Rheumat Key Flashcards
1
Q
Anti-tissue transglutaminase,
Anti-gliadin, Anti-endomysial
A
Celiac Disease
2
Q
ANA
A
RA, SLE (initial), and many other auto-
immune diseases.
3
Q
cANCA
A
Wegener’s Granulomatosis
(Granulomatosis with Polyangiitis)
4
Q
pANCA
A
Churg Strauss (Eosinophilic Granulomatosis
with Polyangiitis) / UC / 1ry sclerosing
cholangitis
5
Q
Anti-smooth muscle
A
Autoimmune hepatitis
6
Q
Anti-mitochondrial
A
Primary biliary cirrhosis
7
Q
Anti-Ro, Anti-La
A
Sjogren’s disease
8
Q
Anti-Jo1
A
Polymyositis
9
Q
Anti-centromere
A
Limited sclerosis/CREST syndrome
10
Q
Anti-histone
A
Drug-induced lupus (e.g. Hydralazine)
11
Q
Anti-scl70
A
Systemic Sclerosis
12
Q
Anti-dsDNA and Anti-smith
A
SLE (but the initial test is ANA – very sensitive)
13
Q
◙ Initial (Screening) test of SLE
A
→ sensitive”
Anti-nuclear antibody (ANA) “More
14
Q
A
15
Q
Confirmatory for sle
A
◙ Confirmatory → Anti-dsDNA “Specific”
16
Q
A 55-year-old woman presents to her GP with sudden onset of pain, swelling,
and redness in the right distal interphalangeal (DIP) joint. The symptoms began
abruptly three days ago and have progressively worsened, with the pain
described as severe and throbbing. She also notes warmth around the joint.
She
has experienced similar episodes in the past that resolved after a few days.
There is no history of trauma, and on examination, the joint is visibly swollen,
erythematous, and tender to touch.
The patient is afebrile.
Q) What is the most likely diagnosi
A
Diagnosis → Gout.
Why it is gout and not osteoarthritis?
The acute onset of severe pain, redness, and swelling, particularly in a single
joint, along with the history of recurrent episodes, strongly points towards gout.
Osteoarthritis typically presents with more chronic, gradual joint pain and
stiffness,
without the intense inflammation and abrupt onset seen in gout.
17
Q
A
18
Q
Q) What is the most appropriate investigation?
Gout
A
Investigation → Synovial aspiration.
Explanation: Synovial fluid aspiration is the most appropriate investigation to
confirm the diagnosis of gout.
It allows for the analysis of the fluid, where the
presence of monosodium urate crystals confirms gout.
Additionally, it helps to
exclude septic arthritis by ruling out infection in the joint fluid.
19
Q
Management of Gout in This Scenario:
A
NSAIDs Colchicine 1. Acute Attack:
o First-line Rx: (e.g., ibuprofen) to reduce inflammation and pain.
o If NSAIDs are contraindicated: colchicine can be used.
o For severe cases or if NSAIDs/colchicine are not effective: Corticosteroids
(oral or intra-articular) can be considered.
- Lifestyle Modifications:
o Advise reducing intake of purine-rich foods (e.g., red meat, seafood).
o Encourage weight loss if overweight.
o Limit alcohol consumption. - Long-term Prevention (if recurrent attacks or chronic gout):
Allopurinol
o Initiate urate-lowering therapy (e.g., but only after the acute attack has resolved “along with NSAIDs and
Colchicine coverage”
20
Q
√ Polymyalgia rheumatica (PMR) is an inflammatory condition predominantly
seen in individuals over the age of 50. It is characterised by pain and stiffness
in the shoulders, neck, and hips, which is often worse in the morning and
improves with activity.
√ The elevated ESR (85 mm/hr in this case) is a common finding in PMR,
indicating inflammation.
A
√ The absence of joint swelling helps differentiate PMR from conditions like
rheumatoid arthritis.
√ The patient’s age, symptoms, and laboratory findings all point towards PMR
as the most probable diagnosis.
√ Corticosteroids: Low doses of corticosteroids, such as prednisone, are the
mainstay of treatment and typically result in rapid improvement of symptoms.
21
Q
A
22
Q
Here is why the other options are less likely
A
:
A) Fibromyalgia: Typically presents with widespread musculoskeletal pain but
does not usually involve elevated ESR levels.
B) Systemic lupus erythematosus: This autoimmune disease can present with
a wide range of symptoms including joint pain, but it often involves additional
symptoms such as a rash, renal involvement, and other systemic signs.
C) Rheumatoid arthritis: Usually presents with joint pain and swelling, which
is not observed in this patient.
E) Giant cell arteritis: Often presents with symptoms like headache, scalp
tenderness, jaw claudication, and visual disturbances.
While it can be
associated with PMR, the absence of these specific symptoms makes PMR the
more likely diagnosis.
23
Q
A 68-year-old gentleman visits his GP with a three-month history of overall
discomfort and pain in his shoulder, neck and hips.
He mentions that he feels
particularly stiff in the mornings, with the stiffness lasting over an hour but
improving slightly with activity as the day progresses.
Upon examination,
there is no evident joint inflammation. Blood tests reveal an erythrocyte
sedimentation rate (ESR) of 85 mm/hr (normal <15).
Which of the following is
the most probable diagnosis?
A
D) Polymyalgia rheumatica.
24
Q
A 45-year-old woman has been having low moods and widespread muscle
pain since her husband died 10 years ago.
She has been feeling extremely
fatigued over the past 3 months. She has difficulty sleeping and concentrating.
A
Resting does not improve her fatigue. In the morning she has vague back pain.
On examination, there is tenderness over multiple specific points such as
upper and lower arms, legs, shoulder girdle, hips, back, and neck. Her blood
results are all normal.
They include kidney and liver function tests, FBC, TSH,
Electrolytes, CK, Rheumatoid factor, ANA, Anti-CCP.
What is the most likely
diagnosis?
→ Fibromyalgia.
