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Connective tissue disorders
Systemic lupus erythematosus
Želmíra Macejová
Systemic lupus erythematosus
autoimmune, organ non specific disease
hyperreaction of B-lymphocyts with
production of antibodies agains organ nonspecific antibodies
multisystem disease
unknown etiology
variable course and prognosis
Clinical features
Inflammation in various organ systems including
skin, mucose membrane, joints, kidney, brain,
serous membrane, lung, heart, gastrontestinal
tract.
Involvement of organ- single or in combination
Mortality: involvement of kidney and CNS
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General clinical features
- fatigue, fever, malaise, weight loss
Ethiology
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unknown, multifactorial
exogenous: virus infection
UV light
stress
drugs: hydralazin
endogenous: hormonal
genetic predisposition
immune reaction:
hyperreactivity of B- cells
pathologic T cell , failure of remove immune complexes
Clinical Features- general
Subfebrility, fever- 80%
 Malaise, tiredness
 Skin
 Mental disorders, psychiatric manifestation
 arthralgia, arthritis
 thrombosis
 Changes of blood count
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The joint
Arthritis, arthralgia – morning stiffness
stuhnutosť
 Involvement: small joints of hand, wrist,
knees,
 arthritis- is not erosive
 Myositis- pain of involved muscles
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Skin manifestation
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Photosenzitivity
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Butterfly erythema
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Vaskulitic lesions:
fingertips, around nails,
purpura, urticaria
Raynaud s phenomenon,
Livedo reticularis
Palmar, plantar rashes
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Hematologic changes
Anaemia
 Neutropenia, lymfopenia, trombocytopénia
 ESR – raised
 CRP normal
 ANA : positiv: dsDNA, DNP, RF, serum
complement- redused, immunoglobulin
IgG, IgM - raised
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The kidney
Lupus nephritis – 30-50%
 cause of death
 immune complex deposits in the kidney
 hypertension
 nephrotic syndrome
 renal failure
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Lungs
Recurent pleurisy
 Pleural effusions
 Pulmonary fibrosis
 Pulmonary hypertension
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Serositis
Pleuritis - 30-60% patients
- clinicaly asymptomatic
- more often in drug induced SLE
 Pericarditis - 60% patients
- clinicaly asymptomatic
 Diffuse peritonitis with little amount of
fluid
- clinicaly asymptomatic
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The heart
- 40%
 Pericarditis
 Myocarditis
arrythgmia, cardiomegaly, tachykardia
 Endocarditis - abakteriálna Libman-Sacks
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The gastrointestinal system
Vasculitis of art. mesenterica:
antiphospholipid syndrome
 Lupus enteritis
 Pancreatitis
 Autoimune hepatitis
Symptoms: nausea, vomiting, anorexia,
diarrhoe
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The nervous systemneuropsychiatric lupus
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N-P lupus : 60-70%
Main cause of death
Mild depression
Severe psychiatric disturbances
Epilepsy
Cerebellar ataxia
Aseptic meningitis
Peripheral neuropathy
Cause: vasculitis, immune-complex deposition
Diagnostic criteria for SLE
Diagnostic criteria SLE
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1. Facial skin erythema
2. Discoid erythema
3. Fotosenzitivity
4. Mouth ulcers
At least 4!!
5. Arthralgia, arthritis
6. Serozitis
7. Involvement of kidney
8. Neurologic manifestation
9. Hematologic manifestation
10. Imunologic manifestation
11. Antinuklear antibodies
Treatment
NSA
 corticosteroids
 Antimalarial drugs - hydroxychloroquine
 Azathioprin
 Cyklofosfamid
 Cyklosporin A
 Plazmaferesis
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Systemic sclerosis
Multisystem disease that predominantly
affect the skin, locomotion system, organs
 Women : men 4:1
 Aetiology: unknown
 HLA B8, DR3
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Clinical features
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Skin involvement – edema štádium
sklerosis skin
skin atrofia
GIT involvement:
hypomotility of oesophagus
hypomotility of small bowel
malabsorbtion
primárna biliar cirhosis
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Lung involvement
alveolitis
fibrosis
pulmonary hypertension
Heart involvement
fibrosis
perikarditis
arrythmias
Vascular involvement:
Raynod s phenomenon
Joints and muscles: arthralgia, arthritis
Kidney involvement: renal failure, nephrotic
syndrome
Treatment
D-penicilamín
 Cyklofosfamid
 Methotrexate
 Corticosteroids
 Cyklosporín A
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Dermatomyositis
Associated with carcinoma- paraneoplastic!!!
 Inflammatory disease of muscles – polymyoszitis
+ skin dermatomyositis
Aetiology: unknown
Clinical features: febrility, Raynaud s phenomenon,
arthritis
Muscle weakness, atrophy of muscles
Skin involvement: inflammation of eyelids,
heliotrope coloration, ragged cuticle
Joint involvement
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Dysphagy, pulmonary manifestation: alveolitis,
pulmonary hypertension
Heart involvement
Investigation: muscle enzymes: CK, aldolase
EMG
muscle biopsy
Treatment: corticosteroids
methotrexate
azathioprin
Poor prognosis
Sjogren s syndrome
Sicca syndrome: dry eyes, dry mouth
 HLA B8, DR3
 Primary SjS
Secundary SjS –RA, SLE
KO: xerostomy
xerophtalmy: keratoconjunctivitis sicca
Involvement: kidney, lungs, joints
Myalgia, artralgia, arthritis
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Biopsies of the salivary gland or of the
lipshow a focal infiltration of lymphocytes
and plasma cells.
 Laboratory abnormalities: ESR, RF,ANA,
anti-Ro (SSA), anti La (SSB)
trombocytopaenia
 Treatment: symptomatic
antimalaric: hydroxychlorochine
corticosteroid drugs
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