Rheumatology SMLE review

Dr Ismail Azhari
Founder :CCR Academy
|| .. Systemic lupus erythematosus .. | |
Clinical features All systems affected

Au toimmu ne diseases: Au to-Antibody or immu ne C omplex

Arthritis 。 Joint pain , arthralgia

Ray naud’s phe nome non 。 Vasoconstriction in Smaller artiories finger tips WBR , wight pale, blue, red
Cased by stress or cold weather

Skin 。
Kidney 。
Lup seeo hr i : igh rena st o r U ri ne • Sx of flare diseases:- fever, weight
(Fornt h uri
t sne)h H matouria
l re l b io psy proteinuria
analysis:
y e a loss or mild lymphadenopathy

Lung 。 Interstitial lung disease, fi brosis, pleural eff usion

• Constant Sx:- fatigue, malaise or

。。
Life Therating , Neuropsychatic symptoms:
seizure,comma, delusions fibromyalgia like symptoms
Ne urological
Hematological hematolyic) Or
Lo w CBC
anemia ofSp ecifically
chronic lymp
disease orho p nea,
iron anemia
deficiency ([Link]
anemia

Gastrointestinal 。
All type of anemia

Colitis,
 Skin manifestations

Rash precipitated by UV light 。 Nonpharam: Avoid sun exposure, put sun block

3 types:- 。
1 - B ut t er fl y facial ras h (er yt hemat o us , rais ed , p ainful o r it ching , over
che e ks and sparing nasolabial fold), photose nsitivity
2- Subacute cutaneous lupus erythematosus (SCLE)
3- Discoid rash
Skin Levido reticularis
manifestations

SCLE butterf ly facial rash Discoid rash

 Cardiovascular manifestations

Pericarditis (most common) 。 Chest pain relieved by forward, ECG diffuse ST elevation

Myocarditis 。
Libman-sacks endocarditis (due to accumulation of sterile fibrin 。
conta ining ve ge ta tion on he a r t va lve s)  se a rch for A PS

Risk of atherosclerosis and MI increased! 。

Most com cau se of death in SLE is cardiovascular disease
Criteria for diagnosis

Mos t s e ns it ive 95% t o r ole ou t s o if ne ga t ive c a n e xc lude dis e as e

Spe c ific , f ollow u p dis e a s e s eve rit y

Mo s t s p e c ifi c t e s t , ‫ﺑﺲ وﺣﺪة ﻣﺮه ﺑﻠﻄﻨﻲ‬

Dis e a s e s e ve r it y, if a c t ive dis e a s e = low c omple me nt

Dis e as e s eve rit y

Updated criteria for diagnosis!
 Investigations

Anti-nuclear antibody (ANA) is nearly 95% sensitive!

-ve ANA exclude SLE!!

Anti-smith antibody is the most specific!

Anti-dsDNA is specific and measuring disease severity as well

 Investigations
Heart block baby
Anti- Ro ( SSA) and Anti- La ( SSB)  + ve in neonatal lupus and SCLE
S cre e n p re g n a n t w om a n w it h t h e se a n t ibodie s

Antiphospholipid antibody:-
- Lupus a nti-coagulant
- An ti -ca rd i o l i p i n
- A nt i B 2 g lyc o p r o t e in

Antiphospholipid sy ndrome = History of miscarriage s + arte rial and venous

thromboembolism, treatment is life-long Warfarin
 Investigations

How to measure disease severity?

- Anti-dsDNA
- Low comple me nt C3 and C4 (e spe cially in lupus ne phritis)
- High ESR
- L e ucope nia
- Ly m phope nia
- T h rom b ocy t ope n ia
- H e m oly t ic a ne m ia

CRP is normal in active SLE, if high search for co-existing infection

 SLE Antibodies …

Antibody … Significance
ANA Screening of lupus
Anti-smith Most specific test
Anti- dsDNA Specific and assessing disease activity
Lupus anti-coagulant, Anti-cardiolipin, Anti B2 glycoprotein Ant iphospholipid syndrome
Anti-Ro (SSA) and Anti-La (SSB) Neonatal lupus
Complements C3 and C4 Disease activity and lupus nephritis
Anti-histone Drug induced lupus
Antineuronal, anti-NMDA receptor, Antiribosomal P neuropsychiatric SLE
 Kidney manifestations