√ Chronic pain: at multiple sites, sometimes (pain all over) for > 3 months.
√ Fatigue and lethargy.
√ Cognitive impairment.
√ Depression, Anxiety, Sleep disturbance, Headaches, Dizziness are common.
+ All blood results are NORMAL.
25
• Ankylosing spondylitis
= Progressive lower back pain + stiffness (worse in
the morning and improves with activity).
± Others (sacroiliac tenderness, pain in hips and shoulders).
• Ankylosing Spondylitis has a strong association with HLA-B27 gene. √
26
◙ Fibromyalgia is a syndrome characterised by widespread pain throughout
the body with tender points at specific anatomical sites.
The cause of fibromyalgia is unknown.
◙ Epidemiology:
√ Women are around 5 times more likely to be affected than men.
√ Typically presents between 30-50 years old.
Features of Fibromyalgia:
√ Chronic pain: at multiple sites, sometimes (pain all over) for > 3 months.
√ Fatigue and lethargy.
√ Cognitive impairment.
√ Depression, Anxiety, Sleep disturbance, Headaches, Dizziness are common.
◙ Diagnosis:
√ It is clinical and by excluding other causes of chronic pain and fatigue.
√ Fibromyalgia laboratory results are normal: eg, renal and liver function
tests, FBC, TSH, Rheumatoid factor… etc.
27
Fibromyalgia mng
The management of fibromyalgia is often difficult and needs to be tailored to
the individual patient. The patient should be reassured and educated that
there is no cure for the condition.
A psychosocial and multidisciplinary approach is helpful.
√ Reassurance, Education and Explanation.
√ Aerobic exercise: has the strongest evidence base.
√ Cognitive behavioural therapy (CBT).
√ Medications for pain and anxiety: eg, tramadol, paracetamol, pregabalin,
duloxetine, amitriptyline.
28
Fibromyalgia dd
◙ Important Note:
The manifestations of fibromyalgia are somewhat similar to that of chronic
fatigue syndrome (also known as myalgic encephalomyelitis). In the exam:
√ If the patient has more pain and tenderness, pick → Fibromyalgia.
√ If they have a history of fatigue after a recent viral or bacterial infection,
Pick → Myalgic encephalomyelitis.
29
Polyarteritis Nodosa (PAN)
• PAN is a rare systemic vasculitis with necrotizing inflammation of the
medium-sized arteries that may be seen in childhood.
• It can present with:
Unexplained fever, maculopapular rash, subcutaneous nodules
, hypertension
and proteinuria.
• Others → Headache, myalgia, vague abdominal pain.
• The symptoms are so vague. They can be seen in other diseases. For
example, Henoch-Schonlein Purpura (HSP):
HSP → PAAN: Purpura, Arthralgia, Abdominal pain, Nephropathy “not always”
(Hematuria, Proteinuria).
So, it is rarely asked in exams and it should be picked by exclusion.
• Treatment mainly depends on corticosteroids. Others: cyclophosphamide
and azathioprine.
30
In De Quervain’s tenosynovitis:
Eickhoff’s test → The r adial styloid process becomes more painful when doing
ulnar deviation.
Example 1, A female has recently given birth presents with pain in her
thumb base. The pain is particularly felt when she picks up her baby and while
changing the diapers
31
.
→ De Quervain’s Tenosynovitis
Example 2, A female who is an avid badminton player presents to the GP
with a gradual onset of pain of her wrist especially when gripping and raising
objects.
The radial styloid process is tender and becomes more painful on
ulnar deviation. What is the most likely diagnosis?
→ De Quervain’s Tenosynovitis
32
◘ De Quervain’s disease: (= washerwoman sprain = mammy thumb = Gamer
thumb): Pain under root of thumb (Base of thumb) (tenosynovitis).
The pain is elicited on gripping things and on ulnar deviation
.
It is common in games that requires wrist gripping (eg, badminton) and is
also
common after pregnancy as repeated carrying a baby can cause repetitive stress
injury
and inflammation of extensor pollicis brevis and abductor pollicis longus.
33
Temporal Arteritis “Giant cell arteritis”
• Features:
>55 YO▐ unilateral headache ▐ pain on chewing▐ sometimes tender scalp ▐
blurry vision.
• The diagnostic modality is → Temporal artery biopsy.
• The treatment is steroids high dose prednisone (oral)
→ If asked about additional treatment, pick → (Aspirin).
If Aspirin is not given in the options, pick → (Bisphosphonates).
34
75 YO man on several medications for systolic heart failure, ankle edema,
Raynaud’s syndrome presents with severe pain in his left knee that started 3
days ago.
His left knee is swollen, tender and red. He is on losartan,
bisoprolol, nifedipine, digoxin and indapamide.
Acute Gout.
The likely Dx →
The drug that likely caused his condition is → Indapamide. (Thiazide diuretic).
Remember, Indapamide and bendroflumethiazide are (thiazide-like diuretics)
and they can cause hyperuricemia → Gout.
35
A 54 YO man on bendroflumethiazide for his hypertension presents with a
painful, red and swollen left index finger.
It happened spontaneously
without a Hx of trauma.
There is no fever. The distal interphalangeal joint is
red, swollen and warm. He had a milder but milder episode 5 years ago and
it was resolved on its own.
The likely Dx → Gout.
Remember, bendroflumethiazide (a thiazide-like diuretic) can cause
hyperuricemia → Gout.
Caution, in acute attack like in this stem, serum uric acid can be normal or
low.
Thus, it is not the appropriate investigation, but synovial fluid aspiration
and analysis.
36
A 14 years old boy presented with painful joints and rash. The pain was
started on the knees then it is now affecting ankles and elbows. The rash are
pink annular on the trunk and upper and lower limbs.
He has a history of sore
throat two weeks ago. A pansystolic murmur is heard over the apex.
Ibuprofen (a NSAID) can help with the pain.
√ The likely Dx → Rheumatic fever.
