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Flashcards in Connective Tissue Disorders Deck (59)
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1

Connective Tissue Disorders

Sjogren's syndrome
Scleroderma
Raynaud's phenomenon
Myositis/Dermatomyositis
CREST syndrome

2

Define Sjogren's Syndrome (SS)

Chronic inflammatory disorder characterized by diminished lacrimal & salivary gland function

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Primary Sjogren's Syndrome

Not associated with other disease

4

Secondary Sjogren's Syndrome

Complication of other rheumatic conditions (RA, scleroderma, lupus)

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Pathogenesis of Sjogren's Syndrome

Lymphocytic infiltration into lacrimal & salivary glands, where lymphocytes are not normal found
Impairs function

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Symptoms of Mild Sjogren's Syndrome

Dry eyes
Dry mouth
Fatigue
Myalgias
Cognitive dysfunction

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Symptoms of Severe Sjogren's Syndrome

Florid salivary gland enlargement
Adenopathy
Antibodies to Ro/SSA & La/SSB antigens
Cryoglobulinemia
Hypocomplementemia
Propensity to develop non-Hodgkin's lymphoma

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Extraglandular Involvement in Sjogren's Syndrome

Vaginal dryness
Dyspareunia
Interstitial nephritis
Recurrent thirties & sinusitis
Bronchial dryness

9

Define Karatoconjunctivitis Sicca

Deficiency in tear production leading to symptoms of dry eyes which usually presents insidiously overall several years

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Complications of Karatoconjunctivitis Sicca

Corneal ulceration
Infection of the eyelids

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Clinical Manifestations of Sjogren's Syndrome

Karatoconjuncitivitis sicca
Xerostomia
Salivary gland enlargement

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Complications of Xerostomia

Dental caries
Oral candidiasis
Laryngotracheal reflux
Chronic esophagitis
Weight loss
Nocturia

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Salivary Gland Enlargement in Sjogren's Syndrome

Firm, diffuse, & non-tender
Occur in parotid & submandibular glands

14

Criteria for Diagnosis of Sjogren's Syndrome

Ocular symptoms of inadequate tear production
Ocular signs of corneal damage
Oral symptoms of decreased saliva production
Salivary gland histopathology demonstrating foci of lymphocytes
Tests indicating impaired salivary gland function
Presence of autoantibodies (Anti-Ro/SSA & Anti-La/SSB)

15

Exclusion Criteria for Sjogren's Syndrome

Prior head/neck irradiation
Infection with hepatitis C
AIDS
Lymphoma
Sarcoidosis
Graft-vs-host disease
Recent use of anti-cholinergic meds

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Primary Sjogren's Syndrome Diagnosis Criteria

Positive salivary gland biopsy or anti-Ro/SSA and/or anti-La/SSB & satifies 4/6 criteria
Satisfies 3/4 objective items (ocular signs, biopsy, salivary gland involvement, autoantibodies)

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Secondary Sjogren's Syndrome Diagnosis Criteria

CTD present
One item indicative of ocular or oral dryness
2/3 objective items

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Diagnosing of Sjogren's Syndrome

Thorough H&P
Schirmer test
Rose bengal test
Labs: CBC w/diff, CMP, ESR, UA

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Specialized Lab Testing for Sjogren's Syndrome

ANA
RF
Antibodies to Ro/SSA, La/SSB, Sm, & RNP antigens
Antibody to anti-centromere B or topoisomerase
ANCA (vasculitis)
Serum C3 & C4
Antibodies to dsDNA
Serum cryoglobulins
SPE
Ig levels

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Diagnosis of Sjogren's Syndrome

Salivary gland biopsy
Antibodies to Ro/SSA
Antibodies to La/SSB

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Treatment of Sjogren's Syndrome

Topical therapy for dry eyes & dry mouth
Treatment of systemic manifestations

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3 Aspects of Treating Sicca Symptoms

General principles
Xerostomia
Karatoconjunctivities

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General Treatment Principles of Sicca Symptoms

Avoidance of irritants
Avoidance of exacerbating drugs
Avoidance of low humidity atmospheres
Consider candidiasis if a painful mouth
Consider blepharitis if adequate hydration
Excellent oral hygiene
Topical moisturizers & lozenges

24

Treating Xerostomia

Stimulation of existing salivary flow: sugarless candy, dried fruit slices, citrus flavored tablets, gum
Muscarinic agonists
Artificial saliva

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Treating Keratoconjunctivities (KCS)

Replacing or supplementing deficient tears

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Immunosuppressive Treatment of Systemic Sjogren's

Hydroxychloroquine
Methotrexate

27

Define Scleroderma

Thickened, hardened skin

28

Spectrum of Scleroderma

Limited anatomic extent affecting only the skin & adjacent tissues
Associated with systemic involvement

29

Pathophysiology of Systemic Sclerosis

Vascular damage
Immune activation
Excessive synthesis of extracellular matrix with deposition of structurally normal collagen results

30

Vascular Damage in Systemic Sclerosis

Vasoconstriction
Endothelia's are fibrinogenic
Sera found to be cytotoxic to endothelial cells