One of the main determinants of prognosis 。

Proliferative glomerulonephritis (Heavy hematuria, proteinuria, cast) 。
Kidney biopsy is mandatory! And it is the next step in lupus patient 。
w ith prote inuria and low C3, C4

Regular monitoring of BP and urinalysis is essential 。

Anti-phospholipid and pregnancy?? Very important

Just +ve APLS profile  Low dose Aspirin

+ve APLS profile + pregnancy loss  Low dose Aspirin + Prophylactic LMWH

+ve APLS profile + DVT  Low dose Aspirin + Therapeutic LMWH

Have indication for therapeutic
 Management (Pharmacological)

Hyroxychloroquine is the cornerstone

Malar rash
M ild to m ode ra te dise a se (cute nous, joint involve me nt):-
NSAIDs + Hyroxychloroquine
If not response to NSAID
Low dose cortecosteroid may be used in combination with
immunosuppre ssant (Me thotre xate , A zathioprine or My cophe nolate
mofe til)
 Management (Pharmacological)

 Pha rm a cologica l:-

L ife -thre ate ning dise ase (re nal, CNS, cardiac
High dose
involve corticosteroid + immunosuppressant
me nt):-
(M e t h y lpre dn isolon e + C y clophospha m ide )

N. B:- C y clophospha m ide ca use he m orrha gic cy st it is (av oide d by good

hy dra t ion a nd m e sna ), a zoospe rm ia a nd a nov ula t ion (ne e ds pre -
t r eat m ent o va and s p er m co llect io n and s t o rag e)
 During pregnancy

Safe drugs: - Contraindicate drugs: -

Glucocorticoids Methotrexate
Hydroxychloroquine Leflunamide
Azathioprine Cyclophosphamide
Cyclosporine Mycophenolate Mofetil
Sulphasalazine
Tacrolimus Belimumab and Rituximab
IVIG (no data)
Please remember this:-

Drug induced lupus (Hydralazine , procainamide and

isoniazid, Diltiazem, quinidine) = Anti-histone anti
body

Drug induced lupus is differ than drug exacerbate

lupus flares like for example Trimethoprim-
Sulphamethaxazole
Grades of lupus nephritis:-

Supportive

Steroid

Immunosuppressive

Immunosuppressive

Immunosuppressive
(MMF is the best)
Lupus in 1 slide

- Sy mptoms:- malar rash, discoid rash, alope cia, arthralgia, Ray naud’s,
pe ricarditis, L ibman-sack e ndocarditis, mouth ulce r, Ne urological or
h e m a t o lo g ica l

- Laboratory:- -ve ANA exclude it, Anti-smith is most specific, Anti-

dsDNA (specific also), low complement C3 and C4, high ESR all
indicate dise ase seve rity

- Treatment:- Hydroxychloroquine + NSAIDs for mild disease, high dose

ste roid + immunosuppressive for life-threatening diseases
 RA
= should be pt on Hyroxychloroquine

Norm al in SLE , RA for disease severity

Scleroderm a Opitm al answer is : NSA ID s + Hyroxychloroquine If no

NA SID choice steroid

M ild to m oderate

High reticulocyte count, high LD H, elevated unconjugated bilirubin, and decreased Haptoglobin,
high reticulocyte

Low ferritin

High ferritin

So she had life threatening

Red m an syndrom e

D rug m ake disease worse

, Steroid ‫ﺳﺐ ﻣﺎ ﻓ ﻲ ﻫﻨﺎ‬

‫ﻓ ﺎر و ح ﻟ ﻠ ﺨ ﻄ ﻮ ة ا ﻟ ﻠ ﻲ ﺑ ﻌ ﺪﻫﺎ‬
Renal Lupus nephritis = life thertening so start steroids and
im m unosuppressant ( m ycophenolate or C yclophospham ide)
|| .. Mono-arthritis .. | |
 Acute Monoarthritis

Sudden pain and swelling in a single joint 。

First step is always joint aspiration 。
Always rule out SEPTIC ARTHRITIS 。
 Causes

Common:- 。
1- Septic arthritis Gradually progressive

2- Gout
3- Pseudogout
4- Reactive arthritis
5- Trauma
6- Haemoarthrosis
o
7- Serone g at iv e s po ndyl arthr i t is
(psoriatic arthritis,
sp ondyliti s, e nt e ropathic ankylosing
arthri t is) Persoinal or family hx of paoriasis and pitting nilas
 Causes

