Chapter 3 - Immunologic Diseases and Conditions

Question 1

Acquired Immunodeficiency Syndrome (AIDS)
(1) Description
(2) S & S
(3) Etiology
(4) Treatment and prognosis

Answer 1

(1) Progressive impairment of the immune system caused by HIV.
(2) In the period after exposure, symptoms include: flu-like illness, lymphadenopathy, weight loss, fatigue, diarrhea, night sweats. There is then a latent period during which there are no signs of the illness, though the immune system is progressively getting worse. In late stages, the virus causes meningitis, encephalitis, and dementia and many opportunistic infections arise that cause death without treatment
(3) HIV destroys CD4 cells (helper T-cells) leaving the body immunodeficient and the virus also directly attacks the immune system
(4) No cure, lifespan can be increased through anti-retroviral drugs and antibiotics for infections

Question 2

Common Variable Immunodeficiency (CVID) AKA Acquired hypogammaglobulinemia
(1) Description
(2) S & S
(3) Etiology
(4) Treatment and prognosis

Answer 2

(1) Acquired B-cell deficiency
(2) Chronic or recurrent infections, GI disease, lymphadenopathy, splenomegaly, hepatomegaly
(3) Due to possible unknown genetic factors causing failed B-cell differentiation, ability of B-cells to produced Ig is reduced
(4) Treated with immunoglobulin replacement and antibiotics to prevent infections. Increased risk of developing autoimmune disease and lymphoma

Question 3

Selective immunoglobulin A deficiency
(1) Description
(2) S & S
(3) Etiology
(4) Treatment and prognosis

Answer 3

(1) Failure to produce normal levels of IgA
(2) Can be asymptomatic if body compensates with extra IgM. If not: recurrent sinopulmonary infections, GI infections, and development of autoimmune disease
(3) Failure of B-cells to produce IgA; family history of CVID or this
(4) Antibiotics are given to prevent infection. Prognosis good

Question 4

X-linked (Bruton) Agammaglobulinemia
(1) Description
(2) S & S
(3) Etiology
(4) Treatment and prognosis

Answer 4

(1) Near absence of serum immunoglobulins
(2) Near-absent tonsils and adenoids, NO lymphadenopathy or splenomegaly, susceptible to gram positive infections
(3) Genetic; sex linked
(4) Immunoglobulin supplementation can help patient achieve near-normal life

Question 5

Severe Combined Immunodeficiency
(1) Description
(2) S & S
(3) Etiology

Answer 5

(1) Disorder of abnormal/disturbed T-cell formation or function. (Also hinders B-cell function since they are reliant on T-cell signaling)
(2) Severe recurrent infections of bacteria, fungi, viruses, and protozoa. Lymphoid tissue absent. Chronic diarrhea.
(3) Can be X-linked or autosomal recessive

Question 6

DiGeorge Anomaly (Thymic hypoplasia or aplasia)
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 6

(1) Congenital anomaly resulting in immunodeficiency; defective development of pharyngeal pouch system
(2) Structural abnormalities including: wide-set eyes, downward slanting eyes, small mouth, abnormalities of palate. Cardiovascular defects including: tetralogy of Fallot. Thymus and parathyroid glands absent or small. Frequent infections
(3) Microdeletion of part of chromosome 22
(4) Vitamin D and parathyroid hormone replacement, repair of cardiac anomalies, Thymic transplant

Question 7

Chronic mucocutaneous candidiasis
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 7

(1) Group of disorders causing immunodeficiency to candida organisms
(2) Large circular lesions on skin, mucus membranes, vagina, or nails. Recurrent thrush, diaper rash, mouth sores, and respiratory infections
(3) Specific candida T-cell deficit, likely genetic
(4) Antifungals; donated proteins from people with normal immune systems

Question 8

Wiskott-Aldrich Syndrome
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 8

(1) Congenital Disorder; inadequate B and T cell function,
(2) Eczema, thrombocytopenia, increased susceptibility to bacterial and viral infections, increased risk of autoimmune disorders and lymphoma
(3) X-linked recessive; protein defect leads to shrinking thymus
(4) Bone marrow transplant, splenectomy

Question 9

Autoimmune hemolytic anemia
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 9

(1) RBC destruction by antibodies
(2) Fatigue, weakness, chills, fever, dyspnea, itching, pallor, easy bruising, jaundice
(3) B-cell antibodies fail to recognize RBCs as “self”
(4) If caused by cold weather, avoid cold. If not, splenectomy or other treatments to reduce antibody effectiveness

Question 10

Pernicious anemia
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 10

(1) Antibody attacks on intrinsic factor and parietal cells causes decreased hydrochloric acid and intrinsic factor (needed to absorb B-12)
(2) Sore tongue, weakness, tingling/numbness in extremities, jaundiced skin while lips/gums/tongue are pale, disturbances in digestion. Demyelination of neurons causes neuritis. Peripheral weakness and cardiac arrhythmias
(3) Antibodies attack parietal cells and intrinsic factor. Resulting deficiency in B12 causes iron deficiency and nerve problems.
(4) B12 supplementation (intravenously)

Question 11

Idiopathic thrombocytopenic purpura
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 11

(1) Acquired disorder resulting from isolated deficiency of platelets
(2) Inability of blood to clot and spontaneous hemorrhages cause petechiae and ecchymoses, epistaxis, GI bleeding, menorrhagia, hematuria, easy bruising
(3) Idiopathic; related to antibodies that reduce life of platelets. Can occur after viral infection
(4) Blood transfusions, vitamin K administration, splenectomy as a last resort (usually effective)

