Ch 3 Immunologic Diseases

Front 1

Polymorphonuclear neutrophils (PMNs) (Neutrophils)

Back 1

Phagocytose bacteria

Front 2

Hyperactive response

Back 2

Allergies. Immune system response is excessive

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Immunodeficiency disorders

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AIDS. Immune system is inadequate

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Autoimmune disorders

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Lupus. Immune response is overactive and misdirected against own tissues

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Natural killer cells

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Kill virus infected cells and tumor cells by secreting toxins

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Macrophages

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Phagocytose bacteria viruses and foreign substances

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Types of T cells

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Cytotoxic T cells (killer T cells)

Helper T cells

Suppressor T cells

Memory T cells

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T cells

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Major component of the type of acquired immunity known as cell mediated immunity

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Helper T cells

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Stimulate B cells to differentiate into plasma cells to produce more antibodies, activate killer T and macrophages

Front 10

Acquired immunodeficiency syndrome (AIDS)

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Progressive impairment of the immune system caused by human immunodeficiency virus (HIV)

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AIDS Signs/symptoms

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Initially not possible to tell if infected w/HIV by observing. May experience flulike illness for about 2 weeks.

Lymphadenopathy, weight loss, fatigue, diarrhea, night sweats. T cells lower, more frequent infections (pneumonia, fever, encephalopathy)

Front 12

AIDS Etiology

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HIV type 1(worldwide) or 2 (W. Africa). Destroy T cells leave body defenseless again infections and malignancies by reducing cell mediated immunity. Damages nervous system. W/o treatment last about 10 yrs. no cure or effective vaccine.

Front 13

AIDS Diagnosis

Back 13

ELISA (enzyme linked immunosorbent assay) if pos confirmed w/ western blot test. Positive p24 antigen indicates HIV antigen. ELISA neg first month. Rapid HIV antibody testing preferred method. Home testing kits available.

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AIDS Treatment

Back 14

No cure. Goal: prolong life and maintain quality of life. CD4 T cells and amount of HIV RNA are markers of disease progression and used to determine when to start HAART (3 drug combo: 2 nucleoside reductase inhibitors and either a nonnucleoside reductase inhibitor or protease inhibitor) serious side effects.

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AIDS Prognosis

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Ultimately fatal. Number of dying Americans dropped with proper treatment.

Front 16

Common Variable Immunodeficiency (Acquired Hypogammaglobulinemia) (CVID)

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Acquired B cell deficiency, decreased antibody production and/or function.

Front 17

CVID Signs/Symptoms

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Chronic or recurrent infections (pneumonia, bronchitis, sinusitis, otitis media, GI diseases). Lymphadenopathy, splenomegaly, hepatomegaly observed. Lung disease common. Increased frequency of autoimmune disorders.

Front 18

CVID Etiology

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Thought to be genetic defect of immune system dysregulation and failure of B cell differentiation. Exact cause unknown. B cell count normal but unable to produce all types of immunoglobulins.

Front 19

CVID Diagnosis

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Hypogammaglobulinemia and IgA and/or IgM deficiency indicative of disease.

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Suppressor T cells

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Inhibit both B and T cell activities and moderate immune response

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CVID Treatment

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Aimed at preventing infections. Regular immunoglobulin replacement decreases infections, antibiotic usage, hospitalization. At risk for lymphoma. Live virus vaccines no given.

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CVID Prognosis

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Often succumb to chronic lung disease.

Front 23

Selective Immunoglobulin A deficiency

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Fail to produce normal levels of IgA.

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Selective IgA deficiency Signs/Symptoms

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Asymptomatic bc increase of IgM production. Those w/Symptoms experience recurring sinopulmonary infections, GI infection, and/or autoimmune disease. Food allergies common. Anaphylactic rxn to blood transfusions many occur. Kids: otitis media, sinusitis, pneumonia.

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Selective IgA deficiency Etiology

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Failure of B cells to produce adequate levels of IgA. Family history common.

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Selective IgA deficiency diagnosis

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Levels of IgA measured, levels low but normal for IgG and IgM. CBC complement assay and vaccine response used to rule out immunodeficiencies. Diagnosis not definite until age 4 bc may spontaneously begin to produce IgA.