√ The most likely lab value to be abnormal is → ESR.
√ The most appropriate/ specific lab test is
→ Antistreptolysin O (ASO) antibodies.
37
Rheumatic fever
• Rheumatic fever develops following an immunological reaction to a recent
(2-6 weeks ago) Streptococcus pyogenes infection.
• Example:
A history of sore throat (strept) 2 weeks ago → then developed arthritis, rash,
pansystolic murmur (cardiac).
◙ Diagnostic criteria
Diagnosis is based on evidence of recent streptococcal infection accompanied
by:
• 2 major criteria, or:
• 1 major with 2 minor criteria
Evidence of recent streptococcal infection
• raised or rising streptococci antibodies,
• positive throat swab
• positive rapid group A streptococcal antigen test
38
Major criteria
♦Major criteria
• Erythema marginatum (pink annular rash on trunk and limbs).
• Sydenham’s chorea: this is often a late feature
• Polyarthritis (in teens with rheumatic fever, the large joints of the lower
limbs e.g., knees are the first to be affected. Then, arthritis would
normally migrate to other joints such as ankles, elbows, wrists).
• carditis and valvulitis (e.g., pancarditis) (Mitral valve regurgitation or
stenosis commonly develop especially mitral regurgitation → pansystolic
murmur).
• subcutaneous nodules
39
♦Minor criteria
♦Minor criteria
• raised ESR or CRP
• fever
• arthralgia (not if arthritis a major criteria)
• prolonged PR interval
Erythema marginatum is seen in around 10% of children with rheumatic
fever. It is rare in adults
40
Rheumatic fever
• Rheumatic fever develops following an immunological reaction to a recent
(2-6 weeks ago) Streptococcus pyogenes infection.
• Example:
A history of sore throat (strept) 2 weeks ago → then developed arthritis, rash,
pansystolic murmur (cardiac).
◙ Labs (imp.):
√ The most likely laboratory value to be abnormal is → √ The most “specific” or the most appropriate test → ESR.
ASO antibodies.
◙ Management
Outline of management
• antibiotics: oral penicillin V
• anti-inflammatories: NSAIDs are first-line
• treatment of any complications that develop e.g., heart failure
41
Raynaud's phenomenon is a condition characterized by episodic vasospasm and
ischemia of the small arteries and arterioles of the extremities, primarily
affecting the fingers and toes, but it can also involve the ears, nose, and lips.
Triggered by cold exposure or emotional stress, it leads to color changes (white
ie, pale to blue to red), numbness, pain, and tingling in the affected areas.
Types:
1. Primary Raynaud's:
o No associated medical condition.
o Common in young women, due to overactive sympathetic response.
2. Secondary Raynaud's:
o Linked to diseases like systemic sclerosis, lupus, rheumatoid arthritis.
o More severe, potential for ulcers or gangrene.
Causes include connective tissue diseases, occupational factors,
medications, and other conditions.
Pathophysiology:
• Vasoconstriction: Excessive narrowing of blood vessels particularly the small.
• Phases:
1. White -Pale- (Ischemia): Reduced blood flow.
2. Blue (Cyanosis): Lack of oxygen.
3. Red (Reperfusion): Return of blood flow, causing pain or tingling.
42
Management: raynauds
• Lifestyle:
o Keep warm, avoid cold, manage stress.
o Stop smoking.
• Medications:
o First-line: Calcium channel blockers (e.g., nifedipine).
o Alternatives: Phosphodiesterase inhibitors, ACE inhibitors (2nd line).
• Treat Underlying Conditions: Manage associated diseases.
• Severe Cases: Specialist referral for advanced therapies like intravenous
prostanoids or sympathectomy
43
44
Raynaud’s Phenomenon
Pale digits, hands – due to ischemia- → become cyanosed “bluish” when
exposed to cold -due to deoxygenation → then become red.
→ Nifedipine The only licenced medication for Raynaud’s phenomenon in the UK is
(a calcium channel blocker). (Imp PLAB 1 question).
“note the amlodipine is also a calcium channel blocker, however; it is not
licensed in the UK for Raynaud’s phenomenon.
45
Sjogren’
s syndrome
SLE, rheumatoid arthritis or other
◘ Sjogren’s syndrome is an autoimmune disorder affecting exocrine glands
resulting in dry mucosal surfaces.
◘ It may be primary (PSS) or secondary to connective tissue disorders
◘ Sjogren’s syndrome is much more common in females (ratio 9:1).
Features “important”
√ Dry eyes → keratoconjunctivitis sicca
“The patient may have itchy eyes, a sandy sensation under their eyes -due to
low lacrimal production”
♦ Schirmer’s test → ↓ tear production.
♦ Rose Bengal stain → may show Corneal ulcerations “2ry to dry eyes”.
√ Dry mouth:
“They may complain of difficulty in swallowing food -due to low saliva”
√ Recurrent Parotitis → Bilateral enlargement of Parotid glands.
46
Sjogrens
Other symptoms
vaginal dryness, arthralgia,
Raynaud’s, myalgia, sensory polyneuropathy,
renal tubular acidosis (usually subclinical)
47
Sjogrens investigation
Investigation
√ Schirmer’s test: filter paper near conjunctival sac to measure tear formation
→ decreased tear production.
√ Rose Bengal stain → may show Corneal ulcerations “2ry to dry eyes”.
√ Rheumatoid factor (RF) positive in nearly 100% of patients.
√ Anti-Ro (SSA) antibodies in 70%.
√ anti-La (SSB) antibodies in 30%.
Management
♦ No Cure.
♦ Give artificial saliva and tears (e.g. Hypromellose drops).
48
Patient with dry eyes. Schirmer’s test shows 8mm (N > 15). (+) ANA.
Management?
◙ Itchy, dry, mildly painful eyes + Reduced lacrimation (Schirmer’s test < 10 mm)
→ Keratoconjunctivitis Sicca
→ Use Artificial tears e.g. Hypromellose drops, NaCl, Sodium hyaluronate.
