The lecture has been given on Apr. 3rd, 2011 by Dr. Kazhan.

1. Autoimmune and Connective Tissue Disorders By: Dr. Kazhan Ali Tofiq Kadir April 2011

2. Connective Tissue Disorders: The cardinal feature of these conditions is inflammation of the CT which leads to dermal atrophy or sclerosis, arthritis and S.T. to abnormalities in other organs. Basic to them all is a complex array of immune responses that target or affect collagen or ground substances. Lupus Erythematosus General autoimmune multisystem disease prevalence 1 in 2,000 9 to 1; female to male (1 in 700) peak age 15-25 immune complex deposition photosensitive skin eruptions, serositis, pneumonitis, myocarditis, nephritis, CNS involvement

3. Systemic Lupus Erythematosus specific labs - native(Double stranded) DNA, SM antigen ANA test positive in 95% LE cells

4. SLE - Diagnostic Criteria

6. larynx and trachea involvement rare

7. Vocal Cord thickening, paralysis, cricoarytenoid arthritis, subglotticstenosis

9. SLE Treatment avoid sun exposure NSAIDS topical and systemic steroids antimalarials low-dose methotrexate azothioprine, cyclophosphamide restricted symptomatic treatment saliva substitutes, Klack’s solution postprandial rinses with H2O2 and H2O

10. DLE: Discoid Lupus Erythematosus: Benign Face (most frequently) Patches:disc like various sized , reddish, well- defined, scaly (adherent ) with hyperkeratosis which tend to heal with atrophy, scarring& pigmentary changes. Hematological & Serological changes in about half (½) the patients, Female: male is 2: 1 Age of onset is 15 – 40 years

11. Precipitating factors: in DLE Acronym: (ITEM) 1. Infection 2. Trauma 3. Exposure to cold 4. Mental stress. * Sunlight is exacerbating factor.

12. Histopathology of DLE: a. Liquefaction degenerationof the basal cell layer b. Hyalinization, Edema & Fibrinoid change below epidermis. c. A patchy dermal lymphocytic infiltrate around the appendages

15. Scale: when the scale is removed its undersurface on direct examination shows horny plugs (which have occupied dilated pilosebaceous canals). Raynaud’s phenomenon

16. D. D. of DLE: - 1. Morphea 2. LSA ( Lichen Sclerosus et Atrophicus): 3. PLE (polymorphic light eruptions). 4. Contact Eczema 5. Seborrheic. Ecz. 6. Psoriasis 7. Rosacea 8. Necrobiosis Lipoidica. 9. Lupus vulgaris (skin TB).

17. Treatment of DLE: 1. General measures: The patients should avoid excessive exposure to sunlight They should wear a broad – brimmed hat & avoid short- sleeved shirts & shorts. They should use a sun- screening cream & lotion. Sunscreens are not for treatment once a reaction has occurred. Patients who note exacerbation of their lesions with the cold should warp up well in the winter Nifedipine may be helpful in those with Raynaud’s phenomenon. Small dose of sedative in mental stress. 2. Topical therapy: Moderately potent steroid cream twice daily. 3. Intralesional Cs injects are helpful in resistant cases. 4. Oral antimalarials: chloroquine tab. ½ tab. twice a day for 4- 6 wks

18. Morphea Definitions: Sclerosis: is diffused or circumscribed INDURATION of the S. C. tissue may also involve the dermis, when the overlying epidermis may be atrophic. Scleroderma: - ↑ed depostion collagen in interstitium of small arteries and CT - sclerotic skin changes, often multisystem disease - is sclerosis of the skin occurs in: 1. LE 2. Morphea (Localized or Generalized) 3. Systemic sclerosis 4. L.S.A 5. Dermatomyositis

19. Localized Morphea: Prevalence: 3 Females to 1 male Localized sclerosis of the skin of unknown cause; 3 clinical types: Circumscribed, Linear & Frontoparietal: (en coup de saber) with or without hemiatrophy of the face. Clinical Features: The lesions: -indurated purplish, spontaneous, usually multiple, round or oval, 2-15 cm - lose their color, become -> thickened & waxy areas, ivory in color with a characteristic lilac- colored edge, surface is usually smooth & shiny - in the lesion the hairs are absent, there will be No sweating - on the trunk & limbs, face & genitalia. - activity is usually between 3 –5 years & spontaneous resolution -can be generalized. Treatment: local steroid, or Intralesional Steroid

20. Systemic sclerosis: (Systemic scleroderma): Face & Hands (most frequently) but the change may extend proximally to involve the forearms & upper arms, Face:facial appearance: is characteristic: - - The forehead is smooth & shiny - The forehead lines are expressless. - The nose becomes small & pinched. - The mouth opening is constricted & radial furrows appear. - Small, mat – like telangiectases are frequently found on the face, occur in 75% of patients. Hands & forearms forearm’s skin will be thinner than normal. Atrophy occurs 1st in the pulps of the fingers & small painful ulcers are formed, heal with scars, the nails are curving over the atrophic phalanges. Paronychia is common Pigmentation occurs in a bout 50% of the patients. Leg ulcers occur in 40% of the patients.