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Selective IgA deficiency Treatment

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No cure. Geared towards prevention and management. Prophylactic antibiotics given. Intravenous immune globulin (IVIG) w/low concentration of IgA may be tried.

Front 28

Selective IgA deficiency Prognosis

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Good. No significant medical problems.

Front 29

X linked Agammaglobulinemia

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Near absence of serum immunoglobulins and increased susceptibility to infection.

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X linked Agammaglbulinemia Signs/Symptoms

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Absent or near absent tonsils and adenoids. Lymphadenopathy and splenomegaly missing. Susceptible to recurring severe gamma pos infections (bacterial otitis media, bronchitis, pneumonia, osteomyelitis, meningitis) notice between 3-18 months when mother’s immunity has been depleted.

Front 31

Memory T cells

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Remain dormant until reactivated by original antigen allow more rapid response

Front 32

X linked agammaglobulinemia Etiology

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Congenital X linked disorder. Only affects males. Defect in Bruton tyrosine kinase (BTK) gene normally expressed in B cells. All 5 immunoglobulins absent as well as circulating B cells. Increased infections w/encapsulated bacteria.

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X linked Agammaglobulinemia diagnosis

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Family history. Serum antibody titers.

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X linked Agammaglobulinemia Treatment

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Intravenous infusions or subcutaneous administrations of immunoglobulin every 2-4 weeks and appropriate antibiotics administered. Should never have live virus vaccines.

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X linked Agammaglobulinemia prognosis

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Regular infusions of immunoglobulin may provide a near normal lifestyle. Miss more school and work and are hospitalized more than general population.

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Severe Combined Immunodeficiency (SCID)

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Disorders that result from disturbance in the development and function of T cells w or w/o B cells.

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Severe Combined Immunodeficiency (SCID) Signs/Symptoms

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Recurrent infection w/ bacteria, virus, fungi, Protozoa. Chronic diarrhea. Pneumocystis pneumonia, persistent mucocutaneous candidiasis. Discernible lymphoid tissue. Low or absent T cells.

Front 38

Severe Combined Immunodeficiency (SCID) Etiology

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2 types: X linked and autosomal recessive. Due to genetic mutations that lead to defects in stem cell differentiation into B and T cells. Resultin disruption in lymphocyte development.

Front 39

Severe Combined Immunodeficiency (SCID) Diagnosis

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Part of routine newborn screening in US. Measurement of immunoglobulin level, antibody titers, and number of T cells and B cells. Absence of thymus shadow on chest radiograph.

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Severe Combined Immunodeficiency (SCID) Treatment

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Bone marrow transplant only curative treatment. Gene therapy being investigated. Placed in completely sterile environment to prevent exposure to infectious agents. Live vaccines not administered.

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Severe Combined Immunodeficiency (SCID) Prognosis

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Unless underlying gene defect corrected children will die of overwhelming infections before 1 year.

Front 42

Reticuloendotheial system (mononuclear phagocytic system)

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Initiates T cell immune response

Front 43

DiGeorge Anomaly (Thymic Hypoplasia or Aplasia)

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Congenital. Defective development of pharyngeal pouch system, presents w/ cardiac anomalies, hypoplastic thymus, and hypocalcemia.

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DiGeorge Anomaly (Thymic Hypoplasia or Aplasia) Signs/Symptoms

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Structural abnormalities. Wide set, downward slanting eyes, low set ears with notched pinnae, small mouth, abnormalities in palate, cardiovascular defects. Thymus and parathyroid glands absent or underdeveloped. Degree of cognitive impairment.

Front 45

DiGeorge Anomaly (Thymic Hypoplasia or Aplasia) Etiology

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Result of abnormal development of 3rd and 4th pharyngeal pouches during 12 week of gestation. Most have microdeletion of part of chromosome 22.

Front 46

DiGeorge Anomaly (Thymic Hypoplasia or Aplasia) Diagnosis

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Reduced number of T cells and 2 of 3 features: cardiovascular defect, hypocalcemia of over 3 weeks duration, and microdeletion of chromosome 22. Absence, hypoplasia, or abnormal placement of thymus gland detected radiographically.

