1. Sjogren’s syndrome

2. 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

3. 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

4. Parotid gland enlargement

5. 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

6. 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

7. 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

8. 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

9. 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 )

10. Adult polymyositis
Symmetrical proximal muscle weakness usually affecting the lower extremities first
Sometimes combined with muscle pain
Onset 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

11. 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

12. Gottron’s papules

13. Gottron’s papules

14. 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

15. Heliotrope rash

16. Heliotrope rash

17. 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

18. dystrophic calcification ( calcinosis )

19. dystrophic calcification ( calcinosis )

20. 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

21. 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

22. 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

23. 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
Treatment by steroids and immunosuppressive drugs