Systemic Autoimmune Disorders

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Questions and Answers

What is the basic underlying cause of autoimmune diseases?

Increased aging factor

Excessive immune responses

Environmental toxins

Failure of self-tolerance (correct)

What can promote T-cell death by apoptosis?

Increased production of antibodies

Recognition of foreign antigens

Signals that recognize self-antigens (correct)

Exposure to viral infections

Which of the following is a type of autoimmune disorder mediated by antibodies?

Rheumatoid arthritis

Multiple sclerosis

Lupus (correct)

Type 1 diabetes

What are susceptibility genes related to in the context of autoimmunity?

Interference with self-tolerance Signup and view all the answers

Which of the following is considered a systemic autoimmune disorder?

Systemic lupus erythematosus Signup and view all the answers

What kind of autoimmune disorder is autoimmune hemolytic anemia?

Organ-specific autoimmune disorder Signup and view all the answers

Which factor is NOT typically considered a predisposing factor for autoimmunity?

Dietary habits Signup and view all the answers

What is a common clinical presentation of autoimmune disorders?

Fatigue and weakness Signup and view all the answers

What percentage of patients experience parotid gland enlargement?

50% Signup and view all the answers

Which of the following extraglandular diseases is not listed as a complication?

Skin rash Signup and view all the answers

What is a common defect observed in patients related to renal function?

Defects of tubular function Signup and view all the answers

Which autoimmune disorder is most commonly associated with this condition?

Rheumatoid arthritis Signup and view all the answers

What is the outcome associated with high titers of anti-SS-A antibodies?

More common extraglandular disease manifestation Signup and view all the answers

What condition arises from the inflammatory destruction of the exocrine glands producing keratoconjunctivitis?

Blurring of vision and dry mouth Signup and view all the answers

Which symptom is specifically associated with xerostomia?

Drying of the mouth Signup and view all the answers

What are potential consequences of keratoconjunctivitis?

Inflammation and erosion of the corneal epithelium Signup and view all the answers

Which organ system can be affected by the same process that causes symptoms like keratoconjunctivitis and xerostomia?

Endocrine system Signup and view all the answers

What is a common symptom indicating keratoconjunctivitis?

Dry eyes and burning sensation Signup and view all the answers

Which statement best describes xerostomia?

It is the drying of the mouth due to gland dysfunction. Signup and view all the answers

Which of the following symptoms results from keratoconjunctivitis?

Blurring of vision Signup and view all the answers

What complication can result from the lack of tears due to keratoconjunctivitis?

Ulceration of the corneal epithelium Signup and view all the answers

Which of the following is a true statement regarding the systemic effects of gland dysfunction related to these conditions?

Other organs such as the GI tract and kidneys can also be involved. Signup and view all the answers

What accumulation occurs in the conjunctival sac due to keratoconjunctivitis?

Thick secretions Signup and view all the answers

What is a common histologic finding in the kidneys of patients with systemic lupus erythematosus (SLE)?

Glomerulonephritis Signup and view all the answers

What type of microscopy reveals deposition of immunoglobulin and complement in SLE?

Immunofluorescence microscopy Signup and view all the answers

Which cellular component is often involved in the perivascular inflammation seen in SLE?

Lymphocytic infiltrates Signup and view all the answers

What histological change occurs in the epidermis due to SLE skin involvement?

Vacuolar degeneration of the basal layer Signup and view all the answers

What finding is NOT specific for systemic lupus erythematosus (SLE) but can occur in other autoimmune diseases?

Deposition of immunoglobulin and complement Signup and view all the answers

In patients with SLE, what type of renal pathology is primarily observed in the glomeruli?

Immune complex-mediated lesions Signup and view all the answers

What skin condition is most closely associated with SLE and characterized by a distinct appearance?

Butterfly or malar rash Signup and view all the answers

What type of necrosis may be prominent in the vasculitis seen in SLE?

Fibrinoid necrosis Signup and view all the answers

What percentage of SLE patients have clinically significant renal involvement?

50% Signup and view all the answers

Which characteristic change in the dermis is associated with SLE skin lesions?

Variable edema Signup and view all the answers

What is one primary result of xerostomia?

Difficulty in swallowing solid food Signup and view all the answers

What might happen to the oral mucosa in patients with xerostomia?

Atrophy with inflammatory fissuring Signup and view all the answers

What type of skin involvement is observed in the limited form of xerostomia?

Confined to fingers, forearms, and face Signup and view all the answers

How does the clinical course of limited xerostomia compare to diffuse xerostomia?

Relatively benign Signup and view all the answers

What is not a symptom associated with xerostomia?

Increased hydration in the mouth Signup and view all the answers

In what way does xerostomia impact the ability to taste?

Decreases the ability to taste Signup and view all the answers

Which of the following is a potential feature of xerostomia?

Atrophy of oral mucosa Signup and view all the answers

What do cracks and fissures in the mouth indicate in relation to xerostomia?

