Immunopathology III Flashcards by tcarlso 3

What is Sjogren’s syndrome?

Autoimmune disease against Exocrine glands, primarily lacrimal and salivary glands

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Who is usually affected with Sjogren’s?

Middle aged women

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What is sicca syndrome? How

dry eye/mouth alone, without other involvement. $05 of Sjogren’s syndrome

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What is the pathogenesis of Sjogren’s?

CD4+ T cells against glandular epithelial self Ag induced by a viral infection

Also Systemic B cell hyperactivity

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What are the infections that are associated with Sjogren’s?

EBV, hep C

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What are the specific ANA’s associated with Sjogren’s?

SS-A and SS-B

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True of false: Rheumatoid factor is also seen with Sjogren’s?

yes, 75%

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What causes the symptoms of Sjogren’s?

Attack by immune system, causing hyperplasia, fibrosis, and follicle formation

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What is the predominate inflammatory cells in SJogren’s

Lymphocytes

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What are the histological changes seenin Sjogren’s?

Ductal hyperplasia, inflammatory cells destroying glands

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What are the symptoms of Sjogren’s?

Xerostomia
Keratoconjunctivitis
Nasal septal erosions

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What is the lymph node characteristics of Sjogren’s?

Massively hyperplastics d/t hyperplasia

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What lymphoma is commonly seen in Sjogren’s

B cell (Marginal zone) lymphoma

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What are the systemic symptoms of Sjogren’s?

Vascultiis
renal problems
Skin problems
Perpheral neuropathy

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What protein is associated with Sjogren’s?

SS-A-Ab

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Which systemic symptoms are more prominant, SLE or sjogren’s ?

SLE

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What is the cause of complications of Sjogren’s?

Infects major organs

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What is systemic sclerosis?

Autoimmune disorder characterized by chronic inflammation, destruction of small vessels, and progressive tissue fibrosis

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Who gets systemic sclerosis?

50-60 yo females

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What are the major problem places in systemic sclerosis?

Heart, GI, or lung involvement

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What are the triggers of systemic sclerosis?

T and B cells autoactivation driving fibrosis

Blood vessel narrow

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What is the major cytokine in Systemic sclerosis that causes increased deposition of ECM?

TGF-beta

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What is the major cytokine in Systemic sclerosis that causes increased deposition of ECM?

TGF-beta

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What is CREST syndrome?

Limited variant of scleroderma

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What is the anti ab seen in CREST syndrome?

Anti-centromere

What are the symptoms of CREST syndrome?

``` Calcinosis Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia ```

What is Raynaud's syndrome seen in CREST?

vasospasms caused intermittent ischemia to digits

What is sclerodactyly seen in CREST syndrome?

Tapered digits

What is telangiectasia seen in CREST syndrome?

Purpuric lesions of the skin and mucous membranes

What is the diffuse variant of Scleroderma?

Widespread involvement with rapid progression

What is the Ab seen in diffuse scleroderma?

DNA topoisomerase I (anti-Scl-70)

What are the early skin changes in scleroderma?

Edema Lymphocyte infiltrates

What are the late skin changes with scleroderma?

Epidermal thinning Dermal fibrosis Subcutaneous calcifications

What causes the autoamputation of digits seen in scleroderma?

Vascular fibrosis, leading to ischemia

What are the early skin changes in systemic scleroderma?

Edema Lymphocyte infiltrates

What is the mask face of systemic scleroderma?

Fibrosis causing pulled appearance of skin

What causes the GI changes, such as LES dysfunction, seen in systemic scleroderma?

Fibrosis or the muscularis

What causes the GI changes, such as loss of villi, seen in systemic scleroderma?

Fibrosis of the muscularis

What are the MS early and late changes seen in systemic Scleroderma?

``` Early = nondestructive hyperplasia and inflammation Late = Fibrosis of synovial and periarticular CT ```

What percent of Systemic scleroderma have myositis with lymphocytic infiltrate?

10%

What are the renal changes in systemic sclerosis?

Thickening of interlobular arteries via concentration of intimal cells

What are the renal changes in systemic sclerosis? What can be a consequence of this?

Thickening of interlobular arteries via concentration of intimal cells HTN may develop

What are the pulmonary changes seen in systemic scleroderma?

Alveolar fibrosis, pulmonary HTN

What are the pulmonary changes seen in systemic scleroderma?

Alveolar fibrosis (restrictive disease), pulmonary HTN

What are the cardiac changes seen in systemic scleroderma?

Pericarditis with effusion Perivascular lymphoid infiltrates Arteriolar thickening

What type of lung and heart problems develop with systemic scleroderma?

Restrictive cardiomyopathy,

What type of lung and heart problems develop with systemic scleroderma?

Restrictive lungs disease/cardiomyopathy,

What are the causes of death in systemic sclerosis?

Renal Cardiac Pulmonary GI dysfunction/failure

What is RA?

Systemic, autoimmune inflammatory disorder

What are the parts of the body affected by RA?

Joints, skin, vessels, heart, lungs, soft tissue

What is the antigenic trigger for scleroderma or RA?

Unknown

What is the gene associated with RA? What is the protein that this codes for?

HLA-DRB1, coding for PTPN22

What is the MOA behind RA?

Autoimmune rxn by CD4 T cells, causing production of cytokines, and activation of macrophages, B cells

What is rheumatoid factor?

IgM auto-Ab to Fc portion of autologous IgG (antigen + IgG, antibody = IgM)

What is the peptide that has been associated with RA?

Citrullinated peptides

What type of hypersensitivity is RA?

Type III, II

What is the MOA behind RA?

CD4 T cells cause production of cytokines/activation of macrophages, B cells in joints Immune complex deposition in joints

What is deposited on synovial surfaces in RA?

Fibrin and granulation tissue, forming a pannus

Where are rheumatoid nodules found?

Pressure points (elbows, occiput, lumbosacrum)

What causes the vasculitis seen in RA?

RF-IgG complexes

What are the histological characteristics of a rheumatoid nodule?

Area of fibrous tissues, with necrosis in the center

What are the clinical symptoms of RA?

highly variable--malaise, pain, swelling etc

What are the usual causes of death with RA?

Amyloidosis | Drug therapy complications

What is Juvenile idiopathic arthritis?

Large joint oligoarthritis

What are the systemic features of Juvenile idiopathic arthritis?

Pericarditis myocarditis Pulmonary fibrosis Glomerulonephritis

Is there ANA in juvenile idiopathic arthritis? RF?

ANA + | RF -

Juvenile idiopathic arthritis + febrile illness, rash, hepatosplenomegaly, leukocytosis = ?

Still's disease

What is mixed CT disease?

Mixed SLE and systemic sclerosis at the same time

What is the Ab seen in mixed CT disease?

Anti-U1RNP) to ribonucleoprotein

What makes mixed CT disease clinical distinctive

minimal renal disease, and good response to steroids

What is the complication of mixed CT disease?

Progression to SLE or SS