Any of which would be a valid answer
49
Woman with pain and stiffness of the joints of her hand. Has rash that
crosses her nose and cheeks.
Medical history significant for mouth ulcers.
The investigation of choice?
◙ The given features are suggestive of Systemic Lupus Erythematosus (SLE).
◙ Initial (Screening) test of SLE → sensitive”
Anti-nuclear antibody (ANA) “More
◙ Confirmatory → Anti-dsDNA “Specific”
♦ Remember, in SLE:
+ve ANA,
-ve ANCA, ↑ ESR
Proteinuria and hematuria might be found if there is renal involvement
(GN).
50
A 72yr old woman presents with hip and shoulder pain. Pain in the wrist.
Synovial joint thickening.
No muscle pains. Raised ESR: 80, normal CK. What
is the most definitive treatment?
In Polymyalgia Rheumatica (PMR),
• The Creatine Kinase is Normal, the ESR is >30 and CRP > 6.
(While in Polymyositis → ↑ CK).
• Polymyalgia Rheumatica also presents with aching and stiffness (while
polymyositis has weakness).
• The sites of pain and stiffness on PMR are proximal muscles: Neck, shoulder,
upper arm, pelvic girdle “
difficulty to get out of bed, or to get up from a
seated position, or to raise her arm above her head.
• 50% of patients with PMR have Temporal arteritis (>55 YO, Headache,
Painful jaw especially on chewing).
• Initial tests → ESR
• Rx → Prednisolone.
51
24 YO boy was able to walk with support when he was younger. However,
he is unable to walk now. What is the next step?
→ Request CK
He was able to walk earlier, thus, not a developmental milestone issue.
We suspect polymyositis → order Creatine Kinase.
52
GCA = Temporal Arteritis
Investigations
• Initial test → ↑↑ ESR. CRP may also be elevated
• To Dx “confirmative” → present
[Note that creatine kinase and EMG normal]
Treatment “important √”
◘ High-dose prednisolone – there should be a dramatic response, if not, the
diagnosis should be reconsidered.
◘ Added-on medication → low dose Aspirin
“Remember, in Kawasaki, which has a febrile vasculitis seen in children < 5
YO,
we give High dose Aspirin to “avoid coronary artery aneurysm”.
53
◘ Another possible Added-on medication → Especially in elderly. As they receive high doses of prednisolone
(corticosteroids), this would cause osteopenia, osteoporosis. Thus, a use of
bisphosphonate may help prevent this
.
◘ Urgent ophthalmology review. Patients with visual symptoms should be
seen the same-day by an ophthalmologist.
Visual damage is often irreversible
(Blindness is a feared complication in Temporal arteritis).
To sum up
A patient presents with temporal arteritis “Giant cell arteritis”
(>55 YO, usually unilateral headache and pain on chewing, sometimes
tender scalp and blurry vision)
→ the treatment is (Prednisolone). If asked about additional treatment, pick
(Aspirin).
If not give in the choices, pick (Bisphosphonates).
54
78 yr old lady with unilateral headache and pain on chewing (i.e. giant cell
arteritis) on prednisolone 60mg and omeprazole.
ESR 70. What additional
medication to add?
bisphosphonates
55
60 YO man presents with unilateral headache and pain on chewing,
sometimes tender scalp and blurry vision.
◙ The most appropriate investigation → Temporal artery biopsy.
◙ The treatment is steroids (Prednisolone).
√ If asked about additional treatment, pick (Aspirin).
Likely Giant Cell Arteritis (Temporal Arteritis)
56
y
16
A hypertensive patient presents with big toe severe pain, swelling and
erythema.
Rx → Naproxen.
He is likely on bendroflumethiazide for his HTN. Thiazide like diuretics and
loop diuretics can cause Gout
√ If the patient is presenting with acute attack “important”:
→ First line → ▐ 2nd → NSAIDs (e.g. Ibuprofen, Naproxen) Colchicine.
√ For long-term (after 2 weeks of acute attack)
“with NSAIDs and Colchicine coverage”
57
Erythema nodosum, (Tender, red nodules over shins).
Bilateral hilar lymphadenopathy, (the most common finding on Chest X ray)
Polyarthralgia
Hypercalcaemia, Fever.
Please, whenever you see 2 of these features, consider “Sarcoidosis”.
Syndromes associated with sarcoidosis
Lofgren’s syndrome is an acute form of the disease characterised by
bilateral hilar lymphadenopathy (BHL), erythema nodosum,
fever and
polyarthralgia.
It usually carries an excellent prognosis.
58
Syndromes associated with sarcoidosis
Mikulicz syndrome: there is associated enlargement of the parotid and
lacrimal glands → Dry mouth, face swelling. Due to sarcoidosis, tuberculosis or
lymphoma
Heerfordt’s syndrome (uveoparotid fever)
there is parotid enlargement, fever
and uveitis secondary to sarcoidosis
59
Remember, in acute gout:
→ First line → NSAIDs (e.g. Ibuprofen, Naproxen) ▐
2nd → Colchicine.
So, if the options have both (Naproxen and Colchicine), pick Naproxen, which
is an NSAID
60
√ Cannot see → Conjunctivitis, Uveitis.
√ Cannot pee → Urethritis.
√ Cannot climb a tree → Arthritis. (pain and swelling of small joints)
+ Skin manifestations → Rash (Maculopapular
Reactive arthritis
61
Young man
Low back pain +
Morning Stiffness +
Uveitis “painful eye” ±
Tenderness of Sacro-iliac joint “X-ray of this joint is the most helpful
”
Dx → Ankylosing Spondylitis “a type of Seronegative Spondyloarthropathy”
Rx → NSAIDs (first line)
▐ Anti-TNF therapy (second line)
To Diagnose → X-ray of Sacroiliac joint. “important” √
Other Points:
√ Back pain is worse in the morning and improves on exercise.
