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Questions and Answers
What is the recommended frequency for annual screening after 5 years of hydroxychloroquine use, provided there are no major risk factors?
Which of the following tests is considered ineffective for hydroxychloroquine retinopathy screening?
Which of the following provides a diagnostic criterion for Giant Cell Arteritis (GCA)?
Which laboratory test is specifically associated with diagnosing Lupus?
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What is a primary management strategy for Arteritic Anterior Ischemic Optic Neuropathy (AAION)?
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Which autoimmune disorder is primarily associated with the presence of Anti-Smith antibodies?
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What test is essential for the diagnosis of Sjogren’s Syndrome (SS) that receives the highest points in diagnostic criteria?
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What does a positive result for HLA-B27 typically indicate?
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In the context of Systemic Lupus Erythematosus (SLE), what ocular effect is specifically mentioned?
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What is the suggested treatment protocol regarding temporal artery biopsy in GCA management?
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Which test is utilized for the detection of Wegner’s granulomatosis?
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Which statement about the systemic complications of Rheumatoid Arthritis (RA) is accurate?
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In a patient with a positive ANA test, which antibodies would be ordered to test specifically for Sjogren’s Syndrome?
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What is the typical clinical presentation of a patient with Arteritic Anterior Ischemic Optic Neuropathy (AAION)?
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Which laboratory test is commonly used to monitor general inflammation in autoimmune conditions?
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Which disease is linked to the presence of Anti-Jo antibodies?
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Which of the following is NOT a common association with the Rheumatoid Factor (RF) test?
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Study Notes
What Are Autoimmune Disorders?
- Autoimmune disorders occur when the immune system mistakenly attacks the body's own tissues.
- These disorders can affect any organ system, causing a variety of symptoms.
How Do We Diagnose Autoimmune Disorders?
- Laboratory tests help identify autoimmune disorders and other conditions.
Laboratory Tests
- Rheumatic Factor (RF) is primarily associated with Rheumatoid Arthritis (RA) but also seen in other conditions like Sjogren's Syndrome, Lupus, and Connective Tissue Disorders.
- Anti-citrullinated protein antibodies (ACPAs or anti-CCPs) are primarily associated with Rheumatoid Arthritis (RA) but are also found in Sjogren's Syndrome, Psoriatic arthritis, and Leukemia.
- Antinuclear antibodies (ANAs) are strongly associated with Lupus but can also be seen in Scleroderma, Sjogren’s Syndrome, Connective Tissue Disorders, TB, Syphilis, Cancer, Grave's Disease, Inflammatory Bowel Disease (IBD), and Myositis.
- Anti-double stranded DNA antibodies (anti-dsDNA) are specific to Lupus.
- Anti-neutrophil cytoplasmic antibody (ANCA) is associated with Wegner's granulomatosis and other vasculitides, but can also be seen in other autoimmune diseases.
- Erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) are elevated in Giant Cell Arteritis (GCA) and other autoimmune conditions.
- HLA-B27 is associated with Ankylosing Spondylitis and other autoimmune conditions.
- Anti-SSA/SSB antibodies are associated with Sjogren's Syndrome and Lupus.
- Anti-Smith antibodies are specific to Lupus.
- Anti-ribonuclearprotein (RNP) antibodies are associated with Lupus and connective tissue disorders.
- Anti-Scl antibodies are associated with Scleroderma.
- Anti-Jo antibodies are associated with Inflammatory Myositis.
How Are Autoimmune Disorders Generally Treated?
- Treatment aims to reduce inflammation, manage symptoms, and prevent organ damage.
- Medications like hydroxychloroquine may be used to suppress the immune system.
Hydroxychloroquine Retinopathy Testing Guidelines
- Baseline examination should occur within 1 year of starting hydroxychloroquine.
- Annual screening is recommended after 5 years of drug use, unless there is maculopathy or major risk factors.
- More frequent screening is needed if major risk factors are present.
- Primary testing includes automated visual fields, OCT of the macula, and dilated fundus exam.
- Adjunctive testing might include multifocal ERG and fundus autofluorescence.
- Fluorescein angiography, full-field ERG, EOG, Amsler grid, and color vision testing are generally ineffective.
Non-Infectious Uveitis and Autoimmune Disorders
- Many autoimmune disorders can cause uveitis, an inflammation of the middle layer of the eye.
Giant Cell Arteritis (GCA)
- GCA is an inflammatory condition affecting medium-sized arteries, particularly the temporal artery in the head.
- It is primarily classified as a vasculitis, affecting mainly the arteries of the head.
- Can cause vision loss, but more importantly, the artery can rupture and cause a life-threatening bleed in the brain.
