Understanding Autoimmune Disorders

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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?

Every year (correct)

Every 2 years

Every 6 months

Every 3 years

Which of the following tests is considered ineffective for hydroxychloroquine retinopathy screening?

OCT of the macula

Fluorescein angiography (FA) (correct)

Automated visual fields

Dilated fundus exam

Which of the following provides a diagnostic criterion for Giant Cell Arteritis (GCA)?

Normalized temporal artery pulsation

New headache development (correct)

ESR of < 50 mm/hr

Age at onset less than 50 years old

Which laboratory test is specifically associated with diagnosing Lupus?

Anti-double stranded DNA antibodies (anti-dsDNA) Signup and view all the answers

What is a primary management strategy for Arteritic Anterior Ischemic Optic Neuropathy (AAION)?

Oral or IV steroids Signup and view all the answers

Which autoimmune disorder is primarily associated with the presence of Anti-Smith antibodies?

Lupus Signup and view all the answers

What test is essential for the diagnosis of Sjogren’s Syndrome (SS) that receives the highest points in diagnostic criteria?

Anti-Ro/SSA Positive Signup and view all the answers

What does a positive result for HLA-B27 typically indicate?

Ankylosing Spondylitis Signup and view all the answers

In the context of Systemic Lupus Erythematosus (SLE), what ocular effect is specifically mentioned?

Lupus retinopathy showing more cotton wool spots than hemorrhages Signup and view all the answers

What is the suggested treatment protocol regarding temporal artery biopsy in GCA management?

Initiate within two weeks of starting treatment Signup and view all the answers

Which test is utilized for the detection of Wegner’s granulomatosis?

Anti-neutrophil cytoplasmic antibody (ANCA) Signup and view all the answers

Which statement about the systemic complications of Rheumatoid Arthritis (RA) is accurate?

RA can lead to cardiovascular and pulmonary complications Signup and view all the answers

In a patient with a positive ANA test, which antibodies would be ordered to test specifically for Sjogren’s Syndrome?

Anti-SSA/SSB antibodies Signup and view all the answers

What is the typical clinical presentation of a patient with Arteritic Anterior Ischemic Optic Neuropathy (AAION)?

Sudden loss of vision in the affected eye Signup and view all the answers

Which laboratory test is commonly used to monitor general inflammation in autoimmune conditions?

ESR/CRP Signup and view all the answers

Which disease is linked to the presence of Anti-Jo antibodies?

Inflammatory Myositis Signup and view all the answers

Which of the following is NOT a common association with the Rheumatoid Factor (RF) test?

Eosinophilic Granulomatosis with Polyangiitis Signup and view all the answers

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.