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144 Cards in this Set
- Front
- Back
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What is the classic lesion of rheumatic fever?
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Aschoff body
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Disease occurring mostly in school age children between 5 and 15 years of age due to untreated streptococcal pharyngitis. A multisystem inflammatory disorder with major cardiac manifestations and sequelae
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Rheumatic fever
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What usually occurs 1 to 4 weeks after an episode of tonsilitis?
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Rheumatic Fever
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What bacteria causes rheumatic fever?
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Groups A beta-hemolytic streptococci
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What is evidence of a recent streptococci infection?
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Antistreptolysin O (ASO)
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What are the three carditis' that are caused by rheumatic fever?
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Pericarditis - serous effusions
Myocarditis - heart failure Endocarditis - valvular damage |
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What are the major Jones' Criteria?
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Polyarthritis
Erythema Subcutaneous Nodules Chorea Carditis |
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Disease that often occurs many years after rheumatic fever, often asymptomatic and fibrotic, deformed, calcified lines of closure on valve leaflets
The mitral valve more than the aortic valve Pulmonary valve rarely involved |
Rheumatic heart disease
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What is defined as having focal interstitial myocardial inflammation, large monocytes, and multinucleated cells.
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Aschoff bodies
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What is the most common clinical presentation of rheumatic fever?
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Migratory polyarthritis, lasting 2-3 weeks which is then accompanied by fever.
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A life threatening condition caused by partial or complete failure of adrenocortical function.
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Addison's disease (primary adrenocortical deficiency)
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What is the most common cause of Addison's disease?
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Idopathic adrenal atrophy
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What are the oral signs of Addison's disease?
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Pigmentation of the gingiva, tongue, hard palate, and buccal mucosa. This persists even after treatment
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What is the test which measures pituitary gland function and is especially used to determine if too much cortisol is being produced (cushing's syndrome) or if not enough cortisol is being produced (addison's disease)
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The adrenocorticotropic hormone test
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What does the adrenal gland produce which stimulates the outer adrenal gland (adrenal cortex)?
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ACTH
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What does ACTH stimulate the release of?
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Hydrocortisone (cortisol)
Aldosterone Androgen |
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What results from prolonged or improper use of glucocorticoid hormones, which are used to treat rheumatoid arthritis, asthma, and other inflammatory illnesses.
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Secondary adrenal insufficiency
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What is used to treat Addison's disease?
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Cortisol (hydocortisone)
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A catastrophic adrenal insufficiency and vascular collapse due to hemorrhagic necrosis of the adrenal cortex.
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Waterhouse-Friderichsen Syndrome
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What is Waterhouse-Friderichsen Syndrome characteristically due to?
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Meningococcemia in association with meningococcal meningitis
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Where are the adrenal glands located?
How many layers do they have? What types of hormones do they produce? |
On top of the kidneys
Outer portion: Cortex Inner portion: Medulla Produces: sex hormones, glucocorticoid hormones, mineralocorticoids hormones |
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What is the most common form of arthritis?
It is a chronic inflammatory joint disease characterized by degeneration of articular cartilage accompanied by new bone formation subchondrally and at the margins of the affected joint. |
Osteoarthritis
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What is the incidence of osteoarthritis?
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Greater in women most often beginning after 50 years of age.
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What kinds of joints are commonly affected in osteoarthritis?
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Joints exposed to wear and tear
Intervertebral joints Phalangeal joints Knees and hips |
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What are the characteristic morphologic changes of osteoarthritis?
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Eburnation of bone
Osteophyte formation Hebernen nodes Bouchard nodes |
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These occur at the perimeter of the articular surface and at points of ligamental attachment to bone in osteoarthritis.
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Osteocyte formation (bone spurs)
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What is it called when osteocytes fracture and begin floating in synovial fluid along with fragments of separated cartilage?
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Joint Mice
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Characterized by polished, ivory-like appearance of bone, resulting from erosion of overlying cartilage.
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Eburnation of bone
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What are the osteophytes at the distal interphalangeal joints called?
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Hebernen
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What are the osteocytes at the proximal interphalangeal joints called?