HLA-B27 √ Ankylosing Spondylitis has a strong association with but we
CANNOT use it for diagnosis as it is Positive in 10% of Normal people.
62
Rheumatoid Arthritis
Typical features
◘ Swollen, painful joints in hands and feet.
◘ Stiffness worse in the morning.
◘ Gradually gets worse with larger joints becoming involved.
◘ Presentation usually insidiously develops over a few months.
◘ Positive ‘squeeze test’
metatarsal joints.
– discomfort on squeezing across the metacarpal or
Management is beyond PLAB scope. However, remember these:
√ NICE updated their rheumatoid arthritis guidelines. They now recommend
DMARD monotherapy (e.g. methotrexate) ± a short-course of bridging
prednisolone.
In the past dual DMARD therapy was advocated as the initial step
√ Please, know that NSAIDs (e.g. ibuprofen) can be used to manage severe joints
pain in RA
63
64
65
66
67
Wegeners
Granulomatosis
(Granulomatosis
with Polyangiitis)
Upper Respiratory problems
(Sinusitis/ Nasal septum
perforation/ Epistaxis /
nasal crusting)
+ Hematuria / Hemoptysis
important to know
Bleeding: nose, lung, kidney
c-ANCA
68
Churg Strauss
(Eosinophilic
Granulomatosis
with Polyangiitis)
Asthma, Nasal polyps, Allergic
rhinitis, Eosinophilia
+ Other organ
e.g. purpura, Glomerulonephritis, HF,
Skin nodules.
CT → Ground-glass
attenuation
Eosinophilia
↑ ESR, CRP, IgE
p-ANCA [√]
69
Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disorder.
It
typically presents in early adulthood and is more common in women and
people of Afro-Caribbean origin.
General features
• Fatigue, fever, lymphadenopathy
• Mouth ulcers (large, multiple, painful)
• Remitting and relapsing illness
Skin
Malar (butterfly) rash: spares nasolabial folds
• discoid rash: scaly, erythematous, well demarcated rash
in sun-exposed areas. Lesions may progress to become
pigmented and hyperkeratotic before becoming atrophic
• Photosensitivity
Raynaud’s phenomenon (1/5th of the patients but often
mild)
Musculoskeletal
• Arthralgia
• Non-erosive arthritis
Cardiovascular
• pericarditis: the most common cardiac manifestation
• myocarditis
Respiratory
• pleurisy
• fibrosing alveolitis
Renal
• Proteinuria
• glomerulonephritis (diffuse proliferative
glomerulonephritis is the most common type)
Neuropsychiatric
anxiety and depression
• psychosis
• seizures
70
◙ The initial tests of SLE: ANA “Anti-nuclear antibody” (More sensitive)
◙ Confirmatory → Anti-dsDNA, ESR, Complement C3 and C4
In SLE
+ve ANA,
-ve ANCA, ↑ ESR
Proteinuria and hematuria might be found if there is renal involvement
(GN).
Painful joints and morning stiffness can also be seen.
“it is not always about rash
71
Anti-histone antibodies → Drug-induced lupus.
(e.g. due to hydralazine used for HF along with isosorbide dinitrate)
Others: Raised ESR, Normochromic Normocytic Anemia, low C3 and C4.
Monitoring
• ESR: during active disease, the CRP is characteristically normal. A raised
CRP may indicate underlying infection.
• complement levels (C3, C4) are low during active disease
• (formation of complexes leads to consumption of complement)
• anti-dsDNA titres can be used for disease monitoring (but note not present
in all patients)
72
Chronic fatigue syndrome.
Diagnosed after at least 4 months of disabling fatigue affecting mental and
physical function more than 50% of the time in the absence of other disease
which may explain symptoms
Epidemiology
more common in females
past psychiatric history has not been shown to be a risk factor
◙ Fatigue is the central feature,
Other recognised features include:
√ Sleep problems, such as insomnia, hypersomnia, unrefreshing sleep, a
disturbed sleep-wake cycle
√ Muscle and/or joint pains
√ Headaches
√ painful lymph nodes without enlargement
√ sore throat
√ cognitive dysfunction, such as difficulty thinking, inability to concentrate,
impairment of short-term memory, and difficulties with word-finding
√general malaise or ‘flu-like’ symptoms
√ dizziness √ nausea palpitations
73
74
75
Investigation of cfs
NICE guidelines suggest carrying out a large number of screening blood tests
to exclude other pathology e.g.
FBC, U&E, LFT, glucose, TFT, ESR, CRP, calcium,
CK, ferritin, coeliac screening and also urinalysis
Management
√ Cognitive behaviour therapy – very effective.
√ Graded exercise therapy.
◙ Fatigue is the central feature,
Other recognised features include:
√ Sleep problems, such as insomnia, hypersomnia, unrefreshing sleep, a
disturbed sleep-wake cycle
√ Muscle and/or joint pains
√ Headaches
√ painful lymph nodes without enlargement
√ sore throat
√ cognitive dysfunction, such as difficulty thinking, inability to concentrate,
impairment of short-term memory, and difficulties with word-finding
√general malaise or ‘flu-like’ symptoms
√ dizziness √ nausea palpitations
76
26 YO female presents complaining of easy fatiguability even with little
exertion, recurrent headaches, difficulty sleeping, difficulty concentrating, and
body aches everywhere.
These symptoms have been persistent for the last 5
months.
She says that these symptoms began after a viral infection 5 months
ago. FBC, KFT, LFT, TFT, Glucose, CRP,
Urinalysis, all have been done and
returned with normal ranges.
The likely Dx → Chronic Fatigue Syndrome.
77
Myopathies
Features
√ Symmetrical muscle weakness (proximal > distal).
√ common problems are: rising from chair, combing hair, getting out of bath
(Proximal muscle weakness).
√ Sensation and reflexes are normal, no fasciculation.
