TB & Autoimmune Path Flashcards by Rob Devine

A potentially serious infectious bacterial disease that mainly affects the lungs. Chronic and granulomatous (masses of immune cells that form at sites of infection or inflammation)

Tuberculosis

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What causes tuberculosis (TB)?

Mycobacterium tuberculosis

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Tuberculosis causes a type __ hypersensitivity reaction

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The 2 unique cells in granulomas are ___ and ___, both derived from macrophages

Epithelioid cells

Langhans giant cells

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When granulomas become necrotic they show ____ necrosis

Casseous (a crumbly, dull white appearance that resembles cheese)

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____ is caused by inhalation of aerosolized tubercle bacilli and occurs in the lung with development of a granuloma (Ghon focus). Occurs in a previously uninfected person.

Primary tuberculosis

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____ tuberculosis occurs in a previously infected person

Secondary (reactivation)

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Secondary tuberculosis is localized to what part of the lung?

Apex

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The initial testing for tuberculosis suspicion is ____

Chest X-Ray (CXR)

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The screening test for high risk groups for tuberculosis is?

PPD test (Purified Protein Derivative)

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High risk groups for tuberculosis are?

homeless
alcoholics
health care workers
recent immigrants
prisoners

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In the PPD test, a person in category 1 (close contacts, steroid users, HIV+ patients) is positive if the skin ‘bleb’ is __ to __mm

5-9mm

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In the PPD test, a person in category 2 is positive if the skin ‘bleb’ is 10-14mm. Who is in category 2?

High risk groups

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In the PPD test, a person in category 3 (low risk groups) is positive if the skin ‘bleb’ is >__mm

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The presence of many small granulomas in any organ

Miliary (if in lungs = miliary tuberculosis)

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Refers to a lack of immune responsiveness to one’s own tissue antigens

Self tolerance

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Refers to deletion of self-reactive T and B lymphocytes during their maturation in central lymphoid organs (thymus for T cells, bone marrow for B cells)

Central tolerance

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Mature lymphocytes that recognize self-antigens in peripheral tissues become functionally inactive or are suppressed by regulatory T lymphocytes (or die by apoptosis)

Peripheral tolerance

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___ autoimmune diseases are non-organ specific

Systemic

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Relating to disease caused by antibodies or lymphocytes produced against substances naturally present in the body

Autoimmune

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Addison’s disease affects the ____ gland

Adrenal

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What test is an important screening test for systemic autoimmune diseases?

Antinuclear antibody test (ANA) - performed w/a fluorescence microscopy method

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The immune complexes of this disease tend to deposit in kidney glomeruli and in small arteries and arterioles in different tissues resulting in necrotizing vasculitis

Systemic Lupus Erythematosus (SLE)

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SLE (lupus) has a female to male ratio of ____

9:1

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Major causes of death is SLE? (2)

Renal failure and CNS involvement

40-60% of SLE patients have this anti-nuclear antibody

Anti-dsDNA (ds=double stranded)

__ to __% of SLE patients have Anti-Smith antibodies

20-30%

>95% of patients with drug-induced lupus have this anti-nuclear antibody

Anti-histone

What is a classic sign of SLE?

Butterfly rash on the face

Treatment for SLE?

Steroids and other immunosuppressive drugs

With treatment, __ to __% have 10yr and 5yr survival rates respectively

80-90%

An autoimmune lymphocytic inflammatory destruction of the lacrimal and salivary glands resulting in dry eyes and mouth

Sjogren syndrome (SS)

Between __ and __% of patients with sjogren syndrome show another autoimmune disease such as RA or SLE

25-60%

90% of cases of sjogren syndrome occur in females between __ and __ years of age

35-45yo

There is a 40-fold increased risk of developing non-Hodgkin B cell lymphoma w/this autoimmune disease

Sjogren syndrome (SS)

Diagnostic test for SS?

Schirmer test (Paper held to eye)

Treatment for SS?

