Download Articular syndrome

Differential diagnosis of
diffuse connective tissue diseases.
Tactics GPs.
Lecturer: Assoc. Akhmedov H.S.
diffuse connective tissue diseases (DCTD)
Autoimmune precces
Lack of mono etiological
factor
The similarity of the
pathological changes
Loss (signs of
inflammation) of blood
vessels
Polysyndromic flow
БТДК
articular
syndrome
The effectiveness of
glucocorticoids, cytotoxic
drugs and NSAIDs
Multifactorial predisposition to a certain type of role immunogenetic
factors related to the sixth chromosome
SPECIFIC
IMMUNE RESPONSE
Тlymphocyt
e
elimination
AG
A
B
IC
С
A
B
AG
С
AUTOIMMUNE
PROCCES
Тlymphocyt
e
elimination
AG
A
B
IC
Immunoinflammatory process
connective tissue
С
DCTD List
 1. SLE - idiopathic; drug lupus syndrome
 2. SSD - idiopathic; induced (chemical, pharmaceutical)
 3. Diffuse eosinophilic fastsiit 4. Dermatomyositis (polymyositis) - idiopathic,
paraneoplastic, juvenile
 5. Sjogren's syndrome - primary (Sjogren's
disease),secondary.
 6. Cross
 7. syndrome.Relapsing polychondritis relapsing panniculitis
(Weber-Christian disease).
 8. Antiphospholipid syndrome

SYSTEMIC LUPUS ERYTHEMATOSUS
The disease occurs predominantly in women (90%),
middle-aged (30-40 years).
Leading clinical features that are crucial
in the diagnosis of SLE:
Articular
syndrome
Lupus "butterfly"
discoid lupus
Raynaud's syndrome
alopecia
photosensitization
stomatitis
Nephritis, cardio,
Psychosis and (or) seizures seizure
LE-cell test, false-positive test Wasserman
syndrome thrombocytopenic purpura,
hemolytic anemia, and (or) leukopenia and (or)
thrombocytopenia.
prolonged fever
Accelerated erythrocyte sedimentation rate,
hypergammaglobulinemia, RF
pleurisy
pericarditis
The defeat of the peritoneum
Features articular syndrome in SLE
Migratory
arthralgia and
arthritis
Symmetrical joint
disease
Morning stiffness
(less than 30
minutes)
Contracture (due to the
destruction of muscles or
ligaments)
Articular
syndrome
Often in combination
with myalgia,
myositis, and
ossalgiya
Predominant
involvement of small
and medium-sized
joints, and sometimes
large
Deformation and
ankylosis are not
observed
Diagnostic criteria articular syndrome in systemic lupus
erythematosus (ARA)
erythema on the face - "Butterfly";
discoid lupus;
Raynaud's syndrome,
alopecia,
photosensitivity,
ulceration in the mouth, nose and throat,
arthritis without deformation;
LE-cells,
false-positive Wasserman,
proteinuria more than 3.5 g per day;
cilindruria,
pleurisy, pericarditis,
psychosis, convulsions,
hemolytic anemia, and (or) leukocytosis and (or) thrombocytopenia.
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Factors associated with poor
prognosis include:
 kidney
disease (especially diffuse proliferative
glomerulonephritis),hypertension, male gender,
onset before the age of 20 years;
 antiphospholipid syndrome; high disease
activity; severe internal organ; accession of
infection, complications of drug therapy.
SYSTEMIC SCLERODERMIA
Prevalence - 12 per 1 million population, mostly
women.
Leading clinical features that are crucial in the diagnosis of
SSD:
Articular
syndrome
•"Solid" edema
•induration
•atrophy
•pigmentation
•"Mask-like" face
•sclerodactyly
•CREST- syndrome
•Anemia and (or) leukopenia, thrombocytopenia
•Osteolysis of the terminal phalanges of hands and feet
•The defeat of the internal organs (esophagitis with
dysphagia, basal pulmonary fibrosis, macrofocal cardio, true
scleroderma kidney)
•Peripheral hyperpigmentation, trophic changes
•Lymphadenopathy, polyserositis, polyneuritis
•Increased erythrocyte sedimentation rate, hyperproteinemia,
hypergammaglobulinemia, RF