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DiGeorge Anomaly (Thymic Hypoplasia or Aplasia) Treatment

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Hypocalcemia treated to stabilize myocardium and reduce risk of seizures. Vitamin D and parathyroid hormone replacement therapy necessary. Repair of cardiac anomalies. Thymic transplantation or BMT to reconstitute immune function for severe forms.

Front 48

DiGeorge Anomaly (Thymic Hypoplasia or Aplasia) Prognosis

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Usually fatal w/in 1st yr. prognosis depends on severity of cardiac and parathyroid defects. Immune function may improve w/age.

Front 49

Chronic Mucocutaneous Candidiasis (CMC)

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Persistent and recurrent candidal ( fungal) infections of the skin, nails, and mucous membranes.

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Chronic Mucocutaneous Candidiasis (CMC) Signs/Symptoms

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Large circular lesions on skin, mucous membranes, nails, or vagina. Sores in mouth make eating difficult. Diaper rash, respiratory tract infections. Associated w/myasthenia gravis, thymoma, or bone marrow abnormalities.

Front 51

Chronic Mucocutaneous Candidiasis (CMC) Etiology

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T cell deficit specific to Candida making them susceptible to infection. Gene mutation in autoimmune regulator (AIRE) or STAT1 genes.

Front 52

Chronic Mucocutaneous Candidiasis (CMC) Diagnosis

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History or recurrent Candida infections. Genetic testing for AIRE mutations.

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Immunoglobulin

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Coated in B cells. Give ability to recognize foreign protein and stimulate an antigen-antibody rxn

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Chronic Mucocutaneous Candidiasis (CMC) Treatment

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Directed at eliminating infections along with correcting immunologic defects. Chronic use of antifungals.

Front 55

Chronic Mucocutaneous Candidiasis (CMC) Treatment

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Treatment w/antifungals has good results. Associated endocrinopathy or autoimmune disease major factor affecting prognosis.

Front 56

Wiskott-Aldrich Syndrome

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Congenital. Inadequate B and T cell function, thrombocytopenia, and eczema.

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Wiskott-Aldrich Syndrome Signs/ Symptoms

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Eczema, thrombocytopenia with severe bleeding (petechiae, pursues), increased susceptibility to bacterial and viral infection. Predisposed to development of autoimmune disorders (hemolytic anemia, vasculitis) lymphoma.

Front 58

Wiskott-Aldrich Syndrome Etiology

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Inherited as X linked trait affect only males. Mutations on WASP protein expressed in hematopoietic cells. Thymus decreases in size as child becomes older resulting in decreased B and T cell function.

Front 59

Wiskott-Aldrich Syndrome Diagnosis

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Mutation in WAS gene. Vaccine response may be defective. Regulatory T cell function impaired.

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Wiskott-Aldrich Syndrome Treatment

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BMT only curative therapy. High success rate if donor is human leukocyte antigen (HLA) matches sibling. Splenectomy may result in increased platelet number and size. IVIG and antibiotics therapy.

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Wiskott-Aldrich Syndrome prognosis

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Long term survival for patients who receive BMT before age 5. Splenectomy w/o BMT have median survival of 25 yrs. if neither die before age 5.

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Autoimmune diseases

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Body’s normal immune system mistakenly misdirected against own tissues.

Front 63

Actions of antigen-antibody complex

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Inactivation of pathogen or it’s toxins through direct binding.

Stimulation of phagocytosis (antibody targets infected cell by binding to surface)

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Active natural immunity

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Contract disease and produce memory cells

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Artificial active immunity

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Receive vaccine and produce memory cells

Front 66

Passive natural immunity

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Receive maternal antibodies through placenta or breast milk ( temporary won’t last forever)

Front 67

Passive artificial immunity

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Receive antiserum w/antibodies from another host. (Temporary immunity)

Front 68

Autoimmune Hemolytic Anemia

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RBCs are destroyed by antibodies

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Pernicious Anemia Treatment

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B12 injections. Blood replacement for severe cases

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Pernicious Anemia Prognosis

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No cure but early detection and treatment with B12 maintains normal hemogram and neurologic function.

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Idiopathic Thrombocytopenic Purpura

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Isolated deficiency of platelets. Platelet destruction and/or inhibition of platelet production.