Common:- 。
1- Septic arthritis
2- Gout
3- Pseudogout
4- Reactive arthritis
5- Trauma
6- Haemoarthrosis
7- Seronegative spondyloarthritis (psoriatic arthritis, ankylosing
spondylitis, enteropathic arthritis)
 Hints to diagnose …

Septic arthritis: redness, tenderness, hotness, slower onset and progressive

Go ut: co mmo n in middle age men, affect 1 st MTP jo int, very rapid o nset (6- 12
ho urs), hypertensive patient taking hydrochlorothiazide

Pseudo go ut: co mmo n i n o l der wo men, affect wri st and sho ul der, very rapi d o nset

Bo th go ut and ps eudo go ut i s tri ggered by i ntercurrent i l l ness, dehydrati o n o r 

surgery
Causes

Reactive arthritis: common in young men, recent sexual contact, Hx o f

GI o r GU i nfecti o n (di arrhea caus ed by C ampyl o bacter j euj eni o r
chl amydi a), o ral ul cer, co nj uncti vi ti s
Can no t bee, Can no t SE E , Can not climb a TREE

Haemo arthro sis: large effusion, absence of periarticular swelling or

s k i n c hange, H x o f trauma o r haemo phi l i a
 Investigations
Aspiration
Send fluid for Culture, Crystal (microscopy) and Gram stain
WBC > 50,000  septic arthritis
-ve birefringent crystal  gout

+ve birefringent crystal  pseudogout

Blood culture, ESR, CRP

Serum uric acid is USELESS

Management

Treat the underlying cause 

Start IV antibiotic if there is any suspicion of sepsis

Focus on management of SEPTIC ARTHRITIS, GOUT and PSEUDOGOUT …

Septic ar thritis (most common organism is S. aureus) >> start empirical Abx with Vancomycin

While culture is pending, if result came MRSA, complete Vancomycin

If result came MSSA, stop Vancomycin and give Oxacillin or floxacillin

IF THE PATIENT IS SEXUALLY ACTIVE, give CEFTRIAXONE (risk of having gonorrhea)

G out a nd pseudogout:- treat w ith NSAIDs or Colchicine (Indomethacin is preferred over colchicine)

BOTH CAN NOT BE USED IN RENAL FAILURE >> give Corticosteroid instead

ALLOPURINOL is HARMFUL during acute attack

|| .. Polyarthritis .. | |
 Polyarthritis

Pain and sw e lling affe cting 5 or more joint or joint groups 。

Infla m m a tory v s non-inflammatory 。
Inflammatory arthritis = morning stiffness > 30 minuets and 。
pain improved with activity, worsened by resting with
s y no v i a l s w e l l i ng a nd t e nd e r ne s s o n e xam i nat i o n
Causes

Common:- 。
Rheumatoid arthritis -
Viral arthritis - Less than 6 week self rimination ,

Osteoarthritis - First meta carpal joint , old age

Psoriatic arthritis - Improve with activity ,

Ankylosing spondylitis -
Enteropathic arthritis -
SLE -
 Key points for diagnosis
Rheumatoid arthritis -
Symmetrical small joint involvement (sparing DIP)
6 weeks, early morning stiffness > 30 minuets

Large rheumatoid nodule, episcleritis, scleritis

Pleuro-pericarditis, fibrosing alveolitis, Hepatosplenomegaly

Viral arthritis -

Similar to RA but duration < 6 weeks and often associated with rash and prodromal illness
History of contact with children or working in kindergarten (parvovirus)
Fever a n d ly mp h a den op a t h y
 Key points for diagnosis

Osteoarthritis - Old age

Symmetrical, affecting knee, PIP, DIP and 1 st CMC joint

Heberden’s a nd Boucha rd’s nodule

Psoriatic arthritis -
Asymmetrical, affecting PIP and DIP

Sausage shape fingers, nail pitting and dystrophy

Personal of family history of psoriasis (but may precede psoriasis)
 Key points for diagnosis
UC

Young male, backpain, eye manifestation (uveitis)

Ankylosing spondylitis and enteropathic arthritis -
Symmetrical sacroiliitis and anterior uveitis for ankylosing spindylitis
Clubbing and associated IBD in enteropathic ar thritis (may precede IBD)

