a = new Array(221);var f = "<FONT FACE=ARIAL SIZE=2>"a[0] = ""a[1] = f + "(A) CORRECT. Food allergies are typically a form of type I hypersensitivity reaction. The allergens react with IgE bound to mast cells, mainly in skin and gastrointestinal tract."a[2] = f + "(B) Incorrect. Cell-mediation is a feature of Type IV hypersensitivity."a[3] = f + "(C) Incorrect. Release of complement C3b is a feature of Type III hypersensitivity."a[4] = f + "(D) Incorrect. Plasma cells are not involved in this clinical setting. Increases in immunoglobulin occur over days to weeks."a[5] = f + "(E) Incorrect. Localized immune complexes occur in an Arthus reaction with Type III hypersensitivity. Systemic immune complex deposition can occur in diseases such as SLE."a[6] = f + "(A) Incorrect. Rheumatoid arthritis is a chronic inflammatory disease that may lead to deposition of AA amyloid."a[7] = f + "(B) Incorrect. Tuberculosis is a chronic infectious disease that may lead to deposition of AA amyloid."a[8] = f + "(C) Incorrect. SLE is a chronic inflammatory disease that may lead to deposition of AA amyloid."a[9] = f + "(D) CORRECT. The light chains elaborated by the plasma cells from the myeloma contribute to the formation of the amyloid. Not all patients with AL amyloid, however, have multiple myeloma."a[10] = f + "(E) Incorrect. There is an amyloid angiopathy with Alzheimer disease, but it is found in cortical arteries the brain, and is not limited to light chains."a[11] = f + "(A) Incorrect. A decreased CD4 lymphocyte count is more typical of AIDS (an immune deficiency) than of an immunologically mediated disease process such as SLE."a[12] = f + "(B) Incorrect. The findings given are non-specific and do not point to a septicemia."a[13] = f + "(C) CORRECT. These non-specific findings put together suggest possible autoimmune disease. An ANA is a good way to begin the workup, then more specific tests can be ordered. Antigen-antibody complexes of many autoimmune diseases can precipitate in a variety of locations, typically trapped in basement membranes in sites such as skin, synovium, pleura, pericardium, and glomeruli."a[14] = f + "(D) Incorrect. The sed rate is a very non-specific test that is elevated in many inflammatory conditions. The history here points more to an immunologic disease."a[15] = f + "(E) Incorrect. CPK is more useful for diagnosis of an acute disease -- myocardial infarction. CPK can be increased with myositis, but with the multiple manifestations in this woman, it is not the best screening test."a[16] = f + "(A) Incorrect. DiGeorge syndrome is predominantly the result of a loss of T-cell function. This is pronounced with complete DiGeorge syndrome, with lesser severity in partial DiGeorge syndrome. There are often congenital anomalies."a[17] = f + "(B) Incorrect. A selective IgA deficiency is seen in about 1 in 600 persons and leads to mild diarrhea and/or occasional respiratory tract infections, but is not life-threatening."a[18] = f + "(C) Incorrect. EBV infection does not lead to immunodeficiency states."a[19] = f + "(D) Incorrect. Acute leukemias can result in immune deficiency, but not so selective and not typically in infancy."a[20] = f + "(E) CORRECT. Recurrent bacterial infections suggest a lack of B-cell immune function with lack of gamma globulin production. Once maternal antibody is gone, the disease is manifested more severely."a[21] = f + "(A) Incorrect. Amyloidosis is a complication from chronic inflammation over many years or from multiple myeloma."a[22] = f + "(B) Incorrect. Cytotoxic CD8 cell activation is primarily a function of class I antigen recognition."a[23] = f + "(C) Incorrect. GVHD is not a consequence of renal transplantation, and few if any donor lymphocytes are present in the allograft."a[24] = f + "(D) CORRECT. Class II HLA antigens are involved in the process of CD4 lymphocyte activation."a[25] = f + "(E) Incorrect. Serum sickness results from antigen-antibody complexes."a[26] = f + "(A) Incorrect. Abnormalities of NADPH oxidase are found with chronic granulomatous disease."a[27] = f + "(B) Incorrect. Wiskott-Aldrich syndrome has atopy with eczema, thrombocytopenia, and immune defects in both cell mediated and humoral arms. The protein is involved with bridging cell surface receptors to actin filaments."a[28] = f + "(C) Incorrect. The most common form of severe combined immunodeficiency (SCID) seen in boys is the X-linked defect in the common gamma chain of the cytokine receptor involved in signlaing for many of the interleukins."a[29] = f + "(D) CORRECT. He has hyper-IgM syndrome, an X-linked disorder. The CD40-CD40L interaction is involved with isotype switching from IgM production, thus accounting for his lack of IgG and IgA. This accounts for bacterial infections. However, some degree of cell mediate immune defect is present as well."a[30] = f + "(E) Incorrect. Lack of C1inh leads to hereditary angioedema."a[31] = f + "(A) Incorrect. The cardiac muscle is usually not involved with polymyositis."a[32] = f + "(B) Incorrect. Myositis is not typical for scleroderma."a[33] = f + "(C) Incorrect. Polymyositis is not accompanied by neuropathy. A neuropathy by itself would not account for inflammation in muscle."a[34] = f + "(D) CORRECT. Polymyositis-dermatomyositis is present. The violaceous skin rash is often quite subtle and limited to the face."a[35] = f + "(E) Incorrect. Renal failure is more typical of systemic lupus erythematosus."a[36] = f + "(A) Incorrect. Amyloid deposition does not result in an immune response."a[37] = f + "(B) Incorrect. Both cell-mediated and humoral immune mechanisms play a role in syphilis, but not specifically a type I hypersensitivity reaction."a[38] = f + "(C) Incorrect. Natural immune mechanisms directed against neoplasia are not generally effective."a[39] = f + "(D) Incorrect. Some organic dusts can lead to a localized type III reaction."a[40] = f + "(E) CORRECT. Parasitic infestations can be accompanied by a type I hypersensitivity reaction."a[41] = f + "(A) Incorrect. DLE is characterized mainly by skin findings."a[42] = f + "(B) CORRECT. The nucleolar pattern suggests scleroderma (systemic sclerosis) more than the other autoimmune diseases, and likely diffuse scleroderma. Complications of scleroderma include hypertension, though signs of renal failure may not occur unless the complication of malignant hypertension supervenes. She also has features of Raynaud phenomenon and pulmonary fibrosis."a[43] = f + "(C) Incorrect. The major autoantibody associated with PM is Jo-1. Renal disease is not a feature of this complex."a[44] = f + "(D) Incorrect. SS primarily affects salivary and lacrimal glands."a[45] = f + "(E) Incorrect. RA mainly affects small joints and is associated with an increased rheumatoid factor (RF). Renal disease is not a major feature of RA."a[46] = f + "(A) Incorrect. Type I hypersensitivity reactions typically occur immediately upon contact with the antigen."a[47] = f + "(B) Incorrect. A rash is not typical for type II reactions."a[48] = f + "(C) Incorrect. These findings are not typical for antigen-antibody complex disease."a[49] = f + "(D) CORRECT. This is a contact dermatitis, typical of exposure to a plant such as poison ivy."a[50] = ""a[51] = f + "(A) Incorrect. This is typical for type I hypersensitivity reactions, such as allergic reactions."a[52] = f + "(B) Incorrect. Anti-GBM is a feature of Goodpasture's syndrome, which produces a linear pattern of immunofluorescence."a[53] = f + "(C) CORRECT. A granular pattern of glomerular immunofluorescence staining is typical of immune complex deposition that occurs with a type III hypersensitivity reaction. This patient's findings suggest SLE."a[54] = f + "(D) Incorrect. These findings do not suggest cell mediated immunity with a type IV hypersensitivity reaction."a[55] = f + "(E) Incorrect. Prostaglandin release occurs with acute inflammation and leads to increased vasodilation."a[56] = f + "(A) Incorrect. In this form of type II hypersensitivity, an antibody is directed against a cellular receptor, such as against acetylcholine receptor in myasthenia gravis."a[57] = f + "(B) Incorrect. Loss of self tolerance is found