Causes
• Inflammatory: Polymyositis
• Inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
• Endocrine: Cushing’s, thyrotoxicosis
• Alcohol
78
Polymyositis
√ Symmetrical and diffuse muscle weakness (proximal > distal) involving the
proximal muscles of neck, shoulders, trunk, hips, thighs, LL (affected earlier).
√ common problems are: rising from a seated position, combing hair, getting
out of bath, lifting objects, climbing stairs.
√ (↑) Creatine Kinase.
√ Anti-jo 1 autoantibodies.
√ To Dx → Muscle biopsy.
79
Important differentiating point,
In Polymyalgia Rheumatica (PMR),
• The Creatine Kinase is Normal, the ESR is >30 and CRP > 6.
(While in Polymyositis → ↑ CK).
• Polymyalgia Rheumatica also presents with aching and stiffness (while
polymyositis has weakness).
• The sites of pain and stiffness on PMR are proximal muscles:
Neck, shoulder,
upper arm, pelvic girdle “difficulty to get out of bed,
or to get up from a
seated position, or to raise her arm above her head.
• 50% of patients with PMR have Temporal arteritis (>55 YO, Headache,
Painful jaw especially on chewing).
• Initial tests → ESR
• Rx → Prednisolone.
80
So, in a patient with proximal muscle weakness “e.g. difficulty climbing stairs
or getting up from a seated position + ↑ CK
→ suspect polymyositis.
81
So, in a patient with proximal muscle weakness “e.g. difficulty climbing stairs
or getting up from a seated position + ↑ CK
Polymyositis
82
Sjogren’s Syndrome
SLE, rheumatoid arthritis or other
◘ Sjogren’s syndrome is an autoimmune disorder affecting exocrine glands
resulting in dry mucosal surfaces.
◘ It may be primary (PSS) or secondary to connective tissue disorders
◘ Sjogren’s syndrome is much more common in females (ratio 9:1).
Investigation
√ Schirmer’s test: filter paper near conjunctival sac to measure tear formation
→ decreased tear production.
√ Rose Bengal stain → may show Corneal ulcerations “2ry to dry eyes”.
√ Rheumatoid factor (RF) positive in nearly 100% of patients.
√ Anti-Ro (SSA) antibodies in 70%.
√ anti-La (SSB) antibodies in 30%.
Management
♦ No Cure.
♦ Give artificial saliva and tears (e.g. Hypromellose drops).
83
Sjogren’s Syndrome
SLE, rheumatoid arthritis or other
◘ Sjogren’s syndrome is an autoimmune disorder affecting exocrine glands
resulting in dry mucosal surfaces.
◘ It may be primary (PSS) or secondary to connective tissue disorders
◘ Sjogren’s syndrome is much more common in females (ratio 9:1).
Features “important”
√ Dry eyes → keratoconjunctivitis sicca
“The patient may have itchy eyes, a sandy sensation under their eyes -due to
low lacrimal production”
♦ Schirmer’s test → ↓ tear production.
♦ Rose Bengal stain → may show Corneal ulcerations “2ry to dry eyes”.
√ Dry mouth:
“They may complain of difficulty in swallowing food -due to low saliva”
√ Recurrent Parotitis → Bilateral enlargement of Parotid glands.
√ Others:
vaginal dryness,
arthralgia,
Raynaud’s, myalgia,
sensory polyneuropathy,
renal tubular acidosis (usually subclinical)
84
85
A 45 YO ♀ presents complaining of a sandy feeling under her eyes and
difficulty in swallowing for the last year.
She also has bilateral parotid
enlargement.
The likely Dx → Sjogren’s Syndrome.
♦ instead of mentioning “dry eyes”, the stem says “sandy feeling under
her eyes”, which results from low tear production.
♦ Instead of saying “dry mouth”, the stem says “difficulty in swallowing”
which occurs 2ry to decreased saliva.
86
A 55 YO ♀ with Hx of SLE presents complaining of dry eyes and altered
taste sensation
. Her voice becomes hoarse when she speaks for long-
time.
The likely Dx → Sjogren’s Syndrome.
√ Please note that Sjogren syndrome can occur primary or secondary to
connective tissue diseases such as SLE and RA.
√ Here, the altered sense of taste and hoarseness are due to low saliva
production which is seen along with dry eyes in Sjogren syndrome.
87
Systemic sclerosis (Scleroderma)
Systemic sclerosis is a condition of unknown aetiology characterised by
hardened, sclerotic skin and other connective tissues. It is four times more
common in females
Raynaud’s Phenomenon: “√”
Pale digits, hands – due to ischemia-
→ become cyanosed “bluish”
when
exposed to cold -due to deoxygenation
→ then become red.
→ Nifedipine
The only licenced medication for Raynaud’s phenomenon in the UK is
(a calcium channel blocker). (Imp question).
“Note the amlodipine is also a calcium channel blocker, however; it is not
licensed in the UK for Raynaud’s phenomenon.
88
“Limited” Scleroderma [CREST Syndrome]
So, Raynaud’s phenomenon develops firstly, then, slow progression (up to
years) → them,
Other CREST may be seen (e.g. small pink, red spots on lips,
fingers. This is Telangiectasia)
• Scleroderma affects face and distal limbs predominately “thick skin”.
• Raynaud’s phenomenon may be first sign
• Slow onset and Slow progression.
• associated with anti-centromere antibodies
• a subtype is CREST syndrome: Calcinosis, Raynaud’s phenomenon,
Esophogeal dysmotility, Sclerodactyly, Telangiectasia
• Scl-70 antibodies (-ve)
• ANA is mostly +ve.
89
Diffuse” Scleroderma
• Scleroderma affects trunk and proximal limbs predominately
• Rapid onset and Fast progression.
• The most common cause of death is now respiratory involvement, which is
seen in around 80%: interstitial lung disease (ILD) and pulmonary arterial
hypertension (PAH)
• Other complications include renal disease and hypertension
• Poor prognosis.
• Scl-70 antibodies (+ve)
• ANA is mostly +ve.