Question 12

Immune Neutropenia
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 12

(1) Decreased number of circulated neutrophils due to anti-neutrophil antibodies
(2) Malaise, fatigue, weakness, fever, stomatitis, recurrent respiratory infections and otitis media
(3) Can be caused by: infection, drug exposure, immune thrombocytopenic purpura, autoimmune hemolytic anemia, or connective tissue disorders such as RA
(4) Corticosteroids, immune globulin, antibiotics for infections. Good prognosis; resolves spontaneously if in infants

Question 13

Goodpasture syndrome
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 13

(1) presence of antibodies directed at antigen in glomerular basement membrane (GBM)
(2) Glomerulonephritis, renal failure, proteinuria, anemia, hemoptysis, hematuria, weight loss, fatigue
(3) Membranes in glomerulus and alveoli are attacked by antibodies
(4) Plasmapheresis to remove anti-GBM antibodies; prognosis good for patients that survive more than 1 year

Question 14

Systemic lupus erythematosus
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 14

(1) Chronic, inflammatory disease caused by autoantibodies in the blood that target body tissues
(2) Characteristic “butterfly rash”; inflammation of skin, joints, nervous system, kidneys, lungs, and other organs,
(3) unknown; may be genetic, environmental, or hormonal factors
(4) Anti-inflammatory drugs, immunosuppressive agents for more serious disease

Question 15

Scleroderma (systemic sclerosis)
(1) Description (two types)
(2) S & S
(3) Etiology
(4) Treatment

Answer 15

(1) Chronic, progressive disease characterized by hardening of the skin
a. diffuse form: symmetric thickening of skin of extremities, face, and trunk
b. limited form: thickening of skin of fingers and face
(2) Hardening of skin and sometimes: GI tract, heart, lungs, and/or kidneys. Raynaud phenomenon is often first symptom
(3) Unknown; thought to be autoimmune. Abnormal activation of scar-forming cells
(4) No specific treatment; only palliative; prognosis depends on organs affected

Question 16

Sjogren Syndrome
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 16

(1) Autoimmune disease; inflammation of moisture secreting glands resulting in dryness
(2) Xerostomia (dry mouth), and subsequent difficulty talking, chewing, and swallowing; keratoconjunctivitis sicca (dry eyes); sores in mouth and nose
(3) Unknown; suspected genetic factors
(4) Aimed at symptoms: increased fluid intake, oral sprays, chewing sugarless gum, artificial tears, sunglasses

Question 17

Rheumatoid Arthritis
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 17

(1) Chronic, inflammatory disease of the joints
(2) Begins with unexplained weight loss, fatigue, fever, malaise, stiff joints. Progresses to bone destruction and joint deformation
(3) Unknown; can follow viral infections and genetic factors suspected
(4) Combination of medication (anti-inflammatory, NSAIDs, disease-modifying antirheumatic drugs (DMARDs)), rest, special exercises, and joint protection

Question 18

Juvenile rheumatoid arthritis
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 18

(1) Form of RA that affects children
(2) Fluctuating temperature with fever in evening, poor appetite, salmon-colored rash, anemia, swollen and painful joints
(3) Unknown; suspected to be an autoimmune disorder with genetic predisposition
(4) Similar to adult; physical therapy, balanced diet with protein

Question 19

Ankylosing spondylitis
(1) Description
(2) S & S
(3) Etiology
(4) Treatment

Answer 19

(1) Progressive inflammatory disease affecting spinal column
(2) Recurrent low-back pain in mornings that improves with activity; fatigue, fever, weight loss, limited range of motion
(3) Genetic factors suspected; unknown
(4) Physical therapy

Question 20

Polymyositis
(1) Description
(2) S & S
(3) Etiology

Answer 20

(1) Inflammatory disease of muscle fibers that has flares and relapses
(2) Muscle weakness mainly in trunk/torso. Can be accompanied by skin inflammation (rash on face, shoulders, arms)
(3) WBCs spontaneously invade and injure muscles

Question 21

Multiple sclerosis
(1) Description
(2) S & S
(3) Etiology

Answer 21

(1) Inflammatory disease of CNS bc autoimmune attack on myelin sheaths
(2) Weakness, numbness, optic neuritis, loss of vision in one eye, diplopia, unsteady gait, vertigo, difficulty urinating, facial numbness/pain, speech issues, dysphagia, hearing loss, fatigue, mental complications
(3) Unknown; genetic predisposition

Question 22

Myasthenia Gravis
(1) Description
(2) S & S
(3) Etiology

Answer 22

(1) Chronic, progressive neuromuscular disease caused by auto-antibodies attacking acetylcholine receptors
(2) Extreme muscular weakness w/o atrophy, progressive fatigue, drooping eyelids, diplopia, impaired speaking/chewing/swallowing. Weakness fluctuates hourly, daily, and/or yearly
(3) Autoantibodies; associated with thymus hyperplasia or thymoma

Question 23

Small vessel vasculitis
(1) Description
(2) S & S
(3) Etiology

Answer 23

(1) Vasculitis of capillaries, arterioles, and venules. Vasculitis is inflammation of blood vessels that causes them to become necrotic. Subsequent thrombosis may cause tissue infection
(2) Petechiae (rash with small spots), purpura, erythema, ulcerations, edema, pain/burning
(3) Unknown; risk factors include: autoimmune disorders, chemicals, certain proteins, meds, foods, and infections

Question 24

Systemic necrotizing vasculitis
(1) Description
(2) S & S
(3) Etiology

Answer 24

(1) Vasculitis of large and medium arteries
(2) Depends on body system or organ affected; headache, fever, weakness, malaise, anorexia, weight loss, muscle/joint pain, angina, dyspnea, hypertension, visual disturbances
(3) Unclear; depends on type; autoimmune response