in many autoimmune diseases."a[58] = f + "(C) Incorrect. Immune complexes are found in many autoimmune diseases and can produce inflammation, but not anemia."a[59] = f + "(D) Incorrect. Type IV hypersensitivity is a feature of inflammation wtih granuloma formation or transplant rejection."a[60] = f + "(E) CORRECT. This is immune hydrops, probably from Rh incompatibility, resulting in maternal antibody crossing the placenta and destroying baby's RBCs through complement mediated lysis, a form of type II hypersensitivity reaction."a[61] = f + "(A) CORRECT. Acute rheumatic fever follows a group A beta hemolytic streptococcal infection. There is cross-reactivity of the immune response to streptococcal M proteins, with the heart mainly affected."a[62] = f + "(B) Incorrect. This mechanism is an example of an autoimmune disease such as SLE, which probably results from several immunologic derangements that result in B-cell hyperactivity."a[63] = f + "(C) Incorrect. This mechanism can be seen with rheumatoid arthritis, which may result from modification of self-antigen (collagen) so that it is recognized as foreign by T-cells."a[64] = f + "(D) Incorrect. This mechanism can be seen with ocular trauma to produce sympathetic ophthalmia in the non-injured eye."a[65] = f + "(E) Incorrect. This is a presumed mechanism for type I diabetes mellitus with destruction of islet cells."a[66] = f + "(A) CORRECT. She has rheumatoid arthritis, which is a chronic inflammatory condition that can be complicated by amyloidosis of the reactive systemic variety with SAA protein contributing to amyloid deposition in a variety of organs."a[67] = f + "(B) Incorrect. Light chains are derived from monoclonal antibodies produced from plasma cells. Some of these patients have multiple myeloma."a[68] = f + "(C) Incorrect. Transthyretin protein can be the precursor for amyloid in some familial forms of amyloidosis and in cardiac amyloidosis."a[69] = f + "(D) Incorrect. APP is seen in the neuritic plaques in the cerebral cortex of persons with Alzheimer disease."a[70] = f + "(E) Incorrect. Beta-2-microglobulin contributes to amyloid deposition in persons on chronic hemodialysis."a[71] = f + "(A) Incorrect. Anti-centromere is a helpful antibody to determine a diagnosis of limited scleroderma (CREST syndrome), but he has more findings than accounted for by CREST."a[72] = f + "(B) CORRECT. The anti-RNP suggests a diagnosis of mixed connective tissue disease (MCTD), which has elements of SLE, rheumatoid arthritis, and scleroderma. Unlike SLE, MCTD is unlikely to be accompanied by severe renal complications."a[73] = f + "(C) Incorrect. Though he has arthritis, the other findings are less typical for just rheumatoid arthritis."a[74] = f + "(D) Incorrect. The C1q is often decreased with SLE, but it is not the most specific test."a[75] = f + "(E) Incorrect. The findings suggest an autoimmune disease more than an immunodeficiency disease."a[76] = f + "(A) Incorrect. Type I reactions are associated with allergy and anaphylaxis."a[77] = f + "(B) Incorrect. Type II reactions are associated with complement-mediated immune reactions."a[78] = f + "(C) Incorrect. GVHD does not produce a granulomatous reaction."a[79] = f + "(D) Incorrect. Granulomatous reactions are based mostly upon cell-mediated immunity."a[80] = f + "(E) CORRECT. Granuloma formation with reactivation or reinfection tuberculosis in an adult is a classic type IV hypersensitivity reaction."a[81] = f + "(A) Incorrect. Anaphylaxis with type I reactions is immediate."a[82] = f + "(B) Incorrect. These reactions are not characterized by immune complex deposition."a[83] = f + "(C) CORRECT. The child has a poststreptococcal glomerulonephritis typical of immune complex deposition,  a form of type III hypersensitivity."a[84] = f + "(D) Incorrect. This is not typical for cell mediated immunity."a[85] = ""a[86] = f + "(A) Incorrect. MCTD is not characterized by significant skin involvement."a[87] = f + "(B) Incorrect. DLE is characterized by immune complex deposition with a rash, not fibrosis."a[88] = f + "(C) CORRECT. The CREST