Sjorgren/Scleroderma Flashcards
Sjögren Syndrome (SS)
general
whatis primary called
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
Sjorgens
Primary SS
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
Sjogrens
Secondary SS
Presents in association with another autoimmune disease
Rheumatoid arthritis
Systemic lupus erythematosus
Polymyositis
Scleroderma
Hashimoto thyroiditis
Sjogren
Patho
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
Sjogrens
Eye Sx
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
Sjogrens
Mouth Sx
Dry mouth (xerostomia)
Difficulty tasting, chewing, and swallowing
Difficulty speaking for long periods of time
Tooth decay
Parotid gland enlargement
Sjogrens
Cutaneous and other Sx
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
Sjogrens
Arthralgia/Arthritis:
Similar distribution to rheumatoid arthritis
Nonerosive and nondeforming
Sjogrens
Labs
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)
Sjogrens
Ophthalmologic evaluation
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
Sjogrens
Dx for salivary function
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
Sjogrens
Diagnostic Criteria
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
Sjogrens
Primary SS Tx
disease limited to ocular and oral symptoms
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)
Sjogrens
Systemic Tx
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
Sjogrens
Complications of disease
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