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Bouchard nodes
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What kind of osteoarthritis occurs without any known cause and is mostly related to aging?
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Primary osteoarthritis
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What kind of osteoarthritis is caused by another disease or condition. Conditions that can lead to this type include obesity, repeated trauma or surgery to the joint structures, abnormal joints at birth, gout, diabetes and other hormone disorders.
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Secondary osteoarthritis
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A term used to describe a group of different types of illnesses that share a common origin. Langerhans cells are increased in number and invade various tissues of the body.
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Histiocytosis X (Langerhans cell histiocytosis
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Cells normally found throughout the body and are most often found in the spleen, lung, liver, and bone marrow.
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Langerhans Cells (Histiocytes)
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What is the trigger for the increase of Langerhans cells and their invasion?
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Unknown
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What diseases are included in histiocytosis X?
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Eosinophilic Granuloma
Hand-Christian-Schuller Disease Letterer-Siwe Disease |
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The most benign or mild form of histiocytosis X, characterized by a solitary lesion of the bone, which can result in pain and swelling
_____% of histiocytosis X Ages _____ |
Eosinophilic Granulomas
60-80% 5-10 |
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Usually refers to children with the classic triad of skull lesions, diabetes insipidus, and exophthalmos caused by involvement of the orbit
Ages _____ |
Hand-Christian-Schuller Disease
1-3 |
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Refers to a generalized disease that has a very aggressive, often fatal ending. Organs that may be involved include bone, lungs, skin, liver, spleen, and bone marrow.
Ages _____ Worst _____ |
Letterer-Siwe Disease
0-1 Prognosis |
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What are the two factors which are involved that affect a child's prognosis to Histiocytosis X (Langerhans cell histiocytosis)
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Age at time of Diagnosis
# of organs involved |
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What makes a poor prognosis for LCH?
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Younger than 2
Presence of major organ Multiple organs involved |
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A common disease that can be subdivided pathologically into adenoma _____ of cases, hyperplasia _____ of cases, and carcinoma _____ of cases.
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Primary hyperparathyroidism
80-85% 15% <1% |
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What are the laboratory findings for hyperparathyroidism?
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Hypercalcemia
Decreased serum phosphorus Increased serum alkaline phosphatase Increased serum PTH |
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What are the clinical characteristics of Hyperparathyroidism?
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Osteitis fibrosa cystica
Nephrocalcinosis Renal calculi Peptic duodenal ulcers |
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Disease characterized by cystic changes in bone due to osteoclastic resorption. Replacement of resorbed bone may lead to the formation of non-neoplastic tumor-like masses called _____ _____.
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Osteitis fibrosa cystica
Brown Tumor |
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A metastatic calcification affecting the kidneys
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Nephrocalcinosis
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What are the dental fingins of hyperparathyroidism?
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Loss of the lamina dura around multiple teeth.
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What are the three classifications of hyperparathyroidism?
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Primary
Secondary Tertiary |
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This type of hyperparathyroidism is the result of an adenoma, glandular hyperplasia, or carcinoma
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Primary Hyperparathyroidism
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This type of hyperparathyroidism is a reactive hyperplastic phenomenon in response to decreased concentration of serum calcium
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Secondary Hyperparathyroidism
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This type of hyperparathyroidism is a term used to describe the instance where secondary becomes autonomous.
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Tertiary Hyperparathyroidism
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What is hyperparathyroidism associated with in rare instances?
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Congenital thymic hypoplasia (DiGeorge's Syndrome)
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What are manifestations of hyperparathyroidism?
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Osteoporosis
Cental Giant Cell Granulomas Metastatic calcifications |
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30% of patients with polyarteritis nodoas have serum antibodies to what?
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Hep B Virus
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Glue-like protein that represents 30% of body protein. Shapes the structure of tendons, bones, and connective tissue.
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Collagen
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What are problems of the immune system that affect collagen?
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Collagen Vascular Disease
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What are examples of Collagen Vascular Diseases?
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Polyarteritis Nodosa
Dermatomyositis Scleroderma Systemic lupus erythematous (SLE) Rheumatoid arthritis (RA) |
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A serous blood vessel disease of unknown cause characterized by necrotizing immune complex inflammation of small and medium sized arteries. There is an association with the Hep B virus.
