Sjorgren/Scleroderma Flashcards

1
Q

Sjögren Syndrome (SS)

general
whatis primary called

A

Autoimmune, inflammatory condition where glandular tissues, such as the salivary and lacrimal glands, are infiltrated by lymphocytes, resulting in decreased tear and saliva production
Affects ~4 million people in the United States
Predominantly seen in middle-aged women (age 40-55 years of age)

Classification
Primary - known as sicca syndrome
Secondary

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2
Q

Sjorgens

Primary SS

A

Etiology is unknown
Possible genetic predisposition
HLA genes
Possible viral connection
Epstein-Barr virus (EBV)
Chronic hepatitis C
Human T cell leukemia virus 1
Human herpesvirus 6
Human immunodeficiency virus (HIV

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3
Q

Sjogrens

Secondary SS

A

Presents in association with another autoimmune disease
Rheumatoid arthritis
Systemic lupus erythematosus
Polymyositis
Scleroderma
Hashimoto thyroiditis

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4
Q

Sjogren

Patho

A

The exact mechanism of SS is unknown
Process may be set off by an environmental trigger in a patient with a genetic predisposition

Salivary, lacrimal, and other exocrine glands become infiltrated with CD4+T cells and to a less extent with B cells
T cells produce inflammatory cytokines (IL-2, interferon-gamma)

Interferons stimulate B cell activating factor (BAFF) production → promotes irregular B cell maturation → B cells release autoantibodies (Anti-SSA and Anti-SSB)

Cytokines and autoantibodies cause damage and atrophy of the glandular and ductal epithelium → ↓ exocrine production (↓ tear and saliva production)
Other exocrine glands throughout the body can be involved, as well

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5
Q

Sjogrens

Eye Sx

A

Often affects the eyes and mouth initially

Eyes:
Dry eyes (xerophthalmia)
Sandy, gritty sensation
Burning
Redness
Blurry vision
Visual impairment

Advanced disease the cornea can be severely damaged
Epithelial strands hang from the corneal surface (keratitis filiformis)
Corneal ulceration

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6
Q

Sjogrens

Mouth Sx

A

Dry mouth (xerostomia)
Difficulty tasting, chewing, and swallowing
Difficulty speaking for long periods of time
Tooth decay
Parotid gland enlargement

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7
Q

Sjogrens

Cutaneous and other Sx

A

Cutaneous:
Dry skin (xerosis)
Raynaud’s phenomenon
Cutaneous vasculitis
Palpable purpura
Urticarial lesions
Ulcerations
Erythema nodosum -shins
Nasal dryness → bleeding, ulceration and perforation of the nasal septum

Dry cough

Vaginal dryness:
Vulvar infections
Dyspareunia- pain during sex

Can also have respiratory, cardiac, endocrine, neurological, and renal manifestations

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8
Q

Sjogrens

Arthralgia/Arthritis:

A

Similar distribution to rheumatoid arthritis
Nonerosive and nondeforming

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9
Q

Sjogrens

Labs

A

Labs
Anti-SSA/Ro and anti-SSB/La antibodies (60%–70% of patients)
Antinuclear antibodies
Rheumatoid factor (40%–60% of patients)

Complete blood count:
Anemia
Leukopenia
Thrombocytopenia
↑ Erythrocyte sedimentation rate (ESR)

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10
Q

Sjogrens

Ophthalmologic evaluation

A

Schirmer’s test
Measures tear production
Filter paper is placed in the lower eyelid
Wetness is measured after 5 minutes
< 5 mm wetness is considered a positive test

Fluorescein slit-lamp examination
Evaluates the cornea and conjunctiva for damage

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11
Q

Sjogrens

Dx for salivary function

A

Evaluate salivary production
Sialometry
Measures the rate of saliva production
Unstimulated saliva is collected and measured over 15 minutes

Scintigraphy:
Measures salivary gland function through radionuclide uptake
Very low uptake is highly specific for SS

Biopsy
Performed on a lower lip salivary gland
↑↑ CD4+ T-cells
Thickening of the inner duct wall

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12
Q

Sjogrens

Diagnostic Criteria

A

Developed by the American-European Consensus Group 2016

Requires 4 of 6 criteria, including a positive biopsy or autoantibodies

Ocular symptoms(at least 1):
Dry eyes for ≥ 3 months
Foreign body sensation in the eyes
Use of artificial tears ≥ 3 times per day

Oral symptoms(at least 1):
Dry mouth for ≥ 3 months
Recurrent or persistently swollen salivary glands
Need for liquids to swallow dry foods

Ocular signs(at least 1):
Positive Schirmer’s test
Positive dye staining on the surface of the eye

Oral signs(at least 1):
Positive sialography
Positive scintigraphy

Positive biopsy
Positive anti-Ro and/or anti-La

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13
Q

Sjogrens

Primary SS Tx
disease limited to ocular and oral symptoms

A

Multidisciplinary approach (rheumatologist, dentist, and ophthalmologist)

Primary SS- disease limited to ocular and oral symptoms
Oral hygiene
Regular dental visits
Smoking cessation
Artificial tears
Avoidance of drugs that decrease salivary secretions (antihistamines, anticholinergics)
Salivary stimulants (muscarinic agonists)
pilocarpine (Salagen)
cevimeline (Evoxac)

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14
Q

Sjogrens

Systemic Tx

A

Systemic manifestations
Glucocorticoids
Hydroxychloroquine (Plaquenil)

Disease-modifying antirheumatic drugs (DMARDs)
Methotrexate
Leflunomide
Azathioprine
Sulfasalazine
Rituximab for severe disease that does not respond to the above therapies

Secondary SScan be improved by managing the primary autoimmune disease

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15
Q

Sjogrens

Complications of disease

A

Complications
Non-Hodgkin’s lymphoma
Occurs in 5%–10% of patients
40x the normal rate
Usually presents around 7 years after onset

