (reduces sperm count)

Answer 6

Sulfasalazine

Question 7

Which DMARD causes

Nightmares

Reduced visual acuity

Answer 7

Hydroxychloroquine

Question 8

Which DMARD causes

Reactivation of TB/Hep B

Answer 8

Anti-TNFs (adalimumab, infliximab, etanercept)

Question 9

What unique side effects does rituximab have?

Answer 9

Night sweats

Low platelet count

Question 10

Why does a little old lady with Rheumatoid arthritis haunt anaesthetists’ dreams? How does the valiant anaesthetist predict this threat?

Answer 10

Potential for slipping of C1 and C2

Can result in spinal cord compression

Pre-op MRI scans

Question 11

Patient reports a 6 month history of joint pain occuring in the DIPs of both index fingers as well as the ankles with a 4 week history of itchy eyes. There is a a 15 year history of psoriasis.

1) What is the likely diagnosis?
2) How does the diagnosis differ between sexes?
3) How do you confirm the diagnosis?
4) How is this treated?

Answer 11

1) Psoriatic arthritis: Joint pain that is symmetrical in many joints/assymetrical in few joints/affects the back, A-A joint and sarcum. Long history of psoriasis typically found.
2) Back (spondolytic) pattern more common in males, symmetrical polyarthritis more common in woemn
3) PEST tool to score symptoms

X-ray imaging of choice: ‘Pencil in cup’ deformity

4) NSAIDs for pain

DMARDS –> Anti-TNF –> Ustekinumab

Question 12

Patient presents with acute joint swelling. The joint is warm, swollen and painful. They also report sore eyes and confess the tip of their penis has also been sore the past 2 days. The patient has been on the ward with a severe bout of gastroenteritis.

1) What is the likely diagnosis?
2) How do you manage this scenario?
3) How can you whittle the differentials using the infective cause?

Answer 12

1) Reactive arthritis: Acute arthritis of a single joint. Triad of ‘can’t see (eye issues), pee (balanthitis) or climb a tree (arthritis)”.Associated with gastroenteritis and chlamydia (among other STIs)
2) Follow ‘hot joint policy’ of antibiotics and joint aspiration until septic arthritis excluded.

Aspirate should be negative for gram stain, culture and sensitivity, as well as no crystals

Manage with NSAIDs, steroids (IA single, systemic multiple)

3) Chlamydia seen in reactive, gonorrhea in septic athritis

Question 13

Patient presents with lower back pain. They report a 3 month history pain and stiffness, particularly overnight but state that it improves throughout the day. The patient also reports some eye and ankle pains. Their dad has told them to get it seen as he has had similar problems for a while.

1) What is the likely differential?
2) What tests and investigations can be done?
3) What treatment options are there including biologics

Answer 13

1) Ankylosing spondylitis: Lower back pain and stiffness worse at rest and improves with activity. More likely to occur if first-degree relative has it. Associated with HLA-B27.

2)

Measure 10cm above, 5 cm below L5 then bend. Will be <20cm total (Schober’s test)

X-ray shows ‘bamboo spine’ outgrowth at tendon inserts (syndesmophytes) and erosions

3) Typical seronegative malarkey –> Secukinumab

Question 14

Regarding connective tisssue diseases, which conditions are most associated with the following?

1) Anti-dsDNA
2) Anti-sm
3) Anti-histone
4) Anti-Ro
5) Anti-La
6) Anti-scl70
7) Anti-centromere
8) Anti-RNP

Answer 14

1-2) SLE (dsDNA more specific)

3) Drug induced lupus (will be dsDNA -ve)

4-5) Sjogren’s (Ro 70%, la 30%)

6) Diffuse systemic sclerosis
7) Limited systemic sclerosis
8) Mixed connective tissue disease

Question 15

Patient presents feeling “poorly”. They have a 6 month history of bouts of fatigue, joint pain and a sore mouth. They also state that the feel more sensitive to the sun. On examination you find a rash across the cheeks and nose bridge and enlarged lymph nodes in the groin. She is of Afro-caribbean and east asian descent.

1) What is the likely differential?
2) What tests aid diagnosis?
3) What is the treatment pathway?

Answer 15

1) SLE: Notoriously ‘systemic’ symptoms of joint pain, fatigue and fever. Classic malar (“butterfly”) rash and photosensitivity. More common in Afro-Caribbean women.
2) 1st line: ANA blood test

GS: Anti-dsDNA, Anti-Smith

Can use SLICC or ACR crtieria to diagnose overall picture

3)

Symptom/flare control: NSAIDs, Steroids, Suncream

Mild maintenance: HCQ

Severe: Immunosuppressants (Methotrexate/Azathioprine/Leflunomide)

Treatment resistant: Rituximab or belimumab

Question 16

How does discoid lupus differ from systemic?