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Polyarteritis Nodosa
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A muscle disease that causes inflammation and a skin rash. It is a type of inflammatory myopathy, and the cause is unknown.
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Dermatomyositis
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A widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs. It is common in young women, has wide spread connective tissue fibrosis, and tight mask-like, facial skin, raynaud's phenomenon, pain, stiffness, and swelling of fingers and joints, GERD, increased chance of Barretts esophagus.
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Scleroderma
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The prototype connective tissue disease. Autoimmune disease. 80% of patients are women usually of child bearing years. It has fever malaise, lymphadenopathy, and weight loss. Characteristic butterfly rash over the cheeks and bridge of the nose (50% of patients), joint pain and arthritis, raynaud's phenomenon, and extensive immune complex-mediated inflammatory lesions, the greatest significance of which are in the kidney's?
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Systemic lupus erythematous (SLE)
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What is the cause of multiple sclerosis?
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Unknown
Possible autoimmune component involving CD4+, CD8+ T-cells |
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Disorder of the brain and spinal cord (CNS) which is caused by progressive damage to the outer covering of the nerve cells (myelin). This results in decreased nerve functioning which can lead to a variety of symptoms.
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Multiple Sclerosis
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What occurs in Multiple Sclerosis?
When do these episodes occur? |
Inflammation of nervous tissue anywhere in the CNS
When body's immune cells attack itself |
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What results in the slowing or blocking of transmission of nerve impulses in MS?
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Inflammation destroys myelin and leaves scar tissue (sclerosis)
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What are some common symptoms of MS?
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Visual disturbances
Speech disturbances Paresthesias Depression Mood swings |
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What are characteristically found in the cerebrospinal fluid of a patient with MS?
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Moderate amount of proteins
Small number of lymphocytes |
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What therapy reduces the frequency of relapses of MS?
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Injectable interferon
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Multiple sclerosis affects about _____ out of _____ people.
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1/1000
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What sex is more commonly affected by MS?
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Women
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When does MS commonly begin in patients?
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20-40 years old but can happen any time
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Primary amyloidosis is associated with abnormalities of what?
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Plasma Cells
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A generic term used to describe various conditions in which proteinaceous material (amyoid) abnormally deposits and accumulates in tissues and /or organs. These amyloids are primarily composed of _____ fibrous proteins
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Amyloidosis
Insoluble |
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How is Amyloidosis classified?
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Chemical and Physical nature of Amyloid
Extent of deposits |
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What are the different classifications of Amyloidosis?
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Systemic (more than 1 organ/body system)
Localized (1 organ) Further subdivided into Primary and Secondary |
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When is Amyloidosis considered a Primary classification?
Secondary? |
Primary - related to immune disorder
Secondary - Complication from other chronic inflammatory process |
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Type of Amyloidosis in which the cause is unknown; it is related to abnormal production of immunoglobulins by malignant plasma cells. Usually systemic in distribution. Typical sites of Amyloid build up are of the heart, lungs, skin, tongue, thyroid glands, intestines, liver, kidney, and blood vessels.
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Primary Amyloidosis
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The amyloidosis is a complication of another disease such as TB, rheumatoid arthritis, familial Mediterranean fever. This amyloid tends to build up in the spleen, liver, kidney's, adrenal glands, and lymph nodes. Heart rarely involved.
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Secondary Systemic (Reactive) Amyloidosis
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Mostly rare form of Amyloidosis and limited to specific geographic areas, the result of genetic mutations. Examples include: Mediterranean fever, systemic senile amyloidosis and several types of familial amyloidotic neuropathies.
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Hereditary Amyloidosis
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What is another name for Hives?
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Urticaria
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Reaction of the skin characterized by small, pale, or reddened swellings (wheals).
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Urticaria (hives)
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What are hives the result of?
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Type 1 hypersensitivity do to exposure of antigens
Leads to IgE dependent degranulations |
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What is often associated with hives and involving larger areas and deeper tissues?