Pilocarpine (^acetylcholine which ^ oral and ocular secretions)

This autoimmune disease causes excess deposition of collagen in multiple organs and tissues. The skin is most often affected

Systemic Sclerosis (Scleroderma)

The limited symptoms of scleroderma are referred to as ____ (acronym)

CREST

CREST stands for what?

``` Calcinosis Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangiectasias ```

Sclerodactyly

Thickening and tightening of the skin on the fingers and hands

Calcium deposits in the skin

Calcinosis

Dilation of capillaries causing red marks on surface of skin

Telangiectasias

Spasm of blood vessels in response to cold or stress

Raynaud's phenomenon

Acid reflux and decrease in motility of esophagus

Esophageal dysfunction

Anti-Scl 70 against DNA Topoisomerase 1 seen in 70% of patients with diffuse ____ who are likely to develop more aggressive disease

Scleroderma

Anti-centromere antibody present in __% of patients with limited scleroderma (benign)

90%

Pathogenesis of this disease is fibroblast activation w/excessive fibrosis

Scleroderma

These two autoimmune diseases are an antibody-mediated tissue injury. Most patients have Jo-1 antibodies (against RNA Synthetase)

Dermatomyositis, polymyositis

T/F: patients w/dermatomyositis or polymyositis would have difficulty getting up from a chair or climbing steps.

True

____ shows an associated skin rash often affecting the upper eyelids and causing periorbital edema

Dermatomyositis

Dermatomyositis might show ___ papules on knuckles

Gottron's

A systemic chronic inflammatory autoimmune disease that mainly involves joints, producing chronic synovitis

Rheumatoid Arthritis (RA)

Rheumatoid factor is present in the blood in __% of RA patients

80%

RA patients have an increased risk of cardiovascular disease including ________

Coronary artery atherosclerosis

It is proposed that this disease is initiated by activation of CD4+ helper cells responding to arthritogenic agent or to a self-antigen

Abnormal stiffening and immobility of a joint due to fusion of the bones

Ankylosis

Treatment for RA?

NSAIDS | Anti-rheumatic drugs

The presence of clinical and pathologic features of systemic lupus Erythematosus (SLE), systemic sclerosis (scleroderma), and polymyositis in combination

Mixed Connective Tissue Disease (CTD)

Pulmonary involvement occurs in up to __% of Mixed CTD patients

85%

In Mixed CTD, there are ^ titers of antibodies to an antigen called ______

U1RNP

Treatment for mixed CTD?

Steroids

Causes of congenital disease? (4)

- drugs and chemicals - ionizing radiation (x-ray) - maternal infections - genetic (chromosomal abnormalities)

A congenital absence of proximal portion of a limb (due to Thalidomide)

Phocomelia

Two or more digits fuse together

Syndactyly

Spina bifida occulta occurs in about __% of people

20%

Protrusion of the meninges

Meningocele

Extension of the CNS tissue through a defect in the vertebral column. Most common in the lumbosacral region. Infection is a common complication

Myelomeningocele

Absence of calvarium (upper skull portion containing the brain) and brain. As a group these are the most common CNS congenital defects and most common congenital defects overall

Anencephaly

In anencephaly, the neural tube closes between __ and __ days gestation

22 and 28

In maternal screening, ____ protein has increased, an early detection of neural tube defects

Alpha-feto

The earliest stage of a developing organ?

Primordium

Partial, but incomplete development of an organ. The organ is of small size

Aplasia

An abnormally formed organ, may be of different sizes. This would be called _____

Dysplasia

No sign of organ development

Agenesis

Failure of the esophagus to develop continuously into the stomach

Esophageal atresia

Clinical sign for esophageal atresia?

Infant w/excessive drooling. Swallows normally, but liquid regurgitates through nose and mouth

A group of microorganisms that either infect the fetus in utero by crossing the placenta, or at the time of birth.

TORCH Syndrome

What do the letters of TORCH stand for?