21. Lichen sclerosis et atrophicans (L. S. A): - * uncommon *small, white areas on the skin associated with an atrophic condition of the vulva & perianal skin. Clinical features: 1- Non – genital L S A: - on the trunk, axillae & flexor surface of the wrists. - small, ivory white, shiny & round macules or papules, (a few mms in diameter) - later atrophy & the surface of the lesions wrinkled. 2- Anogenital LSA in women between 45 – 60 - Irritation may be marked - on the vulva & around the anus. - ivory – colored atrophic papules with follicular hyperkeratosis on the vulva. DDx: 1. Morphea 2. L.P. 3. DLE. Treatment: topical Cs may be of help.

22. Dermatomyositis:Is a disorder mainly of skin, muscle & B.V.s Characteristic Erythematous & Edematous changes in the Skin + Muscle Weakness & Inflammation. in childhood Calcinosis is frequent & is usually ass. with a more favorable prognosis for life. In adults:commonly ass. with an underlying carcinoma or reticulosis - Usually the patient 1st notices aching & weakness of the muscles as difficulty in going upstairs or rising from a chair or in women difficulty in raising the arms high enough to comb the hair.

23. The rash in Dermatomyositis is diagnostic: - Face: A purplish- red heliotrope erythema occurs on the face, especially involving the eyelids, the upper cheeks, the forehead & the temples associated with the edema of the eyelids & periorbital tissues that is why it should be differentiated from Contact Dermatitis (CD) of the face. - Hands: Erythematous changes occur on the backs of fingers especially over the backs of the Joints & the knuckles.

24. Sjogren’s Syndrome General immune mediated destruction of exocrine glands primary sicca syndrome- isolated d/o lacrimal and salivary glands secondary sicca complex- assoc. with other CTD

25. Sjogren’s Syndrome General prevalence 1% population 10-15% of RA patients 9 to 1 F>M onset 40-60 yrs increased risk of lymphoma perhaps 44 times risk

26. Sjogren’s Syndrome General clinical manifestations xerophthalmia, keratoconjunctivitis xerostomia other areas skin, vagina, genitalia, chronic bronchitis, GI tract, renal tubules diagnosis minor salivary gland biopsy labs RF and ANA SS-A/Ro 60% SS-B/La 30%

27. Sjogren’s Syndrome Head and Neck Manifestations 80% xerostomia, most prominent symptom difficulty chewing, dysphagia, taste changes, fissures of tongue and lips, increased dental caries, oral candidiasis

28. Sjogren’s Syndrome Head and Neck Manifestations salivary quantification- salivary scintigraphy salivary gland enlargement

29. Sjogren’s Syndrome eye complaints dryness, burning, itching, foreign body sensation keratoconjunctivitis sicca corneal abrasions - rose bengal staining

30. Sjogren’s Syndrome Schirmer I Schirmer II nasal crusting, epistaxis, hyposmia Diagnostic: history> SchirmerI>Schirmer II or rose bengal staining or other supportiveevidence: minor salivary gland biopsy

31. Sjogren’s Syndrome Treatment symptomatic oral fluid intake saliva substitutes artificial tears avoid decongestants antihistamines diuretics anticholinergic

32. Sjogren’s Syndrome Treatment pilocarpine clotrimazole/nystatin close dental supervision surveillance for malignancy

33. Behcet’s Disease Vasculitis with triad oral, genital ulcers, uveitis or iritis oral aphthous-like painful, clusters on lips, gingiva, buccal, tongue less often palate, oropharynx genital similar in appearance

34. Behcet’s Disease occular uveitis, iritis hypopyon healing in days to weeks some scarring symptoms simultaneously, months apart

35. Behcet’s Disease other findings tinnitus, vertigo nasal, laryngeal, tracheal mucosal ulceration CNS involvement, bowel dysfunction, large vessel arteritis treatment azothioprine, methotrexate possibly, not documented

36. Thank You