Polyarthritis:
Differential diagnosis:
1) rheumatoid arthritis
2) SLE
3) seronegative spondyloarthropathies
4) Henoch-Schonlein purpura
5) sarcoidosis
6) tuberculosis
7) pseudogout
8) viral infection: EBV, HIV, hepatitis, mumps, rubella
Jaccoud's arthropathy
The picture shows joint subluxations and swan neck deformities, caused by recurrent
episodes of synovitis that damage tendon sheaths and slings resulting in joint deformity
but, in this case, there is no bone deformity.
Jaccoud's arthropathy is seen in:
1) SLE
2) Rheumatic fever
3) Parkinson's disease, and
4) Hypocomplementaemic urticarial vasculitis.
1
, Systemic lupus erythematosus
Epidemiology:
much more common in females (F:M = 9:1)
more common in Afro-Caribbean’s* and Asian communities
onset is usually 20-40 years
incidence has risen substantially during the past 50 years (3 fold using ACR criteria)
*It is said the incidence in black Africans is much lower than in black Americans -reason
unclear
Pathophysiology:
autoimmune disease, associated with HLA B8, DR2, DR3
caused by immune system dysregulation leading to immune complex formation
immune complex deposition can affect any organ including skin, joints, kidney &
brain
HAO hereditary angioneurotic oedema (deficiency of C1 esterase inhibitor): This
leads to persistent activation of classical complement pathway and C4 levels are
frequently low, If treatment fails to normalise C4 level, it is a high risk of developing
SLE
Features: SLE is a multisystem, autoimmune disorder.
General features:
1) fatigue
2) fever
3) mouth ulcers
4) lymphadenopathy
Skin:
1) malar (butterfly) rash: spares nasolabial folds
2) Discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas.
Lesions may progress to become pigmented and hyperkeratotic before becoming
atrophic
3) photosensitivity
4) Raynaud's phenomenon
5) livedo reticularis
6) non-scarring alopecia
Musculoskeletal:
1) arthralgia
2) non-erosive arthritis
Cardiovascular:
1) myocarditis
2) Libmansack endocarditis.
Respiratory:
1) pleurisy
2) fibrosing alveolitis
Renal:
1) proteinuria
2) glomerulonephritis (diffuse proliferative glomerulonephritis is the most common
type)
2
,Neuropsychiatric:
1) anxiety, depression, psychosis ,seizures,
2) subacute myelopathy with oligoclonal bands in serum and CSF (paraplegia)
SLE investigations:
Immunology:
1) 99% are ANA positive
2) 20% are RF positive
3) Anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
4) Anti-Smith: most specific (> 99%), sensitivity (30%)
5) anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
Monitoring:
1) ESR: during active disease.
The CRP is characteristically normal - a raised CRP may indicate underlying infection
2) complement levels (C3, C4) are low during active disease (formation of complexes
leads to consumption of complement)
3) anti-dsDNA titers can be used for disease monitoring (but not present in all patients)
Discoid lupus erythematosus
Benign disorder generally seen in younger females.
It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases).
characterised by follicular keratin plugs and is thought to be autoimmune in aetiology
Features:
1) erythematous, raised rash, sometimes scaly
2) may be photosensitive
3) more common on face, neck, ears and scalp
4) lesions heal with atrophy, scarring (may cause scarring
alopecia),pigmentation
Management:
1) topical steroid cream
2) oral antimalarials may be used second-line e.g. hydroxychloroquine,
3) avoid sun exposure
SACLE is ANA positive in 60% patients.
However, only 10-15% progress to SLE with moderate disease activity.
80% patients are anti-Ro antibody positive.
Skin disease may occur as part of SLE, or be present as CLE (frequently without
any systemic disease), and with variable chance of progression to SLE.
Discoid lupus erythematosus (DLE)
Subacute cutaneous lupus erythematosus (SACLE)
Acute cutaneous lupus erythematosus (ACLE)
are examples of CLE which may or may not progress to SLE. However, ACLE often
accompanies flare of systemic disease & presents as diffuse erythema,
maculopapular rash, photosensitivity & oral ulcers, while DLE presents as well
defined scaly plaques heal with central scarring. ( انظر الجلدية مكتوبة احسنp28)
3
, Hydroxychloroquine ocular toxicity includes:
Keratopathy
Ciliary body involvement
Lens opacities, and
Retinopathy.
Retinopathy is the major concern; the others are more common but benign.
The incidence of true hydroxychloroquine retinopathy is exceedingly low.
Risk factors include:
Daily dosage of hydroxychloroquine
Cumulative dosage
Duration of treatment
Coexisting renal or liver disease
Patient age, and
Concomitant retinal disease.
Patients usually complain of:
1) Difficulty in reading, decreased vision, missing central vision, glare, blurred
vision,
2) Light flashes, and metamorphopsia.
3) They can also be asymptomatic.
4) Most patients with advanced retinopathy have a bull's eye (also known as target,
as in darts) fundoscopic appearance.
All patients have field defects including paracentral, pericentral, and central and
peripheral field loss.
Regular screening may be necessary to detect reversible premaculopathy.
Cessation of the drug is the only effective management of the toxicity.