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Angioedema
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What are Hives and angioedemas known collectively as?
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Anaphylactic-type reactions
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What is the therapy for hives and angioedemas?
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Epinephrine, Antihistamines, Steroids
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What is the first symptom of hives?
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Itching followed by wheels
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What does the release of histamine into the blood stream cause?
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Itching, Localized Swelling (allergic Response)
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What is the most common pancreatic endocrine disorder?
Metabolic disease involving mostly carbohydrates (glucose) and lipids. Caused by absolute deficiency of insulin, or resistance to insulin's action in the peripheral tissues. |
Diabetes
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What are the classic triad of symptoms of diabetes?
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Polydipsia
Polyuria Polyphagia |
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Characterized by no insulin secretion, occuring in childhood, makes up 10-20% of cases, consists of a defect that results in the destruction of beta cells, is not associated with obesity, has rapid onset, commonly develops ketosis if untreated, and requires injections and dietary management.
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Diabetes Type 1
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Characterized by normal or exceeded limits of insulin, typically occurring in adults, cosists of 80-90% of cases in patients, has a basic defect resulting in reduced sensitivity of insulin's target cells, usually associated with obesity, has a slow onset of symptoms, rarely develops ketosisand is treated through dietary control and weight reduction, occasionally oral hypoglycemic drugs
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Diabetes Type 2
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What is the cause of rheumatoid arthritis?
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Unknown
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A chronic inflammatory disease that primarily affects the joints and surrounding tissues, but can also affect other organ systems. Unknown cause but there is a possible genetic predisposition.
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Rheumatoid Arthritis (RA)
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What does RA involve?
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Attack on the body by its own immune cells (poss. autoimmune disease)
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What is the Prevalence of RA?
What is it called when it happens in younger people? |
Any age poss. but usu. older people
Begins 25-55 Women 2.5x more 1-2% pop affected Still's Disease |
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What parts of the body does RA occur in?
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Involves small joints of hands and feet, Destructive pannus leads marked joint deformity
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RA is marked by a _____ _____ of the synovial membranes, leading to deformity, ankylosis, and invalidism.
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Proliferative Inflammation
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Classified as Type III hypersensitivity reactions that occur when excess circulating immune complexes (antibody bound to antigen) deposit in tissues).
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Immune Complex Diseases
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A chronic inflammatory disease that primarily affects the joints and surrounding tissues, but can also affect other organ systems. Unknown cause but there is a possible genetic predisposition.
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Rheumatoid Arthritis (RA)
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What do the glomerular lesions in immune complex diseases result from?
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Deposition of IgG
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What system effectively clears the immune complex?
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Reticuloendothelial system
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What do deposits in the immune complex result in?
Does histamine play a part in this reaction? |
Activates complement cascade = inflammation
No not in Type 3 hypersensitivity |
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Disease that appears days after injection of a foreign serum or serum protein, with local and systemic reactions such as urticaria, fever, general lymphadenopathy, edema and arthritis.
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Serum Sickness
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The second most common autoimmune rheumatic disorder after RA. It is characterized by diminished lacrimal and salivary gland secretions (sicca complex). This syndrome occurs mainly in women (90% of patients) and the mean age is 50.
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Sjogren's Syndrome
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Describes Inflammation of the kidney's glomeruli, hindering removal of waste products from the blood. It can occur by type 2 reaction (Goodpasture's syndrome), a type 3 reaction or as part of a multisystem vasculitic process
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Glomerulonephritis
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What type of hypersensitivity reactions are caused by specific antibodies binding to antigens on the patient's own cell surfaces.
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Type 2
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Undesirable (damaging, discomfort-producing and sometimes fatal) reactions produced by the normal immune system.
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Hypersensitivity
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What is required for a hypersensitivity reaction?
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Pre-sensitized immune state of the host
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How many hypersensitivity reactions are there?
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4
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What distinguishes the different types of hypersensitivities?
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Basis of mechanism
Time taken for the reaction |
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What is also known ans immediate or anapylactic hypersensitivity?
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Type 1
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Reactions in which antigens combine with specific IgE antibodies that are bound to membrane receptors on tissue _____ _____ and blood _____.