Malar rash,
SLE -
More usually causing polyarthralgia (synovitis is unusual)
Symmetrical, affecting small joint
Raynaud’s phenomenon, photosensitivity, levido reticularis, oral ulcer

Pleuro-pericarditis, fibrosing alveolitis, Hepatosplenomegaly

Hematuria and proteinuria
Pattern of joint involvement
 Investigations

Routine hematology, biochemistry 。

ESR, CRP 。

Viral serology (e.g. Hepatitis B, parvovirus) 。

。
Ne g a t ive c a n’ t e xc u ld e r h e u m a t oid

ANA, RF, ACPA

US or MRI may be needed if synovitis is not obvious clinically 。

Management

Treat the underlying cause 

NSAIDs and analgesia to control symptoms

Caplan Syndrome

RA + Pneumoconiosis

M ultiple pe riphe ra l nodule

Self remission

Good prognosis
Felty syndrome …
RA + Ne utrope nia + S ple nome galy
Ve ry high titre of rhe umatoid factor is a risk factor
Patie nt usually has rhe umatoid nodule
P r o ne t o have r e c ur r e nt s k in and r e s p ir at o r y infe c t io n

N.B: Ne utrope nia w ith large granular lymphocytosis (LGL) also lnown
as (Pseudo felty syndrome), is similar to Felty syndrome but with
inc reas ed lymp ho c yt o s is in b lo o d and b o ne mar ro w
Treatment of RA in brief …

Do rno t dela y a di mi n stration

go Metho trexat
ate start
Ste oid is giv in g as a bridgin e
ntil to
ac t
Methotrex
If both failed to control symptoms  Adalimumab (Anti-TNF)

N. B: Caution for liver, lung, kidney and bone marrow toxicity

w ith u s in g Meth otrexate
Before star tin g A dalimu mab you sh ou ld test for h epatitis
profile, HIV and screen for latent TB (risk of reactivation)
 This pt m aybe delay diagnosis not com plain on her m edication or sever disease uncontrolled on m edications

No role

SLE

D iagnostic

(hypercalcem ia, high RFT)

 Osteoarthritis

C ontraindication for C K D

C ontraindication for C K D

No role in osteoarthritis

Reactive arthritis: arthritis + urethritis + conjunctivitis

Tx : NSAID

Septic arthritis m ore than 50000

If pt on attack and NSA ID no im provem ent

If pt on attack and NSA ID no im provem ent

Sulpha better than m etho

Reactive arthritis

High lymphocytes, very low glucose, high LDH, low PH

Old age + Tem poral A rteritis + bilateral shoulder pain (stiffness)

Prometric SMLE course for GP

Topics: Rheumatology Part-3

Mentor:
Dr Ismail Azhari
Founder : CCR Academy
|| .. Vasculitis .. | |
What is Vasculitis?

Definition: Inflammation of blood vessels, leading to

vessel wall damage, narrowing, occlusion, or
aneurysm.

Causes: Autoimmune diseases, infections,

medications, malignancies.

Symptoms: Depends on the organ

involved—fever, fatigue, weight loss, rash,
joint pain.

Types :
Small vessel vasculitis :
Medium vessel vasculitis
Large vessel vasculitis
Positive :P-ANCA
C-ANCA positive
Associated with hepatitis -B
What is Your diagnosis?
Which antibody Will be positive in this case?
Ans: EGPA

P-ANCA positive
A 35-year-old male presents with a 2-month history of persistent nasal
congestion, frequent nosebleeds, and crusting in the nose. He also reports a
productive cough with occasional blood-tinged sputum, along with fatigue
and unintentional weight loss of 5 kg.

On examination:

Nasal septal perforation is noted.

Lung auscultation reveals crackles in both lung fields.

Urinalysis shows proteinuria and microscopic hematuria.

Initial blood tests reveal:

Elevated ESR and CRP

Mild anemia

What is Your diagnosis and which antibody Will

be positive in this scenario?
Ans: GPA

C-ANCA positive
What is Your diagnosis, ans which
antibody will be positive?
Question -1

(Expected Answer: Anti-Glomerular Basement Membrane

Disease, also known as Goodpasture’s Syndrome if both
kidney and lung involvement are present.)

Question 2: Investigations

What investigations would you order to confirm the

diagnosis and assess organ involvement?