Answer 16

Discoid only has derm manifestations

Patches of inflamed, dry lesions on face, scalp and ears

Photosensitivity

Can lead scarring alopecia that is hypo/hyperpigmented scars

Question 17

Patient presents with skin changes. They state their hands have become progressively “tight and shiny” and their fingers have white bumps under the skin. They have a pasy medical history of raynauds and reflux.

1) What is the diagnosis?
2) What tests do you want to do?
3) What is the lifestyle advice you would offer?
4) What medical options are there for treatment?

Answer 17

1) Systemic Sclerosis: Presence of Calcinosis (bumps under skin), Raynaud’s, Esophageal problems (reflux, dysphagia), Sclerodactyly (tight skin), Telangectasia (red threads under skin).
2) Antibodies: Anti-ANA, Anti-centromere

Abnormal capillaries and microhaemorrhages in nailfold capillaroscopy

3) Avoid Smoking

Stretch gently + exercise joints to maintain mobility

4) Symptom specific

Analgesia

Antacids

Nifedipine for Raynaud’s

Question 18

How does limited differ from diffuse systemic sclerosis? How does this alter management?

Answer 18

Limited: CREST

Diffuse: CREST + Lung (HTN, PF), Cardio (HTN, CAD), Kidney (Glomerulonephritis, sclerodermal renal crisis)

Steroids + Immunosuppresants to slow the disease

ACE inhibitors for hypertension

Supportive management for pulmonary fibrosis

Question 19

Patient presents with joint pain and stiffness for the past 3 weeks. The pain is present in their shoulders down their elbows, as well as groin pain, and is worse on movement.. They also note stiffness that lasts an hour in the mornings. They have tried to manage it alone but are presenting as it has begun to impact their sleep. The patient is 70 years old.

1) Whats the intentional diagnosis?
2) What tests should you run?
3) What is the management protocol

Answer 19

Polymyalgia rheumatica: Bilateral hip and shoulder pain that is worse on movement. Also have morning joint stiffness that lasts >45 minutes. Symptoms also disrupt sleep. Features need to be present for >2 weeks for a diagnosis following exclusion of other causes.

2) See NICE guidelines for exclusion list

Raised inflammatory markers

CK-EMG is normal

Should respond to steroids to confirm diagnosis (good response after 1 week, ~70% response in symptoms + normal inflammatory markers by a month)

Steroids: 15mg until controlled

Reduce by 2.5 every 3 weeks

Reduce by 1mg every 1-2 months

Question 20

What is the rule of thumb for patients on long term steroids?

Answer 20

DON’T STOP

DON’T: Ensure that they know they are steroid dependent after 3 weeks so DONT stop suddenly in case adrenal crisis occurs

Sick day rules: Discuss increasing dose when sick

Treatment card: So others know they are steroid dependent

Osteoporosis prevention: Give bisphosphonates, calcium and vitamin D supplements

Proton pump inhibitors: For gastric protection

Question 21

Patient phones a GP with a headache. They state they have had an extremely sore left side of the head that is made worse when they hold their head. They also report feeling hot and during the phone call have said their vision is blurry. They are 60 years old and white.

1) What is the suspected diagnosis?
2) How do you confirm the diagnosis
3) Who do you refer to?
4) How is it managed?
5) What complications are you worried about?

Answer 21

1) Giant cell arteritis: Severe unilateral headache exacerbated by touching the scalp. Associated with systemic symptoms of fever, muscle aches, weight loss. Can cause rapid, irreversible loss of vision. More common in Over 50s and white populations.
2) Clinical presentation

ESR >50mm/hour

Multinucleated giant cells found on temporal artery biopsy

3) Immediate ophtho if visual symptoms present

Rheumatology and vascular for diagnosis and mangagement

4) Immediate long term steroids

Aspirin 75mg

PPI

5) Blindness, Stroke, Aortic aneurysm/dissection

Question 22

Patient presents with joint pain for the past 6 weeks. The pain is present in their shoulders, as well as hip pain, and is worse on movement.. They state that they feel quite tired and weak. They have also noticed a scaly rash on their flexor surfaces. The patient is 70 years old.

1) What is the suspected diagnosis
2) How is the condition diagnosed
3) How to treat

Answer 22

1) Polymyositis/dermatomyositis: Muscle pain, fatigue and weakness that occurs bilaterally and usually the shoulders or hips. Skin changes include scaly flexures (Gottron’s lesions), dorsal or facial rashes. Eye swelling and bumps under the skin can also occur.
2) Clinical presentation with

ELEVATED CK (Contrast with normal in PMR)

Reduced EMG readings

Anti-jo1 (both), Mi-2, ANA (derm)

Defiinitive is muscle biopsy showing immune cell infiltration

3) Steroids are first line

Immunosuppresants/IGs/Biologics if that doesnt work

Question 23

27 year old Patient presents to A+E with a suspected stroke. From glancing at her ECS you note that they have had 2 miscarriages in the past. They were diagnosed with lupus nearly 3 years ago.