90
44 YO ♀ has pallor of her hands followed by bluish discoloration after
exposure to cold.
This condition has been persistent for 3 years. She also has
symmetrical peripheral arthropathy.
Now, she develops small pink and red
spots on her lips and fingertips.
The likely Dx → Limited Systemic Sclerosis (CREST Syndrome)
91
A 46 YO ♀ has pallor of her hands followed by bluish discoloration after
exposure to cold.
She also has shortness of breath on walking and dysphagia.
The likely Dx → Systemic Sclerosis
Features of CREST (Raynaud’s), (Esophageal dysmotility)
92
Temporal Arteritis [= Giant Cell Arteritis
].
• Temporal arteritis is large vessel vasculitis.
• It is sometimes associated with polymyalgia rheumatica (PMR).
• Histology shows changes which characteristically ‘skips’ certain sections of
affected artery whilst damaging others
93
Investigations temporal arteritis
• Initial test → elevated esr and crp
• To Dx “confirmative” → temporal artery biopsy -skip lesions
present
[Note that creatine kinase and EMG normal]
Features
√ > 55 YO
√ Usually rapid onset (e.g. < 1 month)
√ Headache
√ Visual disturbances (Blurry vision).
√ Jaw claudication (65%) “Pain on Chewing”
√ Scalp Tenderness.
√ Around 50% have features of Polymyalgia Rheumatica
aching,
morning stiffness in proximal limb muscles (not weakness
94
Temporal arteritis
Especially in elderly. As they receive high doses of prednisolone
(corticosteroids), this would cause osteopenia, osteoporosis. Thus, a use of
bisphosphonate may help prevent this.
◘ Urgent ophthalmology review. Patients with visual symptoms should be
seen the same-day by an ophthalmologist. Visual damage is often irreversible
(Blindness is a feared complication in Temporal arteritis).
Treatment “important √”
◘ High-dose prednisolone – there should be a dramatic response, if not, the
diagnosis should be reconsidered.
◘ Added-on medication → low dose Aspirin
“Remember, in Kawasaki, which has a febrile vasculitis seen in children < 5
YO, we give High dose Aspirin to “avoid coronary artery aneurysm”.
◘ Another possible Added-on medication → Bisphosphonates
95
96
Gca summary
To sum up
A patient presents with temporal arteritis “Giant cell arteritis”
(>55 YO, usually unilateral headache and pain on chewing, sometimes
tender scalp and blurry vision)
→ the treatment is (Prednisolone). If asked about additional treatment, pick
(Aspirin).
If not give in the choices, pick (Bisphosphonates)
97
In Polymyalgia Rheumatica (PMR
),
• The Creatine Kinase is Normal, the ESR is >30 and CRP > 6.
(While in Polymyositis → ↑ CK).
• Polymyalgia Rheumatica also presents with aching and stiffness (while
polymyositis has weakness).
• The sites of pain and stiffness on PMR are proximal muscles: Neck, shoulder,
upper arm, pelvic girdle “
difficulty to get out of bed, or to get up from a
seated position, or to raise her arm above her head.
• 50% of patients with PMR have Temporal arteritis (>55 YO, Headache,
Painful jaw especially on chewing).
• Initial tests → ESR
• Rx → Prednisolone
98
A 58 YO man presents with a 4-day Hx of right sided headache with blurry
vision. He has pain around his jaw that worsens with chewing.
◙ The likely Dx → GCA
◙ Initial Test → Esr
◙ The confirmative test → temporal artery biopsy
◙ The Rx → high dose oral steroids (prednisolone)
◙ If the patient was already commenced on Prednisolone, what other
medication can help in his case? → aspirin
“↓ stroke and visual loss”
99
Example (2),
55 YO ♀, feeling stiffness of neck, shoulders, hips in the morning over the past
2 months.
Difficulty to get out of bed, and out of her chair.
A few days ago,
she developed a pain in her left jaw especially on chewing and a headache.
Her left scalp is tender.
◙ The likely Dx → Polymyalgia Rheumatica associated with Temporal
arteritis.
◙ The Rx → Corticosteroids “High-dose prednisolone”
Important, “√”
The most appropriate management is → Prednisolone.
The most appropriate “initial” investigation → ESR
The most appropriate (or definitive) investigation → Temporal artery biopsy.
Temporal Arteritis is → Arterial inflammation (not infection nor aneurysm
100
Important Points on Gout (Gouty Arthritis): ↑ Uric Acid
◙ The main features of gout
√ Pain: this is often very significant.
√ Swelling.
√ Erythema & warmth.
√ Recurrent episodes/ attacks of similar joint inflammation may occur.
√ Presents acutely “sudden”: pain and swelling develop over a short duration.
◙ Around 70% of first presentations affect:
→ The first metatarsophalangeal joint (of the big toe).
Other commonly affected joints include: ankle, wrist, knee.
This means that gout can affect any site in hands and feet eg, DIP joint.
◙ Important Exam Hints:
○ An antihypertensive and diuretic medication “Thiazide Like Diuretic” such as
bendroflumethiazide can cause Gout.
○ A diuretic used in Heart Failure → Loop diuretic (Furosemide) can cause
Gout.
○ Also, Drinking Too Much Alcohol can cause Gout!
○ X-ray of the painful red swollen joint
→ Well-defined ‘punched-out’ erosions with sclerotic margins.
101
Risk factors
Investigations of gout
◙ Investigations of gout → Important → If there is no fever, send joint aspirate for “microscopy” not for
culture.
“Caution! Do not pick (Serum Uric Acid) as it is usually normal or low in the
acute stage! It can be measured 4-6 weeks after the acute stage has been
passed”.
So, in acute attack, Serum uric acid is not the answer as an investigation!
◙ In short: Some Important Risk Factors of Gout
→ Thiazide-like diuretics
▐ Loop diuretics: Furosemide
▐ Excessive alcohol
intake.
102
.