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Idiopathic Thrombocytopenic Purpura Signs/Symptoms

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Inability of blood to clot and spontaneous hemorrhages in skin, mucous membranes, or internal organs. Petechiae, small spider like hemorrhage’s under skin, ecchymoses, large hemorrhage’s. Nose bleeds, GI bleeding, hematuria.

Front 73

Idiopathic Thrombocytopenic Purpura Etiology

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Unknown. Antibodies that reduce life of platelets often found. May occur after viral infection (rubella mumps)

Front 74

Idiopathic Thrombocytopenic Purpura Diagnosis

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CBC and peripheral blood smear preformed. Thrombocytopenia must be rules out first.

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Idiopathic Thrombocytopenic Purpura Treatment

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Corticosteroid administration increasing capillary integrity for short time. IVIG may be administered to increase platelet count. Anemia corrected w/blood transfusion and vitamin K to improve clotting. Therapeutic plasma exchange attempted, splenectomy last resort, however is effective.

Front 76

Idiopathic Thrombocytopenic Purpura Prognosis

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Children have spontaneous remission. Adult form rarely self limiting 1% die from disease.

Front 77

Immune Neutropenia

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Decreased number of circulating neutrophils

Front 78

Immune Neutropenia Signs/Symptoms

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Malaise, fatigue, weakness, fever, and stomatitis. Recurrent infections common especially upper respiratory and otitis media in infants.

Front 79

Autoimmune Hemolytic Anemia Signs/Symptoms

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Fatigue, weakness, chills, fever, dyspnea, itching. Skin is pale and jaundice. Bruises easily. Hypotensive.

Front 80

Immune Neutropenia Etiology

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Accelerated turnover of neutrophils (increased neutrophils production) but greater increase in destruction mediated by anti neutrophil antibodies. May be associated with infection, drug exposure, ITP, autoimmune hemolytic anemia, or connective tissue diseases.

Front 81

Immune Neutropenia Diagnosis

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Significantly reduced numbers of neutrophils in WBC count. Detection of anti neutrophil antibodies in immunofluorescence, agglutination tests, and antiglobulin analysis.

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Immune Neutropenia Treatment

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Resolved itself in infants. For severe case started with corticosteroids, immune globulin, or granulocyte colony stimulating factor (G CSF). Appropriate antibiotics administered, transfusions of WBCs

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Immune Neutropenia Prognosis

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Benign course with appropriate treatment.

Front 84

Goodpasture Syndrome

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Autoimmune kidney disease. Presence of antibodies directed against antigen in glomerular basement membrane.

Front 85

Goodpasture Syndrome Signs/Symptoms

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Acute glomerulonphritis, acute renal failure with proteinuria, anemia, hemoptysis, hematuria. Weight loss, fatigue, fever.

Front 86

Goodpasture Syndrome Etiology

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Antibodies cause complement mediated tissue damage in glomerular and alveolar basement membranes. Progression leads to end stage renal failure.

Front 87

Goodpasture Syndrome Diagnosis

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Detection of anti GBM antibodies in serum or kidneys. ELISA, immunofluorescence, or renal biopsy preformed. Urinalysis indicates presence of protein and blood in urine.

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Goodpasture Syndrome Treatment

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Plasmapheresis (removes anti GBM antibody) combined with immunosuppressive agents ( corticosteroids and cyclophosphamide) for 6-12 months. Hemodialysis and kidney transplants last resort.

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Goodpasture Syndrome Prognosis

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Self limiting. Those who survive for a year after diagnosis and maintain intact renal function do well. Survival depends on renal impairment and pulmonary involvement. Relapses occur.

Front 90

Autoimmune Hemolytic Anemia Etiology

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B cell produced antibodies do not identify RBCs as self. 2 types: warm antibody anemia (excess IgG) at body temp and cold antibody anemia (excess IgM) at colder temps.

Front 91

Systemic Lupus Erythematosus (SLE)

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Unusual autoantibodies in blood target tissues of the body.

Front 92

Autoimmune Hemolytic Anemia Diagnosis

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Direct Coombs test indicates antibody coated RBCs that agglutinate when antiglobulin is added to medium. Bilirubin is elevated. RBC count, platelet count, hemoglobin concert, and hematocrit decreased. Peripheral blood smear examination.