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Type 1 Hypersensitivity
Mast Cells Basophils |
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The Type 1 reactions a rapid release of what?
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Potent vasoactive and inflammatory mediators.
Preformed - Histamine New from mem. - Leukotrienes/Prostaglandins |
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Cytotoxic reactions resulting when antibody reacts with antigenic components of a cell or tissue elements (with antigen or hapten) that is coupled to a cell or tissue.
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Type 2 hypersensitivity
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What is another name of type 2 hypersensitivity?
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Antigen-Antibody Reaction
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What can a type 2 hypersensitivity activate to produce an antibody cell-mediated reaction?
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Cytotoxic TCells (killer T Cells / Macrophages)
Cell-Mediated cytotoxicity |
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What are the primary mediators of Type 2 hypersensitivity?
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IgM, IgG and Complement
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What are examples of Type 2 Hypersensitivity?
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Goodpasture's Syndrome
Erythroblastosis fetalis Autoimmune Hemolytic Anemia Hyperacute Transplant Rejection |
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An immune complex reaction mostly of IgG although IgM is somewhat involved resulting from the deposition of soluble circulating antigen-antibody IC's in vessels or tissue.
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Type 3 Hypersensitivity
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What are the components of Type 3 hypersensitivity?
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Soluble immune complexes and complement (C3a, 4a, 5a)
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What causes the damage of Type 3 hypersensitivity?
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Platelets and Neutrophils
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What are examples of Type 3 Hypersensitivity?
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Serum Sickness, Systemic Lupus Erythematous, Arthus reaction
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What is associated with healing during the later stages of type 3 Hypersensitivity?
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Macrophages?
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What is also known as Cell-mediated or delayed type hypersensitivity?
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Type 4 Hypersensitivity
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What is the classical example of a Type 4 Hypersensitivity?
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Tuberculin reaction
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What is the mechanism of damage of Type 4 hypersensitivity reaction?
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T cells, monocytes and macrophages
NO circulating antibodies |
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What are examples of type 4 hypersensitivity reactions?
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Contact Dermatitis
Tuberculin Test Chronic Transplant Reaction |
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What disease displays the characteristic butterfly rash over the face?
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Systemic Lupus Erythematous (SLE)
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Disease characterized by chronic inflammation of body tissues caused by autoimmune disease.
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Lupus
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What does Lupus produce in their blood that targets tissues within their body rather than foreign infectious agents?
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Abnormal Antibodies
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Where can lupus affect a person anatomically?
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Can potentially involve tissues anywhere in the body.
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What is lupus called when it only affects the skin?
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Discoid Lupus
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What is lupus called when it affects internal organs?
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Systemic lupus erythematous (SLE)
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What is the prevalence of lupus?
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women > men (8x)
Affects all ages/ Most common 20-45 |
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What is the common cause of death in lupus?
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Renal Failure
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Besides Renal failure what else is associated with later stages of Lupus?
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Severe CNS involvement
Acrocyanosis (Raynaud's phenomenon) |
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What types of auto-antibodies are specific for systemic lupus erythematous?
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Anti-DNA and Anti-Sm antibodies
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What is the precise reason known for the cause of lupus?
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Unknown
Poss. Drug induced Lupus |
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What are the drugs that account for _____ of the Drug induced lupus cases?
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Hydralazine (High BP)
Quinidine & procainamide (Ab Heart rhy) Phenytoin (Epilepsy) Penicillamine (TB) |
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What autoimmune disorder is caused by the production of antibodies against the acetylcholine receptor which results in the inhibition of neuromuscular transmission and eventual paralysis.
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Myasthenia Gravis
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Patients with _____ _____ have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematous.
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Myasthenia Gravis
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What disease is caused by inadequate release of acetylcholine rather than abnormal antibodies to acetylcholine receptors.
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Eaton-Lambert Syndrome
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What is the prevalence of Myasthenia Gravis?
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3 in 10,000
Any age poss. Most common: Young women, old men |
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What do 1% of people with Myasthenia Gravis develop?
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Thyoma (Tumor of the thymus)
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