4
Differential diagnosis:
1) rheumatoid arthritis
2) SLE
3) seronegative spondyloarthropathies
4) Henoch-Schonlein purpura
5) sarcoidosis
6) tuberculosis
7) pseudogout
8) viral infection: EBV, HIV, hepatitis, mumps, rubella
Jaccoud's arthropathy
The picture shows joint subluxations and swan neck deformities, caused by recurrent
episodes of synovitis that damage tendon sheaths and slings resulting in joint deformity
but, in this case, there is no bone deformity.
Jaccoud's arthropathy is seen in:
1) SLE
2) Rheumatic fever
3) Parkinson's disease, and
4) Hypocomplementaemic urticarial vasculitis.
1
, Systemic lupus erythematosus
Epidemiology:
much more common in females (F:M = 9:1)
more common in Afro-Caribbean’s* and Asian communities
onset is usually 20-40 years
incidence has risen substantially during the past 50 years (3 fold using ACR criteria)
*It is said the incidence in black Africans is much lower than in black Americans -reason
unclear
Pathophysiology:
autoimmune disease, associated with HLA B8, DR2, DR3
caused by immune system dysregulation leading to immune complex formation
immune complex deposition can affect any organ including skin, joints, kidney &
brain
HAO hereditary angioneurotic oedema (deficiency of C1 esterase inhibitor): This
leads to persistent activation of classical complement pathway and C4 levels are
frequently low, If treatment fails to normalise C4 level, it is a high risk of developing
SLE
Features: SLE is a multisystem, autoimmune disorder.
General features:
1) fatigue
2) fever
3) mouth ulcers
4) lymphadenopathy
Skin:
1) malar (butterfly) rash: spares nasolabial folds
2) Discoid rash: scaly, erythematous, well demarcated rash in sun-exposed areas.
Lesions may progress to become pigmented and hyperkeratotic before becoming
atrophic
3) photosensitivity
4) Raynaud's phenomenon
5) livedo reticularis
6) non-scarring alopecia
Musculoskeletal:
1) arthralgia
2) non-erosive arthritis
Cardiovascular:
1) myocarditis
2) Libmansack endocarditis.
Respiratory:
1) pleurisy
2) fibrosing alveolitis
Renal:
1) proteinuria
2) glomerulonephritis (diffuse proliferative glomerulonephritis is the most common
type)
2
,Neuropsychiatric:
1) anxiety, depression, psychosis ,seizures,
2) subacute myelopathy with oligoclonal bands in serum and CSF (paraplegia)
SLE investigations:
Immunology:
1) 99% are ANA positive
2) 20% are RF positive
3) Anti-dsDNA: highly specific (> 99%), but less sensitive (70%)
4) Anti-Smith: most specific (> 99%), sensitivity (30%)
5) anti-U1 RNP, SS-A (anti-Ro) and SS-B (anti-La)
Monitoring:
1) ESR: during active disease.
The CRP is characteristically normal - a raised CRP may indicate underlying infection
2) complement levels (C3, C4) are low during active disease (formation of complexes
leads to consumption of complement)
3) anti-dsDNA titers can be used for disease monitoring (but not present in all patients)
Discoid lupus erythematosus
Benign disorder generally seen in younger females.
It very rarely progresses to systemic lupus erythematosus (in less than 5% of cases).
characterised by follicular keratin plugs and is thought to be autoimmune in aetiology
Features:
1) erythematous, raised rash, sometimes scaly
2) may be photosensitive
3) more common on face, neck, ears and scalp
4) lesions heal with atrophy, scarring (may cause scarring
alopecia),pigmentation
Management:
1) topical steroid cream
2) oral antimalarials may be used second-line e.g. hydroxychloroquine,
3) avoid sun exposure
SACLE is ANA positive in 60% patients.
However, only 10-15% progress to SLE with moderate disease activity.
80% patients are anti-Ro antibody positive.
Skin disease may occur as part of SLE, or be present as CLE (frequently without
any systemic disease), and with variable chance of progression to SLE.
Discoid lupus erythematosus (DLE)
Subacute cutaneous lupus erythematosus (SACLE)
Acute cutaneous lupus erythematosus (ACLE)
are examples of CLE which may or may not progress to SLE. However, ACLE often
accompanies flare of systemic disease & presents as diffuse erythema,
maculopapular rash, photosensitivity & oral ulcers, while DLE presents as well
defined scaly plaques heal with central scarring. ( انظر الجلدية مكتوبة احسنp28)
3
, Hydroxychloroquine ocular toxicity includes:
Keratopathy
Ciliary body involvement
Lens opacities, and
Retinopathy.
Retinopathy is the major concern; the others are more common but benign.
The incidence of true hydroxychloroquine retinopathy is exceedingly low.
Risk factors include:
Daily dosage of hydroxychloroquine
Cumulative dosage
Duration of treatment
Coexisting renal or liver disease
Patient age, and
Concomitant retinal disease.
Patients usually complain of:
1) Difficulty in reading, decreased vision, missing central vision, glare, blurred
vision,
2) Light flashes, and metamorphopsia.
3) They can also be asymptomatic.
4) Most patients with advanced retinopathy have a bull's eye (also known as target,
as in darts) fundoscopic appearance.
All patients have field defects including paracentral, pericentral, and central and
peripheral field loss.
Regular screening may be necessary to detect reversible premaculopathy.
Cessation of the drug is the only effective management of the toxicity.
4