(Expected Answers:)

1. Anti-GBM antibodies in serum (confirmatory test)

Kidney biopsy (shows linear IgG deposition along the

glomerular basement membrane on
immunofluorescence)

Initial blood tests show:

Elevated ESR and CRP

Mild anemia

Normal renal function

What is Your diagnosis?

Ans: Polyarteritis Nodosa

Dx?
Dx: Takayasu Arteritis

Inc: MR Angiography
 Granulomatosis with polyangiitis
(previously Wegener’s granulomatosis)

Granuloma formation, mainly affecting the nasal passages, airways and

kidney

Epistaxis, nasal crusting and sinusitis (most common presentation)

Hemoptysis and mucosal ulceration

Deafness (if inner ear involved)

Proptosis (inflammation of the retro-orbital tissue)

Migratory pulmonary iniltrates and nodules (seen in HRCT in 50% of patients)

 Eosinophilic granulomatosis with polyangiitis
(Previously Churg-Strauss syndrome)

Some patients have a prodromal period for many years,

characterized by allergic rhinitis, nasal polyposis and late-onset
asthma that is
often difficult to control

The typical acute presentation (triad):-

1- Skin lesions (purpura or nodules)
2- Asymmetric mononeuritis multiplex
3 - Eosinophilia
 Anti-glomerular basement membrane disease:-
Antibodies against the α3 chain of type 4 collagen GBM
(in basement membranes of glomeruli and lungs)

Rapidly progressive glomerulonephritis or lung hemorrhage

If both organ affected Goodpasture ’s syndrome

Treatment:-
Plasma exchange + glucocorticoids and
immunosuppressants
 Polyarteritis nodosa
Fever, myalgia, arthralgia and weight loss

Palpable purpura (most common skin lesions)

Skin ulceration, infarction and livedo reticularis Can do skin biopsy

Hepatitis B is an important risk factor

Male , abdominal pain, testicular pain, multiple aneurysm anywhere , HBV positive
So if come Q pt with these symptoms contradiction to do renal biopsy.
If pt have foot drop can take biopsy from sural nerve
 Polyarteritis nodosa
Symmetrical neuropathy affects both sensory and motor function

Gold standing
Confirmatory test: conventional or magnetic resonance
angiography (shows multiple aneurysms and smooth narrowing
of mesenteric, hepatic or renal systems)

OR muscle or sural nerve biopsy

Treatment: high-dose glucocorticoids and immunosuppressants

 Takayasu arteritis

Granulomatous inflammation of the vessel wall leading to occlusion

or weakening of the vessel wall

Affects major branches of the aorta (occasionally pulmonary

arteries)

Claudication, fever, arthralgia and weight loss

 Takayasu arteritis
Clinical examination: loss of pulses (pulseless disease), bruits, hypertension
and aortic incompetence
 The golden slide for Vasculitis

Granulomatosis with polyangiitis  Epistaxis, sinusitis, renal involvement, +ve

C -A NC A
Eosinophilic granulomatosis with polyangiitis  Nasal polyposis and difficult to
control asthma
Polyarteritis nodosa  Hepatitis B, testicular involvement, neuropathy,
diagnosed by MRA
Anti-GBM (Goodpasture)  Hematuria + hemoptysis with normal complement
Giant cell arteritis  Temporal headache, jaw claudication, high ESR, initiate
high dose steroid before temporal artery biopsy
Polymyalgia rheumatica  Proximal muscle pain and stiffness, associated
with GCA
Behçet’s disease  Oral and genital ulcer, clinical diagnosis
Takayasu arteritis  Affecting aorta, pulseless disease
Cryoglobulinemia  Purpuric rash with low complement, search for HCV

Any pt Behcet after diagnosis should to do CT angio for chest to detect pulmonary aneurysm can cause hemoptysis leading death
More in pe adiat ric , purple rash in buttock s
|| .. Miscellaneous .. | |
Limited cutaneous systemic sclerosis (LCSS)

Positive Anti-centromere antibody

CREST syndrome 。
R : Raynauds E: Esophagyeal dismottility

C: Calcinosis
S: Sclerodactyly T: Telangiectasia
Renal involvement in systemic sclerosis

Hypertensive renal crisis (rapidly developing malignant

hypertension and renal failure)

One o f the main c ause o f death

Aggressive treatment with ACE inhibitors is needed even if

renal impairment is present