Parotid Gland Enlargement and Related Conditions

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Questions and Answers

What does the yellow line in the graph represent?

Viral copies (viremia) (correct)

Time period

Immune response

CD4 T-cell count

What occurs during the clinical latency phase of HIV?

CD4 T-cell count increases

Viral replication continues while CD4 count decreases (correct)

No immune response occurs

Viral replication stops completely

How long does it typically take for viremia to develop after primary infection?

3-6 weeks (correct)

1-2 weeks

6-12 weeks

6 months

What is the role of dendritic cells in the spread of HIV?

They present the virus to CD4 T cells Signup and view all the answers

At what critical level of CD4+ T-cell count does the risk for AIDS-associated diseases significantly increase?

200 cells/mm³ Signup and view all the answers

What characterizes the acute viral syndrome during the early infection period of HIV?

Dissemination of virus and immune response development Signup and view all the answers

What is a symptom associated with parotid gland enlargement?

Dryness of the nasal mucosa Signup and view all the answers

What condition is characterized by the combination of lacrimal and salivary gland inflammation?

Mikulicz syndrome Signup and view all the answers

Which of the following is NOT a symptom of dryness associated with the condition discussed?

Profuse sweating Signup and view all the answers

What characterizes the progression of the majority of patients with parotid gland enlargement?

Progression to visceral involvement Signup and view all the answers

Which statement about limited scleroderma is correct?

It is often confined to fingers and face Signup and view all the answers

Which disease is often described as having a relatively benign clinical course?

Limited scleroderma Signup and view all the answers

What is a common complication resulting from visceral involvement in patients with parotid gland enlargement?

Cardiac failure Signup and view all the answers

What is one factor that may influence higher risks for extraglandular disease?

High titers for SS-A Signup and view all the answers

What is a common disease that could cause lacrimal and salivary gland enlargement?

Sarcoidosis Signup and view all the answers

What causes the clinical manifestations of common variable immunodeficiency?

Low levels of antibodies Signup and view all the answers

What is a characteristic of X-linked agammaglobulinemia?

Absence of B cells Signup and view all the answers

Which syndrome is associated with a deletion on chromosome 22q11?

DiGeorge syndrome Signup and view all the answers

What is the primary antibody involved in mucosal secretions?

IgA Signup and view all the answers

What is a common outcome of isolated IgA deficiency?

Frequent respiratory allergies Signup and view all the answers

What may happen if a patient with IgA deficiency receives blood transfusions containing normal IgA?

Severe anaphylaxis Signup and view all the answers

Which of the following is NOT a secondary immunodeficiency?

X-linked agammaglobulinemia Signup and view all the answers

Which condition is associated with poor defense against certain fungal and viral infections?

Common variable immunodeficiency Signup and view all the answers

Which of the following describes a common complication of secondary immunodeficiencies?

Autoimmune diseases Signup and view all the answers

What might lead to isolated IgA deficiency in patients?

Impaired differentiation of naïve B lymphocytes Signup and view all the answers

What is a potential complication of cytomegalovirus infection?

Cytomegalovirus-induced pneumonitis Signup and view all the answers

What does the dysfunctional gene in certain bleeding disorders regulate?

Lysosomal trafficking Signup and view all the answers

What is the main effect of myeloablation prior to HSC transplantation?

Delay in the recipient's immune system repopulation Signup and view all the answers

Which condition is associated with a decreased oxidative burst?

X-linked phagocyte oxidase deficiency Signup and view all the answers

What type of cells may be attacked by grafted lymphocytes in a transplantation scenario?

Host's immune cells Signup and view all the answers

Which condition can be a fatal complication following HSC transplantation?

Cytomegalovirus-induced pneumonitis Signup and view all the answers

What is a common condition characterized by a defective oxidative burst?

Chronic granulomatous disease Signup and view all the answers

What does myeloablation primarily aim to achieve before transplantation?

Eliminate malignant cells Signup and view all the answers

What kind of gene defect characterizes an inherited disorder of lysosomal function?

Cytosolic protein deficiency Signup and view all the answers

What is a common consequence of grafted lymphocytes attacking host immune cells?

Graft versus host disease Signup and view all the answers

What condition is primarily caused by mutations in the gene encoding SLAM-associated protein (SAP)?