◙ Radiological features of gout:
• Joint effusion is (early sign).
• Well-defined ‘punched-out’ erosions with sclerotic margins in a juxta-
articular distribution, often with overhanging edges
• Relative preservation of joint space until late disease
• Eccentric erosions
• No periarticular osteopenia (in contrast to rheumatoid arthritis)
• Soft tissue tophi may be seen
◙ Management of gout:
√ If the patient is presenting with → First line → acute attack NSAIDs (eg, Ibuprofen, Naproxen) “
important”:
▐ 2nd → Colchicine.
103
104
Caution:
Gout does not only affect the big toe; it can also affect Knees, interphalangeal
joints and other joints.
Whenever you see “bendroflumethiazide” or another
RF as heavy alcohol along with painful, tender, swollen joint
:
Think → Gout → Synovial fluid aspiration.
→ NSAIDs or Colchicine for acute attack.
Also, the stem may not mention any of the RFs.
105
106
107
Septic Arthritis
Important Topic! Every single detail can be a question.
Please, remember that Rheumatoid arthritis is an important risk factor.
It is
usually given in a stem as a hint with other features of septic arthritis.
Monoarthritis = Single joint involvement (commonly Knee)
Fever/ Pain/ Swelling/ Limited movement/ Hotness
+ A Risk factor
(e.g. DM,
Steroid,
HIV,
Rheumatoid Arthritis) “
important √”
Think of → Septic Arthritis
Commonly affect Knee, but can affect others especially Shoulder
Staphylococcus Aureus.
♦ The commonest causative organism →
♦ A common organism in young SEXUALLY active → N. Gonorrhea.
108
Septic arthritis mng investigation
Dx
√ Aspiration of Synovial Fluid for culture and sensitivity √
√ Blood Culture.
Management
√ Flucloxacillin (for 4-6 weeks) “first-line” “like cellulitis”
should be started empirically before the synovial culture results.
√ If penicillin allergic → Clindamycin.
√ If the causative organism is N. Gonorrhea nor Staph → Cefotaxime or
Ceftriaxone.
√ If still not responding → Repeated percutaneous aspiration.
“IV antibiotics for 1 week until blood cultures become -ve and swelling resolves
Then, Oral antibiotics for 4 weeks”
◙ Note, do not forget (DM, RA) as risk factors for Septic Arthritis. “Hints”
109
Reactive arthritis: Seronegative Spondyloarthritis
(Migratory Oligoarthritis of lower limbs + Back pain + Extraarticular features)
• Typically, there is no fever.
• Typically, seen in young adults.
• Typically follows Urogenital infection “STI” or GI infection “dysenteric
illness”.
• Asymmetric, Migratory Oligoarthritis of LL (Knees and Ankles).
• Extraarticular features: (Reiter’s Triad)
√ Cannot see → Conjunctivitis, Uveitis.
√ Cannot pee → Urethritis.
√ Cannot climb a tree → Arthritis. (pain and swelling of small joints)
+ Skin manifestations → Rash (Maculopapular)
circinate balanitis (painless vesicles on the coronal margin of the prepuce),
keratoderma blenorrhagica (waxy yellow/brown papules on palms and soles)
Erythema nodosum (Tender, red nodules over shins).
110
Reactive arthritis: Seronegative Spondyloarthritis
Mng
Management
♦ Symptomatic: analgesia, NSAIDS, intra-articular steroids
♦ Sulfasalazine and methotrexate are sometimes used for persistent disease
♦ Symptoms rarely last more than 12 months
111
◘ De Quervain’s disease: (= washerwoman sprain = mammy thumb = Gamer
thumb): Pain under root of thumb (Base of thumb) (tenosynovitis)
The pain is elicited on gripping things and on ulnar deviation.
It is common in games that requires wrist gripping (eg, badminton) and is also
common after pregnancy as repeated carrying a baby can cause repetitive stress
injury and inflammation of extensor pollicis brevis and abductor pollicis longus.
112
√ In De Quervain’s tenosynovitis:
Eickhoff’s test → The radial styloid process becomes more painful when doing
ulnar deviation.
113
Lateral epicondylitis
◘ Tennis elbow = lateral epicondylitis → affected wrist extension. Mainly due to
overuse e.g., in tennis players
114
Medial epicondylitis
◘ Golfer’s Elbow = Medial epicondylitis: all flexors to fingers and pronator are
affected.
Seen in baseball players, construction injury, plumber injury.
115
Dupuytren’s contracture
• a condition in which there is a fixed forward curvature of one or more
fingers,
caused by the development of a fibrous connection between
the finger tendons and the skin of the palm.
• Dupuytren’s contracture has a prevalence of about 5%.
• It is more common in older male patients.
• 60-70% have a positive family history.
• Specific causes include→
Manual labour
▐ phenytoin treatment
▐alcoholic liver disease
▐ trauma to the hand
▐ DM
▐ Smoking
• Mechanism
→ Formation of thickened fibrous tissue within the palmar fascia.
• Rx → Fasciotomy
116
Trigger Finger = (Stenosing Tenosynovitis)
◙ More common in the thumb, middle, or ring finger.
◙ Stiffness of a finger, and snapping (click) sound when extending a flexed
digit.
◙ A nodule may be felt at the base of the affected finger.
117
Example 1, A female has recently given birth presents with pain in her
thumb base.
The pain is particularly felt when she picks up her baby and while
changing the diapers.
→ De Quervain’s Tenosynovitis
118
Example 2, A female who is an avid badminton player presents to the GP
with a gradual onset of pain of her wrist especially when gripping and raising
objects.
The radial styloid process is tender and becomes more painful on
ulnar deviation. What is the most likely diagnosis?
→ De Quervain’s Tenosynovitis
119
Example 2, A female who is an avid badminton player presents to the GP
with a gradual onset of pain of her wrist especially when gripping and raising
objects. The radial styloid process is tender and becomes more painful on
ulnar deviation. What is the most likely diagnosis?
120