1) What is the likely underlying cause of the stroke?
2) How do you investigate the suspected cause?
3) How do you manage the condition?

Answer 23

1) Anti-phospholipid syndrome: Recurrent miscarriage and/or thrombosis due presence of anti-phosholipid antibiodies inducing a hypercoagulable state.
2) Reucurrent thrombosis or miscarriage along with

Lupus anticoagulant

Anticardiolipin

Anti-B-GP1 antibodies

3) Warfarin with range 2-3 (3-4 with thrombosis)

If pregnant: LMWH + aspirin

Question 24

Patient with lupus reports a 2 month history of dry eyes, mouth and vagina. What

1) Is the likely diagnosis?
2) Is the clinical and antibody testing you want to do?
3) How do you manage the condition?

Answer 24

1) Sjogren’s Syndrome: Dry mucous membranes that is either primary or secondary to SLE.
2) Schirmer’s test: Place filter paper under lower eyelid for 5 mins, <10mm is significant for Schirmer’s
3) Aritficial saliva, tears, lubricants, HCQ

Question 25

Name the vasculitis associated with the following…

purple spots, joint and abdo pain, renal problems, p-ANCA

severe asthma, elevated eosinophils, p-ANCA

renal failure +/- breathlessness, haemoptysis

Saddle nose, cough, wheeze, glomerulonephritis, c-ANCA

Purplish rash in Hep B,C or HIV patient

Persitent fever, peeling skin, Strawberry tongue, children

Fever, aches, aneurysms in over 40s

Answer 25

Henoch-Schonlein purpura: purple spots, joint and abdo pain, renal problems, p-ANCA

Eosinophilic Granulomatosis with polyangiitis: severe asthma, elevated eosinophils, p-ANCA

Microscopic polyangitis: renal failure +/- breathlessness, haemoptysis

Granulomatosis with polyangiitis: Saddle nose, cough, wheeze, glomerulonephritis, c-ANCA

Polyarteritis Nodosa: Purplish rash in Hep B,C or HIV patient

Kawasaki disease: Persitent fever, peeling skin, Strawberry tongue, children

Takayasu’s arteritis: Fever, aches, aneurysms in over 40s

Question 26

Patient presents with sore joints. They state that they have pain in their fingers and elbows over the past 2 months. In the same time frame, they report a sore mouth, genitals and eyes. They also have large red patches on their shins. They are from Turkey originally

1) What is the likely diagnosis?
2) How do you test for it?
3) How is it treated?

Answer 26

Behcet’s syndrome: Classic symptoms of Oral ulcers, genital ulcers and anterior uveitis. Joint pain, GI, neuro symptoms and erythema nodosum are associated with the condition.

2) Typically clinical

Positive pathergy test: pricking forearm with sterile needle creates >5mm weal.

3) Topical + Systemic steroids

Colchicine

Immunosuppressants and biologics

Question 27

How does gout differ from pseudogout?

Answer 27

Chondrocalcinosis: white line through joint space is pathogmonic

Aspirate: Calcium pyrophosphate crystals, Rhomboid, +ve birerefringence

Osteoarthritic changes

Question 28

Regarding osteomalacia…

1) Which patient groups are more likely to get it?
2) What is the value difference for vit D insufficiency and deficiency
3) What are the 3 vitamin D treatment regimes?
4) What is the maintenance dose following the treatment doses?

Answer 28

1) Darker skin, colder climates, stay inside more
2) <75 insufficiency, <50 deficiency
3) 50,000 IU once weekly over 6 weeks

20,000 IU twice weekly over 7 weeks

4000IU daily over 10 weeks

4) Maintenance dose 800IU day

Question 29

65 year old patient presents with joint pain. They report a 3 month history of deep pain in their upper and lower back. They also report finding it harder to hear. You decide to do an X-ray. What…

1) Is the potential diagnosis from the history?
2) What would you find on X-ray
3) What blood results would you expect?
4) How do you manage the condition
5) What are the two main concerns for the long term?

Answer 29

1) Paget’s disease: Bone pain in the axial skeleton, deformity of long bones and hearing loss.
2) Osteolytic lesions that are well circumscribed, can be v shaped in long bones

Skull has light patchy areas

3) Raised ALP with normal calcium and phosphate
4) Bisphosphonates are mainstay +/- Calcium, vit D

NSAIDs for pain

5) Osteoscarcoma, spinal stenosis/cord compression