SJOGREN´S

SYNDROME

Sicca syndrome
Definition

 Its a chronic, systemic inflammatory

disorder of unknown etiology,
characterized by dryness of the mouth,
eyes, and other mucous membranes.
 Its associated with rheumatic disorders
sharing certain autoimmune features and
which lymphocyte infiltration into affected
tissues is seen.
 An association has been found between
HLA-DR3 antigen and primary SS.
Cont;

 In some, SS affect only the eyes or

mouth.
 In others is associated to other collagen
vascular disorder.
 Ocular symptoms occur when atrophy of
the secretory ephitelium of the lacrimal
glands causes dessication of the cornea
and conjuntiva ( keratoconjuntivitis
sicca).
Cont;
 In advanced cases, the cornea is severely
damaged and ephitelial strands hang from the
corneal surface ( keratitis filiformis).
 One third of SS patients develop enlarged
parotid glands that usually firm, smooth,
flutuating in size , mildly tender.
 Chronic salivary gland enlargement is rarely
painful.
 Intraductal cellular proliferation in the parotid
gland causes luminal narrowing and eventual
formation of compact cellular structures termed
epimyoephitelial islands.
Cont;

 When salivary gland atrophy occurs,

saliva diminishes, and the resulting
extreme dryness of the mouth and lips
(xerostomia) inhibits chewing and
swallowing and promotes tooth decay
and calculi formation in the salivary
ducts.
Cont;

 Dessication may also develop in the skin

and in mucous membrane of the nose,
throat, larynx, bronchi, vulva, and
vagina.
 Alopecia may occurs. Dryness of the
respiratory tract often leads to lunfg
infections and sometimes to fatal
pneumonia.
Others manifestation

 Gi tract, cardiac, renal,neurologic,

hematological manifestation.

Diagnosis

 Schirmer test, ocular staining, tear

lysozyme concentration, slit-lamp
examination, salivary flow, sialography,
salivary scintiscan, biopsy,immunological
tests.
 Prognosis, DD and treatment.
REITER´S SYNDROME

 Its an arthritis associated with non-

bacterial urethritis or cervicitis,
conjuntivitis, and mucocutaneous
lesions.
 RS is a seronegative
spondyloarthropathy.
 Is a reactive arthritis.
ETIOLOGY AND INCIDENCE

 Two forms are recognized: sexually

transmitted and dysenteric.
 The former occur primarily in young men
between ages 20 and 40.
 Genital infections with Chlamydias
trachomatis are most often implicated.
Cont;

 RS is less common in women, children,

and elderlies, who usually adquire the
dysenteric form following enteric
bacterial infections, primarily due to
shiguellas, salmonellas, yersinia and
campilobacter.
 The frecuency of HLA-B27 is 63-96% of
patients with RS, as compared with 6-
15% of healthy controls, supports a
genetic predisposition.
Symptoms, signs and course

 In typical RS, non-bacterial urethritis develops

to 7-14 days after sexual exposure or
dysentery.
 Low grade fever, conjuntivitis, and arthrits
develops over the next few weeks.
 The urethritis in men is less painful and
productive of purulent discharge than that seen
with acute gonorrhea, and may be associated
with hemorrhagic cystitis or prostatitis.
Cont;

 In women, urethritis and cervicitis may be mild (

dysuria and vaginal discharge) or entirely
asymptomatic, making DX difficult.
 Conjuntivitis is the most common eye lesion.
 Keratitis and uveitis coexist.
 Arthritisusually is the second or third feature of
RS and may be mild or severe.
Cont;

 Joint involvement is asymmetric and

polyarticular, occurring in the large joints
of the lower extremities as well as the
toes.
 Back pain may occur, usually with more
severe disease,
 Entesophaty is common
Cont;
 Mucocutaneous lesions, small painless
superficial ulcers, are commonly seen on the
oral mucosa, tongue, and glans penis (balanitis
circinata).
 Patients may also develop hyperkeratotic skin
lesions of the palms and soles and around the
nails (keratodermia blenorrhagica)
 Joint deformity, ankylosis, sacroileitis may
occur with chronic or recurrent RS
 DX, Prognosis and Treatment
PSORIATIC ARTHRITIS

 About 10% of persons with psoriasis

have some forms of inflammatory joint
disease.
 HLA-B27 antigen is present in some
patients, specially when the spine is
involved.
Clinical manifestation

 Onset of the skin disease may long

precede the arthropathy, but
occasionally, the reverse is true.
 The features of PA are:
 1- Predilection for a distal joints of the
fingers and toes, frequently
accompanied by psoriatic involvement of
only a few others joints of the limbs.
Cont;