``` Toxoplasmosis/toxoplasma gondii Other vertically transmitted infections Rubella Cytomegalovirus Herpes Simplex Virus-2 ```

This congenital disease is due to an extra X chromosome. The person will be tall w/long arms and legs, small testis and penis, female pubic hair profile, gynecomastia (man boobs), high pitched voice, reduced facial and body hair

Klinefelter syndrome

In Klinefelter syndrome, associated disorders such as breast cancer and Lupus (SLE) are __x more common as compared to unaffected males

20x

This congenital disease results from absence (57%) or abnormality (43%) of the second X chromosome

Turner syndrome

The females in Turner syndrome w/an ABSENT second X chromosome are most severely affected and are stillborn in an estimated __% of cases

99% !

Name the congenital disease: short stature, infantile genitalia, widely spaced nipples, micrognathia and prominent ears, widened (web) neck, amenorrhea (no menstrual period), congenital renal and aortic abnormalities

Turner syndrome

In this congenital disease, 3 copies of chromosome 21 are present

Down's syndrome

Down's syndrome occurs 1 in __ births overall

700

Features of this congenital disease: flat facial profile, horizontal Palmer crease on hands, intellectual retardation, tend to develop clinical Alzheimer's after age 40, cardiac abnormalities, increased susceptibility to infections and childhood leukemia

Down's syndrome

This congenital disease is an autosomal dominant disorder of connective tissues. The basic defect affects Fibrillin 1, which is a scaffold for elastic fibers

Marfan's syndrome

In Marfan's syndrome, __% of cases are familial

75%

This congenital disease results in long extremities and fingers, depressed or protruding sternum and spinal deformities such as kyphosis and scoliosis. Affects the cardiovascular system and eyes as well

Marfan's syndrome

This congenital disease results from defects in type I and type III collagen. 10 clinical variants are known. Other features include hyperelastic skin and hypermobile joints

Ehlers Danlos Syndrome (EDS)

EDS type __ is the most severe and may cause rupture of the colon or large arteries

This viral infection is a herpes virus. It causes infectious mononucleosis and infects two types of cells - Oropharyngeal epithelial cells & B lymphocytes.

Epstein Barr Virus (EBV)

This viral infection causes extreme fatigue. Lymphocytes are increased in the peripheral blood and some of them show reactive changes

Infectious mononucleosis (Mono)

A skin rash or eruption

Exanthem

A mucosal rash or eruption on the oral mucosa

Enanthem

Common name for the viral infection, Rubeola?

Measles

This viral infection is caused by Morbillivirus-a single stranded RNA virus. It infects the respiratory tract before spreading via the blood and lymph. Results in Koplik's spots followed by a rash behind the ear spreading to the body

Measles

Measles symptoms are the 3 C's:

Cough Coryza (stuffy nose) Conjunctivitis

German measles are also called ____

Rubella

This viral infection infects cells of upper respiratory tract and spreads via blood and lymph. Sx include fever, fine maculopapular rash and enlarged lymph nodes. In pregnancy causes severe congenital defects. Diagnosis confirmed by serological testing for IgM

Rubella (German measles)

A small, unenveloped, single-stranded DNA virus. Also known as Erythema Infectiosum. Rash begins on cheeks and spreads to arms, thighs, trunk, buttocks. Sx: mild fever, slapped cheek appearance. Sunlight aggravates rash

Parvovirus B19

At the time of infection this virus causes chickenpox, and later on can cause shingles

Varicella-Zoster Virus

T/F: Give aspirin to a child with chickenpox to help w/the symptoms

False! DO NOT (liver and brain damage)

Over 100 types of this viral infection are recognized based on DNA sequence differences. It is a non-enveloped DNA virus w/an affinity for epithelial cells of the skin and mucous membranes

Human Papilloma Virus (HPV)

Common name for condyloma acuminatum

Genital warts

The most common sexually transmitted disease in the US

HPV