68 - Sjogren Syndrome Flashcards by dvojnd rivera

Chronic systemic autoimmune disorder characterized by lymphocytic infiltration and destruction of exocrine glands and epithelia leading to dry mouth, dry eyes, and B lymphocyte hyperreactivity

Sjogren syndrome

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Isolated SS disorder

Primary Sjogren syndrome

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SS associated with other autoimmune condition

Secondary Sjogren syndrome

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SS predominantly affects

Women

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SS patients are most commonly diagnosed in their _____ decades of life

Fourth and fifth

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Genetic loci significantly associated with pSS

HLA DQA1*0501

HLA DQB1*0201

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Important transcription factor involved in upregulating the Type I IFN pathway, which is activated by toll-like receptor signals or viral infection and the production of inflammatory cytokines

IRF5

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Protein involved in the Type II IFN pathway and activating the adaptive immune system

STAT4

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_____ mutation has been correlated with an increased risk of lymphoma

TNFAIP3

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Thought to be the first and main target in pSS

Salivary glands

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The role of the salivary gland epithelial cells in pSS has been established. Studies have indicated that epithelial cells play an active role in disease pathogenesis and this is referred to as

“Autoimmune epithelitis”

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Best-defined autoantibodies in SS

Anti-Ro/SSA

Anti-La/SSB

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Recognizes 2 RNA-binding proteins (the 52-kDa or the 60-kDa protein)

Anti-Ro/SSA

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Recognize RNA polymerase III

Anti-La/SSB

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Found in more than 70% of patients with SS but are not specific for SS and are frequently found in SLE and other autoimmune diseases

Anti-Ro/SSA

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More specific for SS

Present in 50% of patients with pSS or SS/SLE but is rarely seen in other diseases

Anti-La/SSB

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Frequently present in patients with both and primary SS

Although they lack specificity, they are markers of a systemic autoimmune response

Antinuclear antibodies

Rheumatoid factor

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Major stimulus for saliva production

Binding of acetylcholine to muscarinic acetylcholine receptors

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A small group of patients positive for anticentromere autoantibody (ACA) present a clinical picture similar to that of

Limited scleroderma

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The ACA-positive group usually has a higher prevalence of

Raynaud phenomenon

Thyroid dysfunction

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The ACA-positive group also shows a higher frequency of

Vasculitis
Peripheral neuropathy
Primary biliary cirrhosis

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Positivity for antimitochondrial antibody (AMA) also has been demonstrated to be related to

Primary biliary cirrhosis

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Sjogren syndrome is a multifocal autoimmune disease, with systemic involvement in _____ of patients

One-third

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The characteristic feature of SS is exocrine gland dysfunction, leading to classic sicca symptoms of

Xerostomia (dry mouth)

Xerophthalmia or keratoconjunctivitis sicca (dry eyes)

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Medical causes of oral dryness, such as _____, should be considered when evaluating a patient for Sjogren syndrome

Dehydration Diabetes Viral infections Drug treatment

Flat, nonpalpable, blanching purpura Characterized by polyclonal hypergammaglobulinemia and rheumatoid factor positivity Skin biopsies reveal ruptured blood vessels with complement deposition

Hypergammaglobulinemic purpura

Y/N: Palpable purpura represents an important marker of more severe disease and is associated with an increased risk of lymphoma development and mortality

Yes

Histopathologically, palpable purpura can be divided into 2 groups

Neutrophilic inflammatory vascular disease | Mononuclear inflammatory vascular disease

Predominantly neutrophilic infiltrate, fibrinoid necrosis, occlusion of the lumen, and extravasation of red blood cells, and is indistinguishable form classical leukocytoclastic vasculitis

Neutrophilic inflammatory vascular disease

Mononuclear inflammatory infiltrate, with invasion of the blood vessel walls

Mononuclear inflammatory vascular disease

The clinical presentation of the 2 forms of palpable purpura are indistinguishable, but _____ is associated more strongly with markers of systemic autoimmunity

Neutrophilic inflammatory vascular disease

Second most frequent form of cutaneous vasculitis in SS

Urticarial vasculitis

Present as palpable purpuric lesions of the lower extremities, which may ulcerate, finally resolving within 1 to 4 weeks

