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Etiology:
The cause is multifactorial,
Genetic susceptibility especially to certain HLA class I & class II
alleles.
Environmental influences are also involved such as the role of
microorganisms especially at the early stages of disease.
Mechanism:
In normal conditions autoimmunity is prevented by self-tolerance.
Classification:
1 .Disorders with organ specific autoantibodies:
Example : pemphigus with intercellular desmosomes
of spinous cell layer act as antigen
2. Disorders with non-organ specific autoantibodies:
Example : sjogrens syndrome with antigens concerning
exocrine glands, thyroid ,kidney , and liver cells
mediated diseases:
3.
1. Lupus Erythematosus
2. Rheumatoid arthritis
Progressive systemic sclerosis
4. Dermatomyositis
Etiology:
vasomotor instability.
cooling or emotional upset precipitate vasoconstriction
Raynaud's phenomenon
Common features of C.T disease.
Affect only few fingers of each hand
Raynaud's disease
Seen in normal person
Spare only the thumb
Management
a. To stay in warm,
b. wear warm clothing,
c. protect the wrists & hands
LUPUS ERYTHMATOSIS
LE is a connective tissue disease with auto immune manifestation, of unknown etiology
and exist in many clinical forms:
1. Systemic LE (acute)
It is serious and aggressive multisystem disease
There is marked systemic manifestation.
There is marked and specific serological abnormalities (antinuclear antibodies "ANA" and
an, cytoplasmic antibodies).
2. Discoid (Chronic) LE
It is mucocutaneousdisease.
Least aggressive form.
There is no serological abnormalities
It rarely progress to SLE
3. Subacute Cutaneous LE
Lies between SLE and DLE.
Mild to moderate skin lesions.
Mild systemic features.
There is some serological abnormalities (anticytoplasmic antibodies) and some ANA may
be found).
It rarely progress to SLE.
1. DISCOID LUPUS
ERYTHMATOSIS
Site: It is relatively common disease
confined only to skin and oral mucosa
(mucocutaneous
disease).
Age: predominantly in the third and fourth
decade.
Sex: more common in female than in male.
Skin manifestation:
Site:
a) Face: where they involve malar regions and cross the ridge of the nose,
which assume
butterfly distribution.
b)The scalp, ears, chest, back and extremities. (b
Character:
a) The cutaneous lesions are slightly elevated red or purple macules
which is often covered with gray or yellow adherent scales. When the
latter is removed it reveals numerous "carpet tack" extensions which
has dipped into enlarged pilosebaceous canals.
b) The lesion has sharp borders which slowly expand and increase in size
by peripheral growth. The periphery of the lesion appears pink or red.
c) The center exhibits atrophic depigmented scarred appearance,
indicative to the chronicity of the lesion, with characteristic central
healing
Oral Manifestation:
Oral lesions has been reported in 25% to 50% of cases of DLE.
The early lesions begins as irregular erythematous macules
which may be elevated or depressed and without keratosis.
Later, the atrophic red area may be surrounded by keratotic
margin or covered by white keratotic spots.
Other may appear as keratotic lesion surrounded by red
(telangiectatic) halo.
A classical lesion has been described as alternating red
(atrophic), white (keratotic)
and red (telangiectatic) zones, provide characteristic
appearance.
Tongue lesion: is usually associated with atrophy of tongue
coating.
Lip lesion: The lower lip may reveal atrophic plaques
surrounded by keratotic border which may involve the entire
vermillion border and extend to circumoral skin. Malignant
transformation of lip lesion has been reported.
N.B. The margins of many of these keratotic lesion reveal a fine lacy
white network of stria (Wickham) so they simulate lichen planus. The
differentiation of both lesions can sometime be extremely difficult on
clinical examination alone. In the DLE however:
1. The lesions are less often symmetrically distributed
2. The pattern of striae is less well defined or conspicuous.
Prognosis:
DLE is a slowly progressive disease over a period of
many years.
Occasionally spontaneous remission occurs or in rare instances it
develops into SLE
Patients with oral DLE alone should be seen at least yearly in
order to:
1. Secure an early diagnosis of eventually developing sign of
cutaneous DLE. Early skin lesion responds better to local treatment
than older lesions.
2. The occurrence of oral ulceration is of clinical significance, it
may indicate the presence of or signal of latter developments of
SLE.
Treatment:
Topical steroid are used for both oral and cutaneous lesion.
In refractory skin lesions antimalarial drug or sulphones may be used.
Pathogenesis:
the autoanibodies include:
A) Antinuclear, antierythrocytes, antithrombocytes, antiphospholipids,
antilymphocyte antibodies (mainly T-lymphocytes) and anticytoplasmic
antibodies, circulating anticoagulant, Rheumatoid factor are found in the
patient's serum and tissue.
B) The autoantibody may be:
i. Directed against RBC, WBC, platelets and coagulation factors and is
responsible for hematological features i.e. anemia, leukopenia,
thrombocytopenia and clotting disorders.