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Autoimmune Hemolytic Anemia Treatment

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Address warm v cold anemia. Warm use corticosteroids and cytotoxic drugs to reduce antibody production. Splenectomy or IVIG administration to reduce antibody production.

Cold: avoid cold by dressing warmly, plasmapheresis may be helpful.

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Autoimmune Hemolytic Anemia Prognosis

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Warm antibody anemia is generally self limiting in children disappearing within 1-3 months. Other is chronic and poorly responsive to therapy.

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Pernicious Anemia

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Caused by chronic atrophic gastritis resulting in decreased gastric production of HCl and shortage of intrinsic factor. Impairment of B12 absorption and B12 deficiency.

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Pernicious Anemia Signs/Symptoms

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Sore tongue, weakness, tingling and numbness in extremities. Lips tongue and gums appear pale, skin and sclera are jaundice. Disturbance in digestion (anorexia, nausea, vomiting, diarrhea, constipation, flatulence, weight loss). Demyelination of nerves and spinal cord due to B12 deficiency. CNS changes, congestive heart failure.

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Pernicious Anemia Etiology

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Anti-parietal cell antibodies (can be cytotoxic to parietal cells). Also have anti-intrinsic factor antibodies (intrinsic factor needed for B12 absorption). Associated w/ Graves’ disease and Hashimoto thyroditis. Common in Northern Europeans.

Front 98

Pernicious Anemia Diagnosis

Back 98

Schillings rest and/or blood evaluation for antiparietal cell or anti intrinsic factor antibodies. Blood test reveal decreased hemoglobin level, RBC count, and increased mean cell volume.

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Systemic Lupus Erythematosus (SLE) Signs/Symptoms

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Inflame or damage connective tissue. Inflammation of skin, joints, nervous system, kidney, lungs, other organs. Butterfly rash on face. Fever, fatigue, joint pain, malaise. Raynaud phenomenon and hair loss common.

Front 100

Systemic Lupus Erythematosus (SLE) Etiology

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Unknown. Thought to be autoimmune. Stress, immunization rxn, pregnancy, overexposure to Uv light precipitate SLE. common in women 30-40 yrs.

Front 101

Systemic Lupus Erythematosus (SLE) Diagnosis

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Formal diagnosis made if 4 or more of symptoms or pos lab results are present at same time:

Butterfly rash, discoid skin lesion, photosensitivity, nasopharyngeal ulceration, polyarthritis w/o deformity, seizures or psychosis, chronic pleuritis or pericarditis, false pos serologic for syphilis, hemolytic anemia, thrombocytopenia

Front 102

Systemic Lupus Erythematosus (SLE) Treatment

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Anti inflammatory drugs (aspirin) for fever or joint pain. Corticosteroids, immunosuppressive when organ threatening

Front 103

Systemic Lupus Erythematosus (SLE) Prognosis

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Depends on internal organ involvement. At risk for cancer and heart disease

Front 104

Scleroderma (Systemic Sclerosis)

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Sclerosis (hardening) of the skin, and certain internal organs.

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Scleroderma (Systemic Sclerosis) Signs/Symptoms

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Hardening and shrinking of the skin and certain internal organs, including GI tract, heart, lungs, and kidney. Skin becomes taut, firm, and edematous, is firmly attached to subcutaneous tissue. Raynaud’s phenomena often first symptom.

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Sjögren Syndrome Prognosis

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Depends on complications. Infections in respiratory tract. Cancer of lymph glands is rare.

Front 107

Rheumatoid Arthritis

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Inflammatory disease affects joints

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Rheumatoid Arthritis Signs/Symptoms

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Inflammation and edema of synovial membranes surrounding joints. Has capacity to destroy cartilage and surrounding bones, deform joints. Experience unexplained weight loss, fatigue, low grade fever, malaise. Edema, pain, tenderness in joints.

Front 109

Rheumatoid Arthritis Etiology

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Unknown. Genetic or viral infection.

Front 110

Rheumatoid Arthritis Diagnosis

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Based on symptoms, family history, physical exam, radiographic study, blood test that show elevated rheumatoid factor. CBC, synovial fluid analysis.

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Rheumatoid Arthritis Treatment

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Primary objective is reduction of inflammation and pain to preserve joint function and deformities. Aspirin to ease pain, NSAIDs as aspirin alternative, corticosteroids for flare ups. Special splints. Surgery last resort.

