RHEUMATOLOGY

SMLE REVIEW
Elaf Faisal
Internal Medicine Resident
Al-Noor Specialist Hospital - Makkah
|| .. Systemic lupus erythematosus .. ||
Clinical features

Arthritis ◦
Raynaud’s phenomenon ◦
Skin ◦
• Sx of flare diseases:- fever, weight
Kidney ◦
loss or mild lymphadenopathy
Lung ◦
• Constant Sx:- fatigue, malaise or
Neurological ◦ fibromyalgia like symptoms
Hematological ◦
Gastrointestinal ◦
Skin manifestations
Rash precipitated by UV light ◦
3 types:- ◦
1- Butterfly facial rash (erythematous, raised, painful or itching, over
cheeks and sparing nasolabial fold), photosensitivity
2- Subacute cutaneous lupus erythematosus (SCLE)
3- Discoid rash
Skin Levido reticularis
manifestations

SCLE butterfly facial rash Discoid rash

Cardiovascular manifestations

Pericarditis (most common) ◦

Myocarditis ◦

Libman-sacks endocarditis (due to accumulation of sterile fibrin ◦

containing vegetation on heart valves) à search for APS

Risk of atherosclerosis and MI increased! ◦

Criteria for diagnosis
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
Anti-Ro (SSA) and Anti-La (SSB) à +ve in neonatal lupus and SCLE
Screen pregnant woman with these antibodies

Antiphospholipid antibody:-
- Lupus anti-coagulant
- Anti-cardiolipin
- Anti B2 glycoprotein

Antiphospholipid syndrome = History of miscarriages + arterial and venous

thromboembolism, treatment is life-long Warfarin
Investigations
How to measure disease severity?
- Anti-dsDNA
- Low complement C3 and C4 (especially in lupus nephritis)
- High ESR
- Leucopenia
- Lymphopenia
- Thrombocytopenia
- Hemolytic anemia

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 Antiphospholipid 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 ◦

with proteinuria and low C3, C4

Regular monitoring of BP and urinalysis is essential ◦

Management (Pharmacological)

Hyroxychloroquine is the cornerstone

Mild to moderate disease (cutenous, joint involvement):-

NSAIDs + Hyroxychloroquine
Low dose cortecosteroid may be used in combination with
immunosuppressant (Methotrexate, Azathioprine or Mycophenolate
mofetil)
Management (Pharmacological)

v Pharmacological:-

Life-threatening disease (renal, CNS, cardiac involvement):-

High dose corticosteroid + immunosuppressant
(Methylprednisolone + Cyclophosphamide)

N.B:- Cyclophosphamide cause hemorrhagic cystitis (avoided by good

hydration and mesna), azoospermia and anovulation (needs pre-
treatment ova and sperm collection and storage)
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
- Symptoms:- malar rash, discoid rash, alopecia, arthralgia,
Raynaud’s, pericarditis, Libman-sack endocarditis, mouth
ulcer, Neurological or hematological

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

Anti-dsDNA (specific also), low complement C3 and C4, high
ESR all indicate disease severity

- Treatment:- Hydroxychloroquine
|| .. 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
2- Gout
3- Pseudogout
4- Reactive arthritis
5- Trauma
6- Haemoarthrosis
7- Seronegative spondyloarthritis (psoriatic arthritis, ankylosing
spondylitis, enteropathic arthritis)
Causes
Less common:- ◦
- Rheumatoid arthritis (very uncommon, consider septic arthritis!)
- Juvenile idiopathic arthritis
- Osteoarthritis (gradual onset, seldom joint swelling)
- Gonococcal infection
- Tuberculosis infection
- Forigen body reaction
- Erythema nodosum
- Leukemia
- Osteomyelitis
 Hints to diagnose …
Septic arthritis: redness, tenderness, hotness, slower onset and progressive

Gout: common in middle age men, affect 1st MTP joint, very rapid onset (6-12
hours), hypertensive patient taking hydrochlorothiazide

Pseudogout: common in older women, affect wrist and shoulder, very rapid onset

Both gout and pseudogout is triggered by intercurrent illness, dehydration or v

surgery
Causes
Reactive arthritis: common in young men, recent sexual contact, Hx
of GI or GU infection (diarrhea caused by Campylobacter jeujeni or
chlamydia), oral ulcer, conjunctivitis
Can not bee, Can not SEE, Can not climb a TREE

Haemoarthrosis: large effusion, absence of periarticular swelling or

skin change, Hx of trauma or haemophilia
Investigations
AspirationØ
Send fluid for Culture, Crystal (microscopy) and Gram stain
WBC > 50,000 à septic arthritis
-ve birefringent crystal à gout
+ve birefringent crystal à pseudogout

Blood culture, ESR, CRPØ

Serum uric acid is USELESSØ

Management

Treat the underlying causev

Start IV antibiotic if there is any suspicion of sepsisv

Focus on management of SEPTIC ARTHRITIS, GOUT and PSEUDOGOUT …

Septic arthritis (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)

Gout and pseudogout:- treat with 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 swelling affecting 5 or more joint or joint groups ◦

Inflammatory vs non-inflammatory ◦

Inflammatory arthritis = morning stiffness > 30 minuets and ◦

pain improved with activity, worsened by resting with
synovial swelling and tenderness on examination
Causes
Common:- ◦
Rheumatoid arthritis -
Viral arthritis -
Osteoarthritis -
Psoriatic arthritis -
Ankylosing spondylitis -
Enteropathic arthritis -
SLE -
Causes
Less common:- ◦
Juvenile idiopathic arthritis -
Chronic gout -
Chronic sarcoidosis -
Polymyalgia rheumatic -
Rare:-Ø
Systemic sclerosis and polymyositis -
Hypertrophic osteoarthropathy -
Hemochromatosis -
Acromegaly -
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 and lymphadenopathy
Key points for diagnosis
Osteoarthritis -
Symmetrical, affecting knee, PIP, DIP and 1st CMC joint
Heberden’s and Bouchard’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
Ankylosing spondylitis and enteropathic arthritis -
Symmetrical sacroiliitis and anterior uveitis for ankylosing spindylitis
Clubbing and associated IBD in enteropathic arthritis (may precede IBD)

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
Key points for diagnosis

Juvenile idiopathic arthritis -

Symmetrical, small or large, upper or lower limb affection
Fever and lymphadenopathy

Chronic gout -
Distal joints more than proximal, Hx of acute attach of gout
Large white nodule (Tophi)
Pattern of joint involvement
Investigations
Routine hematology, biochemistry ◦

ESR, CRP ◦

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

ANA, RF, ACPA ◦

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

Management

Treat the underlying causev

NSAIDs and analgesia to control symptomsv

Caplan Syndrome

RA + Pneumoconiosis

Multiple peripheral nodule

Self remission

Good prognosis
Felty syndrome …
RA + Neutropenia + Splenomegaly
Very high titre of rheumatoid factor is a risk factor
Patient usually has rheumatoid nodule
Prone to have recurrent skin and respiratory infection

N.B: Neutropenia with large granular lymphocytosis (LGL) also lnown

as (Pseudo felty syndrome), is similar to Felty syndrome but with
increased lymphocytosis in blood and bone marrow
Treatment of RA in brief …

Do not delay administration of Methotrexate

Steroid is giving as a bridging until Methotrexate start to act

If both failed to control symptoms à Adalimumab (Anti-TNF)

N.B: Caution for liver, lung, kidney and bone marrow toxicity
with using Methotrexate
Before starting Adalimumab you should test for hepatitis
profile, HIV and screen for latent TB (risk of reactivation)