Lecture 18 - Connective Tissue Pathologies

Question 1

Systemic Lupus Erythematosus (SLE) has several immunologic factors contributing to its pathology. Initially, there’s a failure of self-tolerance in __-cells –> then, CD4+ helper T-cells contribute to the production of high affinity Abs –> and Type I ______ play a role in lymphocyte activation.

Answer 1

B-cells

Interferons

Question 2

Autoantibodies for ____ antigen and dsDNA are both specific for SLE.

Answer 2

Smith Antigen

Question 3

About ____% of all SLE patients will develop some type of Nephritis, and Class ____ is the most common and most severe.

Answer 3

50%

Class IV

Question 4

Immunofluorescence of the skin in SLE patients will reveal Ig and Complement deposition at the _____-______ border.

Answer 4

Dermal-Epidermal border

Question 5

Is myocarditis or pericarditis more common in SLE patients?

Answer 5

Pericarditis (about 50% of patients)

Question 6

___ (aka SS-A and ___ (aka SS-B) are autoantibodies that are specific for Sjogen Syndrome. These patients tend to present with dry ___ and ___ due to infiltration of lacrimal glands and salivary glands with inflammatory cells. Early in the disease, expect to find ____ductal infiltrate. In intermediate stages, expect to find ductal infiltrate. In end stage, the glands and ducts are completely destroyed, and it can progress to ___-cell lymphoma.

Answer 6

Ro (SS-A)

La (SS-B)

Eyes

Mouth

Periductal

B-cell lymphoma

Question 7

Autoantibodies against DNA ______ I (aka Scl-70) are specific for Systemic Sclerosis. Look for patients presenting with ______ (sclerosis of the skin in the hands), diffuse skin sclerosis, and diffuse lung disease. This disease also commonly affects which part of the GI tract?

Answer 7

DNA Topoisomerase I

Morphea

Esophagus

Question 8

Mixed Connective Tissue Disease (MCTD) is characterized by a mix of clinical features of SLE, Systemic Sclerosis, and Polymyositis. It is identified serologically by high titers of autoantibodies to _______ particle-containing U1 _________. Looks for patients that present with _______ phenomenon and mild myositis.

Answer 8

Ribonucleoprotein Particle-containing U1 Ribonucleoprotein

Raynaud’s phenomenon

Question 9

IgG4 Related Disease (IgG4RD) is a constellation of diseases within patients that show plasma cell infiltrates in organ systems and high serum levels of _____. B-cells are thought to have important involvement in this disease bc patients have responded to treatment with _______.

Answer 9

IgG4

Rituximab (anti-B-cell monoclonal Ab)