Necrotizing vasculitis

Annular erythema associated with pSS is found primarily among

Asian pSS patients who have anti-Ro/SSA and anti-La/SSB antibodies

Erythematous lesion with a wide elevated border and central clearing Localized mainly on the faces of Asian patients

Annular erythema associated with pSS

May be triggers of the lesions of annular erythema associated with pSS

Sunlight Cold exposure Mental stress Pregnancy

Annular erythema associated with pSS shares a clinical presentation with

Subacute cutaneous lupus

On histopathology of (annular erythema associated with pSS/SCLE), the dense cell infiltration is localized mainly around sweat glands, and nuclear dust can also be seen when they have vasculitis

Annular erythema associated with pSS

(Annular erythema associated with pSS/SCLE) are common in Asians, but rare in whites, whereas (annular erythema associated with pSS/SCLE) are more common in whites but rare in Asians

Annular erythema associated with pSS | SCLE

Presence of erythema nodosum should raise the suspicion for

Sarcoidosis

Probably the most common abnormality in SS | Can precede sicca symptoms by many years

Raynaud phenomenon

Raynaud phenomenon in SS is not accompanied by _____, as seen in systemic sclerosis

Telangiectasias

Although Raynaud phenomenon is usually mild in pSS, it is a marker of a subgroup with increased

Extraglandular manifestations

The absence of rheumatoid factor and anti-CCP antibodies, and the absence of erosions on radiographs would favor SS over

Rheumatoid arthritis

Thyroid dysfunction is a very common symptom, occurring in 10% to 70% of SS patients. It often presents as

Fatigue

Most common manifestation of peripheral nervous system involvement in SS

Peripheral axonal polyneuropathies, which are typically sensory

Most common form of cranial neuropathy in SS

Unilateral trigeminal neuropathy

The incidence of lymphoma in SS patients is increased _____-fold

15 to 44

Y/N: Recent studies have suggested that women with pSS may experience more complications in pregnancy than healthy controls

Yes - significant increases in the rates of spontaneous abortions, preterm deliveries, and low-body-weight infants

Anti-Ro/SSA and anti-La/SSA can cause _____ in the fetus

Congenital heart block | Neonatal lupus

Characterized by an annular rash with central regression or mild atrophy in the scalp and around the eyes, as well as hepatic and hematologic abnormalities

Neonatal lupus

Involves measurement of the total saliva produced from all salivary glands in a time period of 15 min

Sialometry

The whole unstimulated salivary flow is considered suggestive of SS if it is

<1.5 mL in 15 minutes

A "stimulated" salivary flow can be measured after administration of

Lemon juice or citric acid

Functional study to assess saliva production by measuring the secretion of a radioisotope into the oral cavity

Salivary gland scintigraphy

Can potentially distinguish between decreased production and/or decreased excretion of saliva

Salivary gland scintigraphy

Imaging method based on retrograde injection of contrast media into the parotid duct

Sialography

Performed by placing a standardized paper strip in the inferior fornix of each eye and measuring the length of filter paper that becomes wet after 5 minutes

Schirmer test

The American-European classification system uses a cut-off value for the Schirmer test of

5 mm in 5 minutes

Dye that preferentially stains the devitalized cornea and conjunctiva

Rose Bengal or lissamine green

The most reliable objective diagnostic feature of SS is seen on

Biopsy of the minor salivary gland

There are 2 patterns of pSS that define disease categories with very different clinical risks

``` Type I (High-risk) Type II (Low-risk) ```

Patients with _____ may be classified as high-risk disease syndrome (Type I)

Low complement C4 levels and/or | Palpable purpura early in their disease course

The high-risk disease syndrome group carries a significantly increased risk of _____; it also has an increased _____

Lymphoproliferative disease | Mortality rate

Two drugs approved for the treatment of dry mouth

Pilocarpine 5 mg 4 times/d | Cevimeline 30 mg 3 times/d

Pilocarpine and cevimeline are contraindicated in

Narrow-angle glaucoma | Uncontrolled asthma

Cholinergic side effects

Excessive sweating Urinary frequency Flushing Headaches

Has been approved by the FDA for the treatment of keratoconjunctivitis sicca

Cyclosporine 0.05% ophthalmic solution

Most SS-associated lymphomas are

Low-grade B cell-lymphomas, localized to the exocrine glands