شوگرن دکتر نوری روماتولوژیست

1. شوگرندکتر نوریروماتولوژیست

2. Sjögren's syndrome Sjögren's syndrome is a chronic, slowly progressive autoimmune disease characterized by lymphocytic infiltrationof the exocrine glands resulting in xerostomia and dry eyes

3. Sjögren's syndrome .. The disease presents alone (primary Sjögren's syndrome) or in association with other autoimmune rheumatic diseases (secondary Sjögren's syndrome)).

4. Middle-aged women (female-to-male ratio, 9:1) are primarily affected, although it may occur in all ages, including childhood.

5. . The prevalence of primary Sjögren's syndrome is approximately 0.5–1%, while 30% of patients with autoimmune rheumatic diseases suffer from secondary Sjögren'ssyndrome.

6. Siogren Syndrome • prevalenc 0/29%- 0/77% • Female/Male = 9 • In Fourth and Fifth decade of life • Secondary SS • Etiology • High risk of Lymphoma • RA ,SLE, Scleroderma • Polymyositis, PAN • Organisms • Genetic factor( HLADR3-HLADQ2) • Autoantibodies ( SSA/Ro - SSB/La)

7. Pathogenesis of Sjogren’s: The salivary and lacrimal glands are not fully destroyed. In fact, only 50% of the acini and ducts are destroyed. The residual ducts are not functioning due to the release of cytokines and metalloproteinases.

8. In Sjogren’s syndrome,many acini and ducts are spared Areas of “lazy” gland Sjogren’s Normal Lymphocytic infiltrate

9. Siogren : a multisystemic Diseases

10. Sjögren's syndrome often contain autoantibodies directed against non-organ-specific antigens such asimmunoglobulins(rheumatoid factors) and extractable nuclear and cytoplasmic antigens (Ro/SS-A, La/SS-B). Ro/SS-A autoantigen

11. . Autoantibodies to Ro/SS-A and La/SS-B antigens are usually detected at the time of diagnosis and are associated with earlier disease onset, longer disease duration, salivary gland enlargement, more severe lymphocytic infiltration of minor salivary glands, and certainextraglandular manifestations

12. Salivary glands

13. oral symptom • dryness (xerostomia). • difficulty in swallowing dry food, • inability to speak continuously, • a burning sensation, • increase in dental caries,

14. . • Physical examination • dry, erythematous. • atrophy of the filiform papillae on the dorsum • Enlargement of the parotid or other major salivary glands occurs in two-thirds of patients with primary Sjögren's syndrome but is uncommon in those with the secondary syndrome.

15. Oral Symptoms • Dry Mounth > 3 months • Dental caries • Dysphagia • Angular chelitis • Abnormalities of taste & Smell • chronic erythematosis Oral candidiasis • Hoarsness • Enlargement of the parotid & • Submandibular glands

18. Xerostomia

19. Xerostomia

20. Parotid enlargement

23. . Diagnostic tests include sialometry, sialography, andscintigraphy. Newer imaging techniques including ultrasound, MRI or MR sialographyof the major salivary glands are also being used. The labial minor salivary gland biopsy permits histopathologic confirmation of the focal lymphocytic infiltrates.

25. Sialogram

26. Sialogram

27. Sialogram

28. Ocular Manifestation of SS • Aqueus tear Deficiency • Irregularity of corneal surface • Sensation of sand or gravel in the eyes • test by : • Shirmertest • Staining of Rose bengal

29. Congenectivitis

30. Ocular involvement • burning, accumulation of thick strands at the inner canthi, • decreased tearing, redness, itching, eye fatigue, and increased photosensitivity. These symptoms are attributed to the destruction of corneal and bulbar conjunctival epithelium, defined as keratoconjunctivitissicca.

31. Diagnostic evaluation of keratoconjunctivitissicca*measurement of tear flow by Schirmer's I test. Slit-lamp examination of the cornea and conjunctiva after rose Bengal staining reveals punctuate corneal ulcerations and attached filaments of corneal epithelium.

32. Filament

33. Rose bengal staing

34. Keratocongenective

36. Shirmer test

38. Involvement of other exocrine glands occurs less frequently and includes a decrease in mucous gland secretions of the upper and lower respiratory tree,.

39. Lung Involvement in SS • Bronchitis , Bronchiolitis • Interestitial pulmonary fibrosis • Tracheobronchitis Sicca (Dry Cough) • Pleural effusion ( uncommon) • Pseudolymphoma • Pulmonary hypertension ( uncommon)

40. , resulting in dry nose, throat, and trachea (xerotrachea), and diminished secretion of the exocrine glands of the gastrointestinal tract, leading to esophageal mucosal atrophy, atrophic gastritis, and subclinical pancreatitis.

41. Gasterointestinal in SS Cutaneus in SS • Dyspepsia & difficulty in Swalling • Chronic atrophic gastritis • Acute Pancreatic • Chronic active hepatitis • Primary billiary Cirrhosis • Nonspecific Cirrhosis • Allergic drug erupion • Leukoclastic vasculitis • Dry skin ( Pruritus )

42. Dyspareunia due to dryness of the external genitalia and dry skin also may occur.

43. Nervous System in SS • Peripheral Neuropathy ( Sensory ) • Cranial nerve Involvement • Entrapment Syndromes • Transverse Myelitis • MS like lesion • Aseptic meningitis • Encephalopathy • Parasympatic nerve disturbance

44. Renal Involvement in SS Musculoskeletal in SS • Tubulointerestial nephritis • Distal & Proximal renal tubular acidosis • Glomerolonephritis ( uncommon ) • Bladder Symptoms ( Interestitial Cystitis) • Nephrocalcinosis • Arthritis , Arthralgia , Fibromyalgia • Raynaud phenomenon

45. Other Findings in SS • Dypareunia • Hypothyroidism • Autoimmune thyroiditis • Increased prolactin • Vasculitis • 44 fold increase in frequency of B cell • Lymphoma( C3,C4 – new onset leukopenia • Previous ab ( ANA,SS-A, SS-B ) Purpura/skin vasculitis)

46. Differential Diagnosis of Sicca Symptoms • Xerostomia • Viral infections • Drugs •     Psychotherapeutic • Parasympatholytic •     Antihypertensive • Psychogenic • Irradiation • Diabetes mellitus • Trauma • Sjögren's syndrome

47. Revised international Classification Criteria for Siogren Syndrom • 1-Ocular symptoms • 2-Oral symptoms • 3-Ocular Signs • 4-Histopathology of minor labial glands • 5-Salivary gland involvement • 6-Autoantibodies (SSA/Ro , SSB/La) • The presence of any four of the six Items as • long as either item 4 or 6 is positive

48. Classification Criteria for Sjögren'sSyndromea,B, • I. Ocular symptoms: a positive response to at least one of three validated questions.   1. Have you had daily, persistent, troublesome dry eyes for more than 3 months?   2. Do you have a recurrent sensation of sand or gravel in the eyes?   3. Do you use tear substitutes more than three times a day?

49. Classification Criteria for Sjögren'sSyndromea,B,C • II. Oral symptoms: a positive response to at least one of three validated questions.   1. Have you had a daily feeling of dry mouth for more than 3 months?   2. Have you had recurrent or persistently swollen salivary glands as an adult?   3. Do you frequently drink liquids to aid in swallowing dry foods? .

50. . Ocular signs: objective evidence of ocular involvement defined as a positive result to at least one of the following two tests:   1. Shirmer's I test, performed without anesthesia (5 mm in 5 min)   2. Rose Bengal score or other ocular dye score) .