Autoimmune and Connective Tissue Disorders

By:Dr. Kazhan Ali Tofiq Kadir

April 2011

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

Systemic Lupus Erythematosus– specific labs -

native(Double stranded) DNA, SM antigen

– ANA test positive in 95%– LE cells

SLE - Diagnostic Criteria

SLE

• Head and Neck Manifestations– Malar rash first sign in 50% (Butter Fly Rash)– Erythematous maculopapular eruption after sun

exposure– Oral ulceration

• 3-5% perforated nasal septum• larynx and trachea involvement rare• Vocal Cord thickening, paralysis, cricoarytenoid

arthritis, subglottic stenosis• acute parotid enlargement 10%

-xerostomia occars-neuropathy 15%-discoid lupus

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

DLE: Discoid Lupus Erythematosus:

• Benign• Face (most frequently)

• Patches: disc likevarious 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

Precipitating factors: in DLE Acronym: (ITEM)

1. Infection 2. Trauma 3. Exposure to cold4. Mental stress.

* Sunlight is exacerbating factor.

Histopathology of DLE: a. Liquefaction degeneration of the basal cell layer

b. Hyalinization, Edema & Fibrinoid change below epidermis.c. A patchy dermal lymphocytic infiltrate around the appendages

Symptoms:- Rash (Patches) + - history of Raynaud’s phenomenon, chilblains or poor peripheral circulation Signs: - The Face is most commonly affected & the scalp, ears, nose, arms, legs & trunk to a lesser extent.-The circumscribed or discoid type is the most frequent & occurs

particularly on the cheeks, the bridge of the nose, the ears, the side of the neck & the scalp.

- Unilateral, may be bilateral. - Permanent alopecia + well defined erythematous patches, varying in

size, - Scale: when the scale is removed its undersurface on direct

examination shows horny plugs (which have occupied dilated pilosebaceous canals).

Raynaud’s phenomenon

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. Rosacea8. Necrobiosis Lipoidica.9. Lupus vulgaris (skin TB).

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

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. LE2. Morphea (Localized or Generalized) 3. Systemic sclerosis 4. L.S.A5. Dermatomyositis

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

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 commonPigmentation occurs in a bout 50% of the patients. Leg ulcers occur in 40% of the patients.

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.

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.

• 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.

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

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

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%

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

Sjogren’s Syndrome

• Head and Neck Manifestations– salivary

quantification- salivary scintigraphy

– salivary gland enlargement

Sjogren’s Syndrome

– eye complaints• dryness,

burning, itching, foreign body sensation

– keratoconjunctivitis sicca• corneal

abrasions - rose bengal staining

Sjogren’s Syndrome

– Schirmer I– Schirmer II– nasal crusting, epistaxis,

hyposmia– Diagnostic:

• history>• Schirmer I>Schirmer II or

rose bengal staining or other supportive evidence:

• minor salivary gland biopsy

Sjogren’s Syndrome

• Treatment– symptomatic• oral fluid intake• saliva substitutes• artificial tears

– avoid• decongestants• antihistamines• diuretics• anticholinergic

Sjogren’s Syndrome

• Treatment– pilocarpine– clotrimazole/nystatin– close dental

supervision– surveillance for

malignancy

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

Behcet’s Disease

– occular• uveitis, iritis• hypopyon

– healing in days to weeks some scarring

– symptoms simultaneously, months apart

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

Thank You