Transcript Sjogren`s syndrome

Sjogren’s syndrome
Sjogren’s syndrome
• Autoimmune disorder of unknown etiology
characterized by lymphocytic infiltration of
salivary and lachrymal glands leading to
glandular fibrosis and exocrine failure
• 40 – 50 years of age
• Female 9 : male 1
• Primary or secondary disease
Clinical features
• Dry eyes ( keratoconjunctivitis sicca ) due to
lack of lacrimation and tears , conjunctivitis
and blepharitis lead to filamentary keratitis
• Oral involvement :dry mouth , difficulty in
swallowing , dental caries
• Salivary gland enlargement
• Non erosive arthritis
• Fatigue and Raynaud's phenomenon
Parotid gland enlargement
Clinical features
• Less common features : low grade fever
,anemia , interstitial lung disease , vasculitis ,
peripheral neuropathy , lymphadenopathy ,
glomerulonephritis , renal tubular acidosis ,
lymphoreticular malignancy , leucopenia ,
thrombocytopenia and cryoglubulinemia
• 40 fold increased risk of lymphoma
Investigations
• Increased ESR
• Auto antibody : ANA , RF , anti Ro , anti La
antigastric parietal cell , antithyroid
• Sicca established by Schirmer tear test
• Lip biopsy showed focal lymphocytic infiltrate
in the minor salivary gland
Management
• Lachrymal substitutes such as hypromellose
drop during day and lubricating ointment at
night
• Soft contact lenses can be used in patients
with filamentary keratitis
• Occlusion of lachrymal duct is occasionally
needed
• Artificial saliva and oral gel can be used for
xerostomia
Management
• Stimulation of saliva flow by sugar – free
chewing gum or lozenges may be helpful
• Vaginal dryness is treated with lubricants such
as k.y. gelly
• Steroid or immunosuppressive drugs may be
used in extra glandular or musculoskeletal
manifestations
Polymyositis and dermatomyositis
• Idiopathic inflammatory myopathies (IIMs )
are rare connective tissue disorders defined by
presence of muscle weakness and
inflammation
• Usually only skeletal muscle is affected ,
occasionally the distribution is focal ( orbital
myositis )
Adult polymyositis
• Symmetrical proximal muscle weakness
usually affecting the lower extremities first
• Sometimes combined with muscle pain
• Onset 40 -60 years with gradual onset
• Fever , wt loss and fatigue are common
• Respiratory or pharyngeal muscle involvement
leading to ventilatory failure / aspiration
• Interstitial lung disease occur in 30 % of
patients and strongly associated with the
presence of antisynthetase ( e.g jo1
)antibodies
Adult dermatomyositis
• Muscle involvement with characteristic
cutaneous manifestation
• Gottron’s papules are scaly erythematous /
violaceous plagues or papules occurring over
the extensor surfaces of the proximal and
distal interphalangeal joints
Gottron’s papules
Gottron’s papules
Adult dermatomyositis
• Heliotrope rash is violaceous discoloration of
the eyelid in combination with periorbital
edema
• Erythematous rash on the upper back , chest
and shoulder ( shawl distribution )
• Peri unqual nail – fold capillaries are often
abnormal
• Arthralgia , weight loss and fever
Heliotrope rash
Heliotrope rash
Childhood dermatomyositis
• Three times more common in girls than in
boys
• Affect 6-9 years old
• The presentation is similar to adult
dermatomyositis except the cutaneous
manifestation are more common
• Cutaneous ulceration , lypodystrophy and
dystrophic calcification ( calcinosis ) in the
skin subcutaneous tissue and muscle fascia
• Vasculitis
dystrophic calcification
calcinosis )
(
dystrophic calcification
( calcinosis )
Investigations
• Increase creatin kinase
• EMG confirm presence of myopathy and
exclude neuropathy
• muscle biopsy ( necrosis , regeneration and
inflammatory cell infiltrate
• MRI usual to identify areas of abnormal
muscles
• There is increase risk of malignancy in patients
with dermatomyositis
Investigations
• Screening investigation when malignancy is
suspected should include chest / abdomen /
pelvis CT , GI imaging and mammography
• Malignant disease particularly ovarian , lung ,
pancreatic , stomach and colorectal
Management
• Oral steroids ( 40 – 60 mg / day of
Prednisolone )
• In severe cases ( respiratory or pharyngeal
weakness may need methylprednisolone 1 g
daily for three days
• Additional immunosuppressive therapy may
be needed as azathioprine and methotrexate ,
if not tolerated we use cyclosporine ,
cyclophosphamide or tacrolimus is an
alternative
Inclusion body myositis
• Over 50 years , mainly male
• Distal involvement is more common and
asymmetrical
• Neuropathic changes may be present on EMG
• Muscle biopsy showed abnormal fibers
containing rimmed vacuoles and filamentous
inclusions in the nucleus and cytoplasm . These
inclusions contain paired helical filament that
resemble those seen in the brain in Alzheimer’s
disease