Possible inflammation and ulceration Signup and view all the answers

Which statement about the symptoms of xerostomia is accurate?

Symptoms may vary significantly based on the form. Signup and view all the answers

What could be a long-term complication of untreated xerostomia?

Development of oral mucosal diseases Signup and view all the answers

What disorder is most commonly caused by antiplatelet autoantibodies in SLE patients?

Immune thrombocytopenic purpura Signup and view all the answers

What percentage of lupus patients may present with antiphopholipid antibodies?

30 to 40% Signup and view all the answers

Which clinical condition is referred to as the secondary antiphospholipid antibody syndrome (APAS)?

Recurrent spontaneous miscarriages Signup and view all the answers

Antinuclear antibodies (ANAs) are primarily directed against which of the following?

Nuclear antigens Signup and view all the answers

Which pattern of glomerular disease is categorized as minimal mesangial lupus nephritis?

Class I Signup and view all the answers

Which statement is true regarding antiphospholipid antibodies in the context of SLE?

They may lead to both venous and arterial thrombosis. Signup and view all the answers

What autoimmune response is classified under Type IIa autoantibodies?

Response against blood cells Signup and view all the answers

What is a potential clinical outcome associated with the presence of antiphospholipid antibodies?

Venous and arterial thrombosis Signup and view all the answers

Which area is NOT mentioned as being affected by scleroderma?

Nose Signup and view all the answers

What type of autoantibody is specifically anti-Scl 70?

Antibody against DNA topoisomerase 1 Signup and view all the answers

Which of the following is a condition that may result from severe scleroderma?

Decreased blood circulation to the extremities Signup and view all the answers

What describes the specific skin changes associated with advanced stage scleroderma?

Cutaneous ulcerations and atrophic changes Signup and view all the answers

What is a potential consequence of loss of blood supply to the phalanges in scleroderma?

Auto-amputation of fingertips Signup and view all the answers

Which role do autoantibodies play in the pathology of scleroderma?

They stimulate fibrosis Signup and view all the answers

What type of antibodies are present in virtually all scleroderma patients?

Antinuclear antibodies (ANAs) Signup and view all the answers

What is the specificity of anti-Scl 70 antibodies?

It is highly specific against DNA topoisomerase 1 Signup and view all the answers

What might indicate the presence of auto-amputation in scleroderma patients?

Severe cutaneous ulcerations Signup and view all the answers

How might anti-nuclear antibodies contribute to the disease mechanism in scleroderma?

By stimulating fibrosis Signup and view all the answers

What is a common clinical feature of systemic sclerosis?

Skin fibrosis Signup and view all the answers

Which symptom often precedes other symptoms in systemic sclerosis?

Raynaud’s Phenomenon Signup and view all the answers

What is a known complication associated with myocardial fibrosis in systemic sclerosis?

Arrhythmias Signup and view all the answers

What age group has the peak incidence of systemic sclerosis?

50-60 years Signup and view all the answers

What gastrointestinal issue is commonly associated with systemic sclerosis?

Dysphagia Signup and view all the answers

What are the potential symptoms of myocarditis?

Resting tachycardia and electrocardiogram changes Signup and view all the answers

Which condition is characterized by the presence of fibrinous exudate on mesothelial surfaces?

Acute pericarditis Signup and view all the answers

What is a potential complication of chronic inflammation in the cardiovascular system?

Partial or total obliteration of the serosal cavity Signup and view all the answers

Which valves are primarily affected by valvular abnormalities?

Mitral and aortic valves Signup and view all the answers

What happens to mesothelial surfaces as inflammation progresses into the chronic phase?

They become thickened and opaque Signup and view all the answers

Which of the following statements is true regarding pericarditis?

Up to 50% of patients may have symptomatic or asymptomatic involvement. Signup and view all the answers

What types of effusions may accompany myocarditis?

Pericardial and pleural effusions Signup and view all the answers

What is a typical electrocardiogram change seen in myocarditis?

Tachycardia with abnormal patterns Signup and view all the answers

In which phase does the serosal cavity begin to present with shaggy fibrous tissue?

Chronic phase Signup and view all the answers

Which phase of inflammation includes covering of mesothelial surfaces with fibrinous exudate?

Acute inflammation Signup and view all the answers

What is the potential consequence of malignant hypertension in patients with systemic sclerosis?

Renal failure Signup and view all the answers

Which autoantibody is associated with diffuse systemic sclerosis?

Anti-Scl-70 Signup and view all the answers

Which of the following findings is common in patients with CREST syndrome?

Pulmonary fibrosis Signup and view all the answers

What type of vascular change is associated with hypertension in systemic sclerosis?

Thickening of blood vessel intima Signup and view all the answers

In systemic sclerosis, what percentage of patients has anti-centromere antibodies?

20-30% Signup and view all the answers

What is a characteristic feature of malignant hypertension as described in systemic sclerosis?

