Anti fodrin antibodies are positive in
Anti fodrin are directed against muscarinic receptors seen in sjogren. Sjogren&;s syndrome is a late onset chronic systemic autoimmune disease characterized by lymphocytic infiltration and destruction of the salivary and lacrimal glands resulting in dry mouth(xerostomia) and dryness and atrophy of conjunctiva and cornea (keratoconjunctivits sicca). This disease is caused by an immune-mediated inflammation of salivary,lacrimal and sweat glands as Sicca Syndrome or with internal organ involvement. Clinical Types PRIMARY SS - Alone. SECONDARY SS - associated underlying connective tissue diseases (RA / SLE / Scleroderma ) SICCA SYNDROME -- Xerophthalmia + Xerostomia --Internal Organ / Bone C/F * Glandular manifestation Dry mouth (Xerostomia) due to decreased production of saliva by salivary glands * Lacrimal Gland Involvement -- Dryness of eyes causes keratoconjuctivitis sicca. * Burning itchy sensation in the eyes. SKIN MANIFESTATIONS (50% Xeroderma, pruritus and scaling Annular erythema, Papular Erythema including Sweet&;s-like lesions. Raynaud&;s syndrome Hyperglobulinemic Purpura Vitiligo Sweating abnormalities Cutaneous Amyloidosis Alopecia diffuse and generalized Diagnosis SS patients of both primary and secondary Sjogren&;s syndrome have marked hypergammaglobulinemia (IgG>IgA>IgM),ANA(>50c)/0) elevated total protein and sedimentation rate. Anti-Ro and Anti-La Antibodies occur in approximately 60% of patients with Sjogren&;s syndrome * Histolgy of skin shows an absence of sebaceous glands and decrease in the sweat glands. Revised classification criteria for Sjogren&;s Syndrome Ocular symptoms : at least one of - * Dry eyes for more than 3 months * Sensation of sand or gravel in the eyes * Need for tear substitutes more than 3 times a day * Oral Symptoms : at least one of -- * Dry mouth for more than 3 months * Recurrently or Persistently swollen salivary glands * Need liquids to swallow dry food * Ocular Signs --at least one the following two tests positive * Schirmer&;s test * Rose Bengal score 4. Histopathology: in minor salivary glands, focal lymphocytic sialoadenitis (focus score ?A). 5. Salivary gland involvement: a positive result for at least one of the following diagnostic tests: 1 Unstimulated whole salivary flow (1.5 ml in 15 min) 2 Parotid sialography showing punctate, cavitary, or destructive pattern, without evidence of obstruction in the major ducts 3 Salivary scintigraphy showing delayed uptake, reduced concentration 6. Autoantibodies -- Anti Ro and Anti La Criteria For primary SS In patients without any potentially associated disease. primary SS may be defined as follows: a. The presence of any four of the six items is indicative of primary SS, as long as either item 4 (Histopathology) or 6 (Serology) is positive. b. The presence of any three of the four objective criteria items (that is, items 3, 4, 5, 6) For secondary SS In patients with a potentially associated disease. the presence of item 1 or item 2 plus any two from among items 3, 4, and 5 may be considered as indicative of secondary SS Treatment Symptomatic treatment for dryness of eyes -- by lubricating agents such as 0.5% methylcellulose eye drops for 4-5 times daily. Cyclosporine ocular drops Bromhexine 16 mg TDS has been found to increase the lacrimal secretion. Aificial saliva and cyclosporin(2.5-5mg/kg), for xerostomia. Routine dental care Steam inhalation may help dryness of the respiratory tract. ref - Harrisons internal medicine 20th ed pg2560
Sarcoidosis
Sjogren
Systemic sclerosis
Dermatomyositis
Immune system