 1.
Diagnosis of SSc criteria (American Associations for
Rheumatology):
Main
•
scleroderma skin lesions proximal to the metacarpophalangeal or
metatarsophalangeal joints
 2.
Minor criteria:
 • sclerodactyly ("bird's foot" because of flexion
contracture);
 • scars on the distal phalanges;
 • bilateral basal pulmonary fibrosis.
CLASSIFICATION OF DERMATOMYOSITIS
A. Origin:
idiopathic,
paraneoplastic
B. Current: acute, subacute, chronic
B. Periods: prodromal, symptomatic, dystrophic
G. The extent of activity: 1,2,3
D. The main clinical signs (syndromes)
Leading clinical features that are crucial in the diagnosis of
diabetes:
muscle
syndrome
•"Typical skin changes
- periorbital edema
and erythema
(symptom score)
•telangiectasia
•Progressive weakness,
myalgia, edema and
further muscle atrophy
(proximal parts of the
limbs)
The increase in
performance:
creatine phosphokinase,
aldolase
transaminase
Diagnostic criteria for dermatomyositis (ARA)
 The main
 characteristic skin lesions: periorbital edema and erythema
(symptom of "points"); telangiectasia, erythema on the open areas of
the body (face, neck, upper chest, limbs).
 affected muscles (mainly proximal extremities), which is expressed in
muscle weakness, myalgia, edema, and later - atrophy.
 Patomorphology characteristic muscle biopsy (degeneration
necrosis, basophils, inflammatory infiltrates, fibrosis).
 Increase of serum enzymes - creatine kinase, aldolase,
transaminases 50% or more compared to the norm.
 Typical data electromyographic studies.
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Additional
calcification.
Dysphagia.
Diffuse eosinophilic fasciitis It
is a systemic disease of the connective tissue
with a primary infiltrative lesions of fibrous fascia
limbs, accompanied by dense sclerodermic
swelling of the skin, flexion contracture,
eosinophilia and hyper gamma globulinaemia
Diagnostic criteria DEF
 acute onset with seal tissues in the forearms and shins,
 limitation of movement,
 flexion contractures,
 skin lesions by type "orange peel",
 eosinophilia in the peripheral blood
 cell infiltration fascia with a predominance of
macrophages and lymphocytes and eosinophils
admixture,
 the positive effect of GCS


favorable prognosis
 Mixed
connective tissue disease
clinical
and immunological syndrome
systemic connective tissue disorders,
manifested by a combination of
individual clinical signs of SSc,
polymyositis, SLE and the presence of
in the blood of patients with antibodies
to ribonucleoprotein
 get
sick more often women.
 Patients of complainpain in the joints - polyarth-,
muscle weakness, muscle compression,
increase in blood creatine kinase. The skin
telangiectasia, erythematous and hypo- or
hyperpigmented spots, discoid lupus, alopecia,
etc. . The lesions of the
internalorgans:myocarditis, pericarditis, aortic
insufficiency, enlargement of the liver, spleen,
kidney-type glomerulonephritis. In the
blood, signs of anemia, leukopenia, increased
erythrocyte sedimentation rate. The main thing is
biopsy - a picture of myositis and necrosis of
muscle
 Sjogren
This
disease
is a systemic autoimmune
disease characterized by chronic
inflammation of the exocrine glands
that secrete predominantly Ig A,
especially the salivary and lacrimal,
with the gradual development of
secretory failure in combination with
various systemic manifestations.
The clinical picture consists of:
1. The lesions of the salivary glands, cornea,
conjunctiva, xerophthalmia as a dry
ceratoconyuktivita that appears foreign body
sensation in the eye, photophobia, burning sensation
in the eyes, redness of eyes, lack of tears, erosion and
corneal opacity.
2. The defeat of the salivary glands - mumps with
decreased secretion of saliva, dry mouth
(xerostomia). Parotid, submandibular gland is
enlarged, vayutsya, little saliva, it is thick, develops
cheilitis, glossitis, dental caries, stomatitis.
3. Defeat other exocrine glands with decreased
function, as manifested by dryness of the skin, putsconductive, esophagus, nose, throat, etc.
4. polyarth- or the development of arthritis by type RA
5. Systemic manifestations: fever, limfoadeno-Patiala,
vasculitis, myositis, liver enlargement, village-Zenk,
kidney disease, Raynaud's syndrome
DCTD APPLY TO 2ND CATEGORY SERVICES
GPs in conjunction with a dermatologist
should
Achieve clinical and laboratory remission.
Prevent injury to vital organs and systems in the first
place - the kidneys and central nervous system.
THIS IS ACHIEVED

suppression of immune inflammation and
immunocomplex pathology;

prevention of complications of immunosuppressive
therapy;

Direct impact on the pathological manifestations

treatment of complications arising during the course of
immunosuppressive therapy;

the impact on the individual, pronounced syndromes;

removing from the body of circulating immune complexes
and antibodies.
Suppression of immune inflammation and
immunocomplex pathology
1.
2.
glucocorticoids
cytostatics
Directly affect the pathological manifestations (DMARDs)
1.
2.
3.
antifibrotic therapy
aminohinolinovogo drugs
Inhibitors of TNF alpha
With the development of complications appoint:
Antibacterials (with intercurrent infection);
TB drugs (with the development of tuberculosis, often
pulmonary localization);
Formulations of insulin diet (at development of
diabetes);
Antifungal agents (candidiasis);
The course of anti-ulcer therapy (when a "steroid"
ulcers).
NSAID’s
basic drugs
Phase mucoid swelling
Phase fibrinoid swelling
granulation phase
phase fibrosis
Cytostatics
Тlymphocyt
e
AG
A
B
IC
С
Education of the patient
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The patient must be advised of the need to long
(lifelong) treatment, as well as directly dependent on
the accuracy of the results of treatment compliance
recommendations.
Should explain the negative effect of sunlight on the
course of the disease (acute provocation)
The importance of contraception and pregnancy
planning under medical supervision in view of disease
activity and functional state of vital organs.
Patients should be aware of the need for regular
monitoring of clinical and laboratory manifestations
and know the side effects of drugs used.
Medical rehabilitation at DCTD
Indications for physical rehabilitation and spa
treatment at DCTD:
mainly peripheral manifestations of the disease;
chronic or subacute with active pathological
process is not higher than I degree;
functional failure of the musculoskeletal system
are not above II degree.
Contraindications to the physio-functional and
sanatorium treatment at DCTD:
predominantly visceral forms of systemic connective
tissue diseases;
The high activity of (II and IIIstepeni)
expressed functional disorders of the musculoskeletal
system with the loss of self-care and independent
movement;
treatment with high doses of corticosteroids (more than
15 mg of prednisone per day) or receiving cytotoxic
drugs.
Indications for hospitalization

A Fever.
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Signs of diffuse CNS.
Hemolytic crisis.
Active forms.

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Severe comorbidities (pulmonary hemorrhage,
myocardial infarction, gastrointestinal bleeding,
and others.).
Keep in mind!
•Articular syndrome
(especially visceral);
•Prolonged fever of unknown
origin;
•serozity;
•Rash manifestations of
vasculitis;
•weight loss;
•Raynaud's phenomenon;
•The appearance of seals
Should be
deleted
DCTD
PREVENTION
primary
prevention
identification of persons threatened by this disease
(mainly relatives of patients).
Upon detection of them even one of the symptoms persistent leukopenia, antibodies to DNA, increasing
the ESR, hypergammaglobulinemia, or other signs of
pre-disease - should warn them against excessive sun
exposure, hypothermia, vaccinations, physiotherapy
(eg, ultraviolet radiation, mud).
Particular attention should be given to patients with
discoid lupus. To prevent the generalization of the
pathological process, such patients should not receive
ultraviolet irradiation, treatment with gold, spa
treatment.
secondary
prevention
•a thorough clinical examination;
•permanent and long-term daily use of hormonal
drugs in maintenance doses
•Glucocorticosteroids and aminohinolinovogo drugs
can be canceled only upon the occurrence of
complete remission;
•mode patient should be protective, lightweight, but,
if possible tempering (morning gymnastics, tiring
exercise and workout, wiping with warm water, long
walks in the fresh air).
•readjustment of foci of infection
tertiary
prevention
social and vocational rehabilitation
psychological adjustment