X-linked lymphoproliferative disease Signup and view all the answers

Which of the following contributes to the depletion of CD4+ Helper T cells?

Human Immunodeficiency Virus infection Signup and view all the answers

What is one of the consequences of defects in SAP regarding viral infections?

Increased susceptibility to viral infections Signup and view all the answers

What disorder is characterized by chronic mucocutaneous candidiasis and bacterial infections of the skin?

Job syndrome Signup and view all the answers

What process is impaired by irradiation and chemotherapy treatments?

Development of leukocyte precursors Signup and view all the answers

Which of the following is a potential outcome of a splenectomy?

Increased risk of infections Signup and view all the answers

Which type of infections are associated with inherited defects in Th1 responses?

Atypical mycobacterial infections Signup and view all the answers

Which condition can lead to decreased phagocytosis of pathogens?

Protein-calorie malnutrition Signup and view all the answers

What immune response is primarily diminished by chronic infections or immunosuppressive conditions?

T-cell activation Signup and view all the answers

Which of the following therapies is known to increase susceptibility to infections due to decreased bone marrow function?

Chemotherapy Signup and view all the answers

Study Notes

Parotid Gland Enlargement and Sjögren Syndrome

Parotid gland enlargement affects around half of the patients.
Symptoms include dryness of nasal mucosa, epistaxis, recurrent bronchitis, and pneumonitis.
Progression can lead to visceral involvement, resulting in death due to renal, cardiac, or pulmonary failure.

Extraglandular Manifestations

Extraglandular disease is present in one-third of patients with Sjögren syndrome.
Associated symptoms include synovitis, diffuse pulmonary fibrosis, and peripheral neuropathy.
High titers for SS-A are more common in patients with severe manifestations.

Mikulicz Syndrome

Characterized by simultaneous enlargement of lacrimal and salivary glands.
Previously referred to as Mikulicz disease.
Gland enlargement can result from various causes like sarcoidosis or lymphomas and is not exclusive to Sjögren syndrome.

Scleroderma Types

Diffuse Scleroderma: Widespread skin involvement from onset with early visceral involvement.
Limited Scleroderma: Often initially affects fingers and forearms; visceral involvement occurs late, with a relatively benign course.

CREST Syndrome

A variant of limited scleroderma that includes features such as calcinosis and bleeding disorders.

Immunodeficiency Disorders

DiGeorge Syndrome: Characterized by absence of cell-mediated immunity due to low T lymphocyte counts, linked to a deletion on chromosome 22q11.
Common variable immunodeficiency leads to antibody deficiency; it is caused by a failure of B cells to produce immunoglobulins.

Isolated IgA Deficiency

Arises from impaired differentiation of naïve B lymphocytes into IgA-producing cells, often asymptomatic but can lead to infections.

Secondary Immunodeficiencies

Can occur due to conditions like cancer, diabetes, malnutrition, or effects of chemotherapy and radiation.
Severe anaphylaxis may occur in IgA-deficient individuals when transfused with normal IgA-containing blood.

X-Linked Lymphoproliferative Disease

Caused by inability to eliminate Epstein-Barr virus (EBV), leading to severe infections and B-cell tumors; often linked to mutations in SLAM-associated protein (SAP).

Defects in Lymphocyte Activation

Mutations affecting Th1 responses result in susceptibility to atypical mycobacterial infections.
Job syndrome is associated with inherited defects in Th17 responses, leading to recurrent candida and bacterial infections.

Proposed HIV Pathogenesis

Initial infection phase involves viral dissemination, immune response development, and early acute viral syndrome.
Clinical latency follows, characterized by ongoing viral replication and gradual CD4+ T-cell decline, increasing the risk for AIDS-associated diseases.

HIV Clinical Progression

Significant changes in viremia and CD4+ T-cell counts occur over weeks to years.
Dendritic cells in mucosal tissues present the virus to CD4 T cells, which play a critical role in immune response control.

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Description

Explore the associations and complications of parotid gland enlargement in patients. This quiz delves into the impact on various organs, including the gastrointestinal tract, kidneys, and lungs, as well as symptoms like dryness of the nasal mucosa. Test your knowledge on this medical topic to understand its progression and implications.