Secondary oral Candida infection
Blindness due to corneal scarring
Renal failure from progressive renal involvement
Pulmonary infections
Reduced quality of life

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16
Q

Systemic Sclerosis

general

A

Also known as scleroderma
Rare, chronic autoimmune disorder in which there is progressive deposition of collagen in the skin, blood vessels, and internal organs causing tightening and fibrosis
♀>♂
Women more likely to present
Younger, with limited disease
Peak age at presentation 35-50 years
Most severe among African-American patients

17
Q

Systemic Sclerosis

Three classifications:

A

CREST syndrome

Generalized systeic sclerosis (w diffuse skin involvement)

Systemic sclerosis sine scleroderma

18
Q

Systemic Sclerosis

Limited systemic sclerosis (CREST syndrome)

A

Calcinosis cutis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly (tight skin over digits), Telangiectasias
Characterized by slow progression and is often complicated by pulmonary hypertension

19
Q

Systemic Sclerosis

Generalized systemic sclerosis (with diffuse skin involvement)

A

Raynaud phenomenon and gastrointestinal (GI) complications

Evolves rapidly

Interstitial lung diseaseand scleroderma renal crisis are the major complications

20
Q

Systemic Sclerosis

Systemic sclerosis sine scleroderma

A

Systemic sclerosis–related antibodies and visceral manifestations of the disease but no skin tightening

21
Q

Systemic Sclerosis

etiology

A

Etiology is unknown
Genetics likely play a role

Suspected environmental triggers include
Infectious (cytomegalovirus (CMV), herpesvirus, parvovirus B-19)
Environmental
Silica exposure
Solvents (vinyl chloride, benzene)
Drugs (bleomycin)
Radiation exposure

22
Q

systemic sclerosis

patho

A

3 interlinked complex processes are involved:

Abnormal activation of both humoral and cell-mediated immunity→ autoimmune disorder
Microvascular endothelial injury likely caused by anti-endothelial antibodies
Perivascular accumulation of T and B lymphocytes, macrophages, and fibroblast precursors
Secretion of cytokines and growth factors by inflammatory cells

Abnormal stimulation of fibroblast activation and proliferation within small arteries and arterioles

Progressive deposition of collagenand other extracellular matrix molecules within the skin and internal organs

23
Q

systemic sclerosis

Skin S/Sx

A

Raynaud phenomenon (most common initial symptom) often leading to ulceration

Skin and nail manifestations:
Swelling of the skin
Symmetric
Gradual thickening of the skin
Confined to the fingers (sclerodactyly) causes the fingers to curl inward
Face becomes mask-like
Perioral skin → reduced mouth opening

Telangiectasia on the fingers, chest, lips, and tongue

Subcutaneous calcifications
Most common over the fingertips and bony eminences

Abnormal capillary and microvascular loops in the nails

24
Q

Scleroderma

A
25
Q

Scleroderma

Constitutional/GI S/Sx

A

Constitutional symptoms:
Fever
Fatigue
Weight loss

Gastrointestinal (90%):
Dysphagia due to esophagus dysfunction (most common visceral symptom)
Gastroesophageal reflux disease → Barrett esophagus in 1/3 of patients

Hypomotility of the small bowel causing bacterial overgrowth → malabsorption

Vascular ectasia - “watermelon stomach

Blood vessels in the lining of the stomach become fragile and are prone to rupture and bleeding

26
Q

Scleroderma

Renal/MSK S/Sx

A

Renal:
Hypertension
Chronic kidney disease
Scleroderma renal crisis:
Life-threatening acute renal failure
Malignant hypertension

Musculoskeletal:
Polyarthralgias/arthritis
Flexion contractures
Fingers, wrists, and elbows
Tendon friction rubs
Tendinitis

27
Q

Scleroderma

Pulm & Cardio S/Sx

A

Pulmonary (80%):
Interstitial lung disease (ILD) → pulmonary fibrosis
Pulmonary arterial hypertension

Pulmonary embolism

Cardiac:
Restrictive cardiomyopathy
Constrictive pericarditis
Arrhythmias

28
Q

scleroderma

A

9+ is positive

The total score is determined by adding the maximum weight (score) in each category
Patients with a total score ≥9 are classified as having definite scleroderma

The European Alliance of Associations for Rheumatology formerly the European League Against Rheumatism

29
Q

Scleroderma

labs and antibodies

A

Antinuclear antibodies (ANA)
Nucleolar pattern
90% of patients

Rheumatoid factor
Positive in 1/3 of patients

Antibodies
Anti-centromere (ACA) = limited disease (CREST)

Anti-Scl 70 or anti-DNA topoisomerase I = diffuse disease
Increased mortality and greater incidence of interstitial lung disease
Anti-RNA polymerase III (ARA) = diffuse disease

30
Q

Scleroderma

Suspected lung involvement Diagnostics

A

Pulmonary function testing (PFT)
Chest CT
Assessing interstitial lung abnormalities
Echocardiography
Screening for PAH

Right heart catheterization
Gold standard to confirmpulmonary arterial hypertension

31
Q

Scleroderma

Tx

A

There is no curative treatment

Management goals
Minimize symptoms
Delay the progression of organ-specific complications

Immunosuppressants: methotrexate, azathioprine, cyclophosphamide

Glucocorticoids- Used for myositis. Should be avoided if there is renal disease

NSAIDs for pain- Should be avoided if there is renal disease

Raynaud phenomenon-Calcium channel blockers
GERD- H2 blockers or proton-pump inhibitors
Esophageal strictures- Periodic dilation

Period screenings every 1-2 years
Pulmonary hypertension with PFT
Echocardiography