Rapid downhill course Signup and view all the answers

What factor increases the likelihood of pulmonary fibrosis in systemic sclerosis patients?

Presence of anti-Scl-70 antibody Signup and view all the answers

What histopathological change is NOT typical in patients with systemic sclerosis concerning renal health?

Glomerular morphologic changes Signup and view all the answers

Which symptom is associated with hypertension leading to renal complications in systemic sclerosis?

Oliguria Signup and view all the answers

What is a common risk associated with the presence of autoantibodies in systemic sclerosis?

Increased risk of vascular disease Signup and view all the answers

Study Notes

Apoptosis and Autoimmunity

T-cells that encounter self-antigens may receive signals for apoptosis, preventing autoimmune reactions.

Failure of Self-Tolerance

Autoimmune diseases stem from the breakdown of self-tolerance, leading to immune responses against self-antigens.

Predisposing Factors of Autoimmunity

Susceptibility Genes: Inherited genes can disrupt self-tolerance, increasing autoimmune disease risk.
Examples of Organ-Specific Autoimmune Disorders:
- Autoimmune hemolytic anemia
- Autoimmune thrombocytopenia
- Autoimmune atrophic gastritis

Systemic Lupus Erythematosus (SLE)

Kidney Involvement: About 50% of SLE patients experience significant renal issues, primarily glomerulonephritis and tubulointerstitial nephritis.

Histologic Findings of SLE

Light Microscopy:
- Glomerular lesions: immune complex deposition on glomerular membrane.
- Epidermis shows vacuolar degeneration; dermis may exhibit variable edema.
- Perivascular inflammation with lymphocytic infiltrates may be observed.
Immunofluorescence Microscopy:
- Deposition of immunoglobulin and complement at dermoepidermal junction, not exclusive to SLE.

Salivary Gland Involvement

Symptoms of Inflammation:
- Keratoconjunctivitis: Leads to blurred vision, burning, and itching due to dry eyes.
- Xerostomia: Results in swallowing difficulties, loss of taste, oral fissures, and buccal mucosa dryness.

Other Symptoms in Autoimmunity

Parotid gland enlargement occurs in 50% of cases.
Nasal mucosa dryness, leading to epistaxis; recurrent bronchitis and pneumonitis are common.
Extraglandular manifestations affect about one-third of patients and may include synovitis and peripheral neuropathy.

Major Categories of Autoimmune Conditions

Diffuse vs. Limited:
- Diffuse: Widespread skin involvement; more aggressive clinical course.
- Limited: Skin involvement primarily on fingers, forearms, face; typically, a more benign course.

CREST Syndrome

Observed in some patients with limited disease:
- Characterized by Calcinosis, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia.
Patients usually live longer with less visceral organ involvement compared to diffuse forms.

Autoantibody Types in Systemic Lupus Erythematosus (SLE)

Antinuclear antibodies (ANAs) target nuclear antigens, found in 30-40% of lupus patients.
Autoantibodies can cause immune thrombocytopenic purpura in up to 10% of SLE cases.
Antiphospholipid antibodies are linked to venous and arterial thrombosis; may lead to secondary antiphospholipid antibody syndrome (APAS).
Recurrent miscarriages and focal cerebral or ocular ischemia can also occur.

Glomerular Disease Patterns in SLE

Six distinct patterns of glomerular disease observed in SLE patients.

Cardiovascular System Involvement

Cardiovascular issues may arise, affecting various heart layers.
Pericarditis occurs in up to 50% of patients, can be symptomatic or asymptomatic.
Myocarditis may present with tachycardia, ECG abnormalities, though uncommon.
Valvular abnormalities primarily affect the mitral and aortic valves.

Scleroderma Overview

Scleroderma is characterized as systemic sclerosis, leading to progressive fibrosis in various organs.
Female predilection with a 3:1 ratio of women to men affected, particularly in the 50-60 age group.
Autoantibodies including Anti-Scl-70 present in 10-20% of diffuse cases, associated with pulmonary fibrosis.
Anti-centromere antibodies occur in 20-30% of cases, indicative of CREST syndrome (limited type).

Skin and Vascular Changes in Scleroderma

Skin changes signify striking cutaneous fibrosis; may lead to atrophic alterations and ulcers.
Hypertension is common, with a subset experiencing malignant hypertension potentially resulting in renal failure.
Raynaud’s Phenomenon often precedes other symptoms in approximately 70% of patients, serving as an early indicator.

Clinical Features of Systemic Sclerosis

Distinctive skin features and organ fibrosis are notable clinical signs.
Difficulty swallowing (dysphagia) is common, linked to esophageal involvement.
Endothelial injury, microvascular disease, and chronic ischemia contribute to disease progression and fibrosis.

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This quiz explores the anatomy and function of salivary glands, including symptoms and effects of inflammatory diseases. Test your knowledge on the role of exocrine glands and their impact on health. Dive into the details of related conditions and treatments.