 Destructive and mutilating changes of

the phalanges adjacent to the inflammed
joints, which produce the radiographic
appearence of a whittling of pencil point
of cup of the proximal phalanx and a
cupping of the central portion of the base
of the apposing distal phalanx, with bony
proliferation of the borders.
Cont;

 3- Shortening, angulation, and

telescoping of the fingers due to
extensive bone resorption of the
phalanges.
 4- Frequent involvement of the sacroiliac
joints and spine, whic simulates
ankylosing Spondylitis.
Other features

 Absence of RF and rheumatoid nodules

 Presence of HLA-B27 in the serum of
90% of patients with sacroilñiitis and
spondylitis.
 More frequent remission and
arthropathy.
 Radiography evidence of gross
destruction of isolated small joints, with
marked osteolysis and bone erosion.
Cont;

 Periosteitis of the shafts of bones.

 Non-marginal as well as marginal
syndesmophytes as features of
spondylitis.
Lab. Studies

 Elevated ESR
 Mild anemia
 Serum uric acid elevated
 Synovial fluid analysis reveals only an
exudative , polymorphonuclear
response.
Treatment

 Is directed at control of skin lesions and

joint inflammation.
 Therapeutic measures are the same as
those indicated for rhematoid arthritis,
except for the use of antimalarials.
 The therapy with Etretinate 0.5-1
mg/kg/day, orally in 2 doses is effective
in severe psoriasis and arthritis also.
Cont;

 Photochemotherapy using oral

methoxsalen and long wave ultraviolet A
light, folic acid antagonists and
immunosuppressive agents , especially
methotrexate.
 They should be used only in very severe
cases
 Surgical procedures are performed to
correct deformities and replace joints.
ANKYLOSING SPONDYLITIS

 A heterogeneous and systemic

rheumatic disorder characterized
primarily by inflammation of the axial
skeleton and large peripheral joints.
 Three times more frequent in men than
in women.
 AS begins most often betwee the ages
of 20 and 40
Cont;

 Familial clustering and the higher than expected

frequency of the HLA/B27 tissue antigen among
patients support a genetic basis for the disease,
although environmental factors also appear to
be operative.
 Calculations based on 2 american surveys, one
B/27 positive blood donors and another of B/27
negative blood donors, indicate that the risk od
AS developing in individuals with HLA/27 may
be 20%
Symptoms and signs

 Back pain
 The disease can begin atypically in
peripheral joints, especially in children
and women, and rarely even with acute
iritis ( anterior uveitis).
 The patients present recurrent back pain
that is often nocturnal and of vaying
intensity is an eventual complain, as is
early morning stiffness that is
characteristically relieved by activity.
Cont;

 This is followed by paraspinal muscle spasms

by adopting a flexed or bent –over posture.
 In untreated patients, some degree of kyphosis
is common
 Diminshed chest expansion that result from
diffuse costovertebral involvement.
 Additional early symptoms are fever, fatigue,
anorexia, weight loss and anemia.
Cont;

 Systemic manifestation include recurrent

attacks of acute iritis that affects 1/3 of patients
but usually are self –limiting.
 Neurologic signs result from compression
radiculitis or sciatica , vertebral fracture or
subluxation ,and the cauda equina syndrome.
 The latter produces impotency ,nocturnal
urinary incontinence,diminished bladder and
rectal sensation, and abscense of ankle jerks.
Cont,

 Cardiovascular manifestation include

angina, pericarditis, EKG conduction
abnormalities, and rarely aortic
insufficiency.
 A rare pulmonary findings is upper lobe
fibrosis, occasionally with cavitation that
may be mistaken for TB and may be
complicated by infection with Aspergillus.
 Tuberculous spondylitis is considered in
the differential DX.
Diagnosis

 ESR elevated, high levels of IGA,

 RF negative, ANA negative
 Positive test for HLA/27 is the best single labs
clue.
 Diagnosis may be confirmed by X-ray.
 The earliest abnormalities occur in the
sacroiliac joints and include pseudowidening
and narrowing of these articulations from
subchondral erosions and sclerosis.
Cont;

 Early changes in the spine are diffuse

vertebral squaring and deminralization
as well as spotty ligamentous
calcification and 1-2 evolving
syndesmophytes.
 The classic bamboo spine with
prominent syndesmophytes and diffuse
ligamentous calcification
Differential diagnosis and
therapy
 Herniated intervertebral disk
 DISH syndrome
 Therapy includes NSAIDS,
corticosteroids, radiotherapy of the spine
 Biologicals agents.