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Rheumatoid Arthritis Prognosis

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Depends on adequate treatment. Early medical intervention best outcome.

Front 113

Juvenile Rheumatoid Arthritis

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Affects children less than 16 yrs begins between age 2-5.

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Juvenile Rheumatoid Arthritis Signs/Symptoms

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Various forms: pauciarticular (few joints), polyarticular (many joints), systemic (high fever and rash. Systemic symptoms: temp fluctuates, poor appetite, blotchy rash, anemia, swollen stiff joints. Skeletal development may be impaired

Front 115

Juvenile Rheumatoid Arthritis Etiology

Back 115

Unknown. Autoimmune or Hereditary?

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Scleroderma (Systemic Sclerosis) Etiology

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Unknown. Appears to be autoimmune disease. Scar forming cells activated. 4x more active in women.

Front 117

Juvenile Rheumatoid Arthritis Diagnosis

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Based on history and physical exam. Blood test for rheumatoid factor. May show lack or delayed growth.

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Juvenile Rheumatoid Arthritis Treatment

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Similar to adult. Physical therapy, braces or splints.

Front 119

Juvenile Rheumatoid Arthritis Prognosis

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Usually improve over time. Majority grow to lead normal life.

Front 120

Ankylosing Spondylitis

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Progressive inflammatory disease affecting spinal column.

Front 121

Ankylosing Spondylitis Signs/Symptoms

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Common in young men. First affects sacroiliac area. Recurrent lower back pain. Fatigue, weight loss, fever, diarrhea, eye pain, photophobia. Inflammation and ossification can progress up the spine. Vertebrae becomes fused.

Front 122

Ankylosing Spondylitis Etiology

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Unknown. Genetic may be linked to antigen HLA-B27

Front 123

Ankylosing Spondylitis Diagnosis

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Lab results beg for rheumatoid factor. Mildly elevatedESR or C reactive protein, HLA-B27 antigen present in 90%. Bamboo spine.

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Ankylosing Spondylitis Treatment

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No cure. Goal to relieve pain with anti inflammatory. Physical therapy.

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Ankylosing Spondylitis Prognosis

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Variable periods of remission. Can be severe and rapid with severe deformities.

Front 126

Polymyositis

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Inflammation of muscle fibers

Front 127

Scleroderma (Systemic Sclerosis) Diagnosis

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Complete history and physical examination are all that are necessary for diagnosis to be made.

Front 128

Polymyositis Signs/Symptoms

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Muscle weakness close to body trunk. Can be gradual or rapid. Trouble swallowing and lifting head. Difficulty getting up, climbing stairs, lifting. Skin inflammation. Mostly women.

Front 129

Polymyositis Etiology

Back 129

Unknown. Immune cells of inflammation invade and injure muscle

Front 130

Polymyositis Diagnosis

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Medical history, physical exam, blood analysis show elevated levels of muscle enzymes. EMG shows abnormal electrical activity.

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Polymyositis Treatment

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Directed towards stopping inflammation and inhibiting overactive immune system. Immunosuppressant. Exercise therapy later added.

Front 132

Polymyositis Prognosis

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Ultimately can recover. Disease often becomes inactive

Front 133

Multiple Sclerosis

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Inflammation of CNS. attacks myelin sheath debilitating nerves

Front 134

Multiple Sclerosis Signs/Symptoms

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Weak/ numb in one limb, loss of vision in one eye,Diplopia, unsteady gait, vertigo, difficulty urinating, speech problems, hearing loss, face numbness, depression short temper.

Front 135

Multiple Sclerosis Etiology

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Unknown. Virus? Inherited?

Front 136

Multiple Sclerosis Diagnosis

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Challenging to Diagnose. Have two or more distinct episodes of CNS dysfunction. MRI used to diagnosis. Examine CSF, elevated immunoglobulin

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Multiple Sclerosis Treatment

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Corticosteroids. Immunosuppressive therapies. Treated symptomatically.

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Scleroderma (Systemic Sclerosis) Treatment

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No specific treatment known. Treatment directed at areas affected. Physical therapy helps maintain muscle strength does not change course of joint disease.

