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 Treatment by steroids and immunosuppressive drugs