- Oral steroids are the primary treatment.
- GCA diagnosis is challenging and requires prompt treatment.
GCA Signs and Symptoms
- Headache, scalp tenderness, jaw claudication (pain in the jaw muscles), and visual changes in the affected eye.
GCA Diagnosis
- Diagnosed based on symptoms, laboratory findings, and temporal artery biopsy.
- Elevated ESR and CRP levels can be seen in other conditions, so they are not diagnostic alone.
- A temporal artery biopsy is the gold standard for diagnosis, but it can be inconclusive.
GCA Diagnostic Criteria
- Age at onset greater than 50 years old.
- New headache.
- Temporal artery abnormalities (tenderness to palpation or decreased pulsation).
- ESR of ≥50 mm/hr by Westergren method ( > 100 mm/hr is usually indicative of GCA).
- Abnormal Temporal Artery Biopsy showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells.
Arteritic Anterior Ischemic Optic Neuropathy (AAION)
- A sudden loss of vision in one eye due to inflammation of the optic nerve.
- Often occurs with GCA.
- Prognosis is not good in the affected eye, but treatment helps prevent blindness in the other eye.
- Prompt treatment is essential for better outcomes.
AAION Signs
- Loss of peripheral vision.
AAION and GCA Management
- Oral or IV steroids are the primary treatment.
- Temporal artery biopsy: within two weeks of starting treatment.
- Close monitoring of ESR/CRP.
- Biologics or DMARDs are used for those who cannot take long-term steroids.
- Treat complications arising from treatment.
- Ongoing monitoring by primary care, rheumatology, and/or neuro-ophthalmology.
Systemic Lupus Erythematosus (SLE)
- A chronic autoimmune disease affecting multiple organ systems.
Lupus Diagnostic Criteria
- Diagnosed based on a combination of clinical and laboratory findings.
- Symptoms range from mild to life-threatening.
Other Systemic Lupus Complications
- Lupus nephritis, arthritis, pleuritis, pericarditis, and vasculitis can occur.
Lupus Management
- Managing symptoms and preventing organ damage.
- Medications include corticosteroids, immunosuppressants, and antimalarials.
Ocular Effects of Lupus
- Uveitis, retinal vasculitis, and dry eye disease are common.
Lupus Retinopathy
- More common than other retinal complications in lupus.
Rheumatoid Arthritis (RA)
- A chronic autoimmune disease affecting joints, causing inflammation, pain, and stiffness.
- Can also affect other organs.
RA Diagnostic Criteria
- Diagnosed based on symptoms, physical examination, and laboratory findings.
Systemic Complications of RA
- Rheumatoid nodules, vasculitis, and lung disease can occur.
Ocular Effects of RA
- Rheumatoid nodules can occur on the eyelids and in the orbit.
- Scleritis (inflammation of the sclera) and episcleritis (inflammation of the outer surface of the sclera) are common.
Sjogren’s Syndrome (SS)
- An autoimmune disease affecting the salivary and lacrimal glands, causing dry eyes and dry mouth (xerostomia).
- Can affect other organs.
SS Signs
- Dry eyes, dry mouth, fatigue, and joint pain.
SS Symptoms
- Xerostomia, keratoconjunctivitis sicca (dry eye), and dental problems are common.
SS Diagnostic Criteria
- Diagnosis requires a combination of clinical features, laboratory testing, and salivary gland biopsy.
- At least 4 points are needed:
- Anti-Ro/SSA Positive (3 points).
- Lip Biopsy with Focus > 1 foci/4mm^2 (3 points).
- Ocular Staining Score ≥ 5 (1 point).
- Schirmer’s Test (without anesthetic) ≤ 5mm/5min (1 point).
- Unstimulated Salivary Flow Rate ≤ 0.1 mL/min (1 point).
- Patients may also have anemia, leukopenia, and lymphopenia.
SS Management
- Managing symptoms like dry eyes and dry mouth.
- Medications include artificial tears, saliva substitutes, and medications to stimulate saliva production.
Spondyloarthropathies
- A group of inflammatory diseases affecting the spine and other joints.
- Includes ankylosing spondylitis, psoriatic arthritis, reactive arthritis, and inflammatory bowel disease-associated arthritis.
- These diseases often affect joints and tissues around the spine, causing pain, stiffness, and inflammation.
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Description
Explore the complexities of autoimmune disorders and learn how the immune system can mistakenly attack the body's own tissues. This quiz covers diagnosis through laboratory tests, including Rheumatic Factor and Antinuclear antibodies, among others. Gain insights into the different conditions associated with these tests.