Front 139

Multiple Sclerosis Prognosis

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No cure. Can die w/in few months. Avg duration is 30 yrs or longer.

Front 140

Myasthenia Gravis

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Chronic progressive neuromuscular disease caused by autoantibodies to acetylcholine receptor at nerve synapse.

Front 141

Myasthenia Gravis Signs/Symptoms

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Extreme muscular weakness w/o atrophy and progressive fatigue. Dropping eyelids, diplopia, difficulty talking, chewing, swallowing. Prolonged exposure to sunlight, cold, infections, and emotional stress exacerbate symptoms

Front 142

Myasthenia Gravis Etiology

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Mainly women 20-40. Men over 60. Autoimmune, majority have thymus hyperplasia or thymoma.

Front 143

Myasthenia Gravis Diagnosis

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Physical exam to text fatigability of diff muscles. Tensilon test, iv administration of short acting acetylcholinesterase inhibitor to see if muscle strength improves. Look for antibodies against acetylcholine.

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Myasthenia Gravis Treatment

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Symptomatic and supportive. Anticholinestrase drugs for fatigue and weakness but become less effective as disease progresses.

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Myasthenia Gravis Prognosis

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Unexplained spontaneous remission can occur but disease is lifelong.

Front 146

Small Vessel Vasculitis

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Affects capillaries, arterioles, venules.

Front 147

Small Vessel Vasculitis Signs/Symptoms

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Inflammation of small vessels cause petechiae, purpura, erythema, ulcerations, edema. Often lower extremities. Pain and burning sensation. Ocular lesions, genital or oral ulceration, abdominal pain, arthralgia, weakness.

Front 148

Small Vessel Vasculitis Etiology

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Unknown. Immune disorder, exposure to chemicals, meds, foods, infections are possible cause.

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Scleroderma (Systemic Sclerosis) Prognosis

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Depends on organs affected. Patients w/ pulmonary hypertension, interstitial lung disease, and uncontrolled bp have higher risk of mortality.

Front 150

Small Vessel Vasculitis Diagnosis

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History and immunofluorescence studies of biopsy tissue.

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Small Vessel Vasculitis Treatment

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Corticosteroids therapy. Analgesic and rest encouraged.

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Small Vessel Vasculitis Prognosis

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If developed due to meds avoid medications.

Front 153

Systemic Necrotizing Vasculitis

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Affect medium and large arteries.

Front 154

Systemic Necrotizing Vasculitis Signs/Symptoms

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Headache, fever, weakness, fatigue, malaise, anorexia, weight loss. Muscle or joint pain, angina, dyspnea, hypertension, visual disturbance. Paralysis of nerve.

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Systemic Necrotizing Vasculitis Etiology

Back 155

Unknown. Autoimmune. Illicit drug use.

Front 156

Systemic Necrotizing Vasculitis Diagnosis

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Complete history and physical. Blood studies (CBC, ESR, RA factor determination) radiograph if pulmonary system involved. Angiogram.

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Systemic Necrotizing Vasculitis Treatment

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Focuses on decreasing inflammation of arteries and improving functioning affected organ. Corticosteroids, immunosuppressant.

Front 158

Systemic Necrotizing Vasculitis Prognosis

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Guarded until conditions controlled. Can permanently damage organs.

Front 159

Sjögren Syndrome

Back 159

Inflammation in moisture secreting glands. Lead to dryness in affected areas.

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Sjögren Syndrome Signs/Symptoms

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Xerostomoia, keratoconjunctivitis sicca. Dryness of nasal, oral, and laryngeal pharynx make it difficult to talk, chew, swallow. Experience sores on mouth, nose, dental decay may occur.

Front 161

Sjögren Syndrome Etiology

Back 161

Unknown. Genetics play role. Most females.

Front 162

Sjögren Syndrome Diagnosis

Back 162

Dryness of eyes defined by abnormal Schirmer test. Enlarged salivary glands, Lower lip biopsy indicates infiltration of lymphocytes. SSA and SSB antibodies found.

Front 163

Sjögren Syndrome Treatment

Back 163

Directed towards relieving symptoms especially dryness in eyes and oral cavity. Increased fluid intake, chew sugarless gum, oral sprays, artificial tears, wearing sunglasses. Block tear ducts to maintain moisture.