Clinical Features:
SLE is predominantly a disease of young women, there is female predominance
of 10: 1 and has higher incidence among blacks.
SLE is serious cutaneous systemic disorder which characteristically manifest
repeated emission and exacerbation.
Diagnostic Criteria:
of SLE. This classification is based on 11 criteria and requires
that the patient exhibits four or more criteria either serially or
simultaneously, during any interval of observation in order to be
diagnosed positively.
1.The skin manifestations may be widely spread over the
body and appear as erythematous, edematous, macules.
2. Discoid scaly rash is less common. }Skin
3. Photosensitivity
7.Musculoskelatal:
a. Arthralgia .
b. Polyarthritis
c. Myosits.
8.Cardiopulmonary :
a. Pericarditis, myocarditis, endocarditis
often
associated with pleurisy
b. Localized myositis of the diaphragm result
in shrinking lung syndrome diminution of both lung
volume & normal gas transfer
c. Circulating immune complex damage
heart valve called libman sacks endocarditis . as
a result fibrin & platelets deposits at site of damage &
act as nidus for bacterial colonization during
bacteremia ( e.g. as result of extraction, scalling). This
patient need prophylactic antibiotic to guard against
infective endocarditis.
9.Neurologic:
a. Epileptic attacks.
b. Psychosis.
10. Immunologic
a.(+) ve anti- ds DNA
99%
b. (+) ve LE cell.
80%
c. Circulating antibodies against R.B.C,
W.B.C., platelets.
11.Hematological feature:
a. Normocytic normchrornic anemia .
b. Leucopenia .
c. Thrombocytopenia,
Oral Manifestation:
1) The oral manifestations of SLE may be similar to those described for DLE
but with little keratinization.
Oral lesions of SLE may be confused with oral Lichen planus , and
can be differentiated by histopathological and direct immunoflourescent
findings.
2) Erythematous patches,superficial erosion, or ulceration (induced
by vasculitis secondary to immune complex formation and complement
activation.)
3) Pallor, petechea, secondary infection or bleeding,(to
autoantibodies against RBCs,platelets, WBCs & clotting factors.)
4) The gingival tissues may take the form of desquamative gingivitis.
5) 30% of the patients complain of salivary gland swelling , ocular dryness ,
xerostomia & soreness as a part of Sjogren's syndrome.
Histopathologic Findings:
1. Hyperorthokeratosis or hyperparakeratosis with areas of atrophic
epithelium.
2. Acanthosis.
3. Hydropic degeneration of the basal cell layer
4. Vascular dilation with edema of the upper dermis or submucosa.
5. Diffuse infiltration of lymphocytes with small number of plasma
cells and occasional polymorphonuclear leukocytes in the superficial
and deep connective tissue. There is perivascular collection
oflymphocytes.
6. Degeneration of collagen and elastic fibers.
In SLE the degenerative features and collagen disturbance are
more prominent and inflammatory features are less sever than DLE.
SLE exhibit histopathologic changes in the oral lesion which is
identical with DLE but with lack of keratinization.
Complement Level:
The serum complement is frequently reduced in the presence of
active disease because of increased utilization due to immune
complex formation, reduced synthesis or combination of both
factors. C3 and C4 decreased during the disease activity.
N.B.: Complement C2 deficiency predispose to SLE,
Treatment:
Systemic coricosteroids are probably the most useful
therapy to control early acute manifestation and
together with immunosuppressive agents such as
azathioprine , for the potentially lethal lesions such as
renal involvement.
Otherwise most of the patient appear to do well in the
long term with non-steroidal
anti-inflammatory agents or if these agents are
ineffective, a very low doses of corticosteroids taken
on alternate days.
Anti-malarials such as chloroquine also appear to be
effective especially for skin and joint lesions.
Topical steroids , local analgesic and antifungal drugs
are used for treatment of oral lesions
Rheumatoid arthritis
the usual manifestations of RA ( +)
splenomegaly and leukopenia,( mainly
neutropenia)
Rheumatoid arthritis characterized by
inflammation of the synovial membranes.
Subtypes:
1-Felty's syndrome:
2-Juvenile RA (Still's disease):
systemic extra-articular symptoms are prominent,
including fever, lymphadenopathy,
hepatosplenomegaly, carditis, rash, ...
Laboratory findings
Anemia : normocytic normochromic .
Mild lymphocytosis.
Mild thrombocytopenia.
Hypergammaglobulinemia
Positive rheumatoid factor (60%-70% of cases)
ANA: found in low titre (20%-60% patients)
Diagnostic criteria
Diagnosis of RA is made with 4 or more of the following criteria:
Morning stiffness of more than 6 weeks.
Arthritis of 3 or more joint areas.
Arthritis of hand joints.
Symmetrical arthritis.
Rheumatoid nodules.
Positive rheumatoid factor.
Radiological changes.
Clinical features:
Onset: insidious
Symptoms: fatigue, loss of weight, numbness and tingling of the
extremities with increased stiffness of the hands or feet in the morning
Age: 30-40 years.
The small joints are first involved followed by larger joints The joints are involved bilaterally showing the signs of acute
inflammation, e.g. pain and swelling.
The affected joints have fusiform appearance.
The skin covering the joints become atrophic, smooth and glossy in
texture. The acute joint symptoms disappear gradually followed by
painful disabling contractures, muscular weakness and atrophy
Extra capsular features
Subcutaneous nodules in pressure points.
Enlargement of lymph nodes and spleen.
Chronic skin ulcers from diffuse arteritis.
Pleural effusion.
Pulmonary fibrosis
Treatment:
l. Rest in acute stage.
2.NSAIDs.
3. Gold, penicillamine, methotrexate, cyclosporine, corticosteroids.
4.Iron therapy.
5.Physiotherapy & psychological care
Dental management
1. The most common complication relates to the toxicity of the drugs treatment.
a. The most common adverse effects ofNSAIDs involve
GIT and the kidneys.
b. Aspirin at dosages approaching 5 g/day affect platelet function
c. Gold salts can cause stomatitis, blood dyscrasias, and nephrotic syndrome.
d. penicillamine cause bone marrow suppression and renal toxicity, heptotoxicity,
or drug-induced pemphigus.
e. Corticosteroids and immunosuppressive.
2. Patients with prosthetic joints may require prophylactic antibiotic.
3. Patients with Sjogren's syndrome may require additional instruction in personal
oral care.
4. Felty's syndrome affects bone marrow.
Dermatomyositis
It is degenerative inflammatory condition
of :
a. the skin,
b. the skeletal muscles and
c. occasionally the blood vessels.
The disease is characterized by
progressive muscular weakness with skin
rash
Laboratory investigations
a) Elevated serum enzyme levels derived from muscle destruction :
creatin Kinase, creatinurea
b) EMG evidence of muscle disease
c) Muscle biopsy
d) MRI
Clinical features
Course: the disease is acute, sub acute or chronic
Sex :
more women
Age: 40 to 50 years.
1. Diffuse bilateral, and symmetric proximal muscle weakness and wasting
of limbs, neck and trunk
2. The weakness is progressive and spreads to muscles of the face, larynx
and heart.
3. Skin:
a. purplish red - erythema on the face and edema of the eyelids that
become pinkish and violet and may be tender to touch.
b. periungual erytherna with telangectasia.
c. Flat topped violaceous papules on the dorsum of the interphalangeal
joints.
4. Risk of malignancy
Management:
Corticosteroids
Azathioprine
Methotrexate
Cyclosporin
Cyclophosphamide
Scleroderma
Scleroderma is multisystem C.T. disease of unknown
etiology characterized by:
1. Fibrosis -hardening of skin & mucosa.
2. Smooth muscle atrophy.
3. Fibrosis of internal visceral organs:
- GIT
- Lung
- Kidney
- Heart
Secondary changes due to pressure may occur in the
underlying osseous structure.
Age: 30-50y
Sex: Female three times as male
Etiology:
Unclear, it may be attributed to abnormal activation of immune system
and microvascular injury.
Pathogenesis:
Vascular wall fibrosis of small and medium-size arterioles is a
prominent alteration in PSS and plays a crucial role in the pathogenesis
of pulmonary hypertension, renal crisis, myocardial dysfunction, and
digital gangrene.
Excessive collagen deposition in affected tissues is responsible for
most of the clinical manifestations of this disease
Types:
A) Progressive Systemic Sclerosis (PSS):
1) Diffuse cutaneous scleroderma
Initial wide spread skin involvement.
Early visceral involvement.
Major morbidity& mortality.
2) Limited cutaneous scleroderma, CREST syndrome
Minimum skin involvement confined to fingers & face.
Visceral involvement occurs late.
Fairly benign course.
CREST syndrome: Calcinosis cutis, Raynauds phenomenon,
Oesophageal dysfunction, .Sclerodactyl and Telangiectasia.
B) Localized Scleroderma:
Scleroderma primary involve skin.
Minimum if any systemic involvement.
No visceral diseases.
Rarely progress to PSS.
Clinical features:
1. Raynaud 's phenomenon
2. Cutaneous Manifestations:
Skin: become indurated, smooth, atrophic with telangiectesia.
Face: expressionless, mask like.
Fingers: fibrosis, lead to stiffness & atrophy of the skin over the digits,
ischemia & ulceration of finger tips.
Perioral skin: rigidity
Salivary gland: fibrosis, xerostomia.
3. Musculoskeletal Manifestations
4. Gastrointestinal Manifestations
5. Cardiac Manifestations
6. Pulmonary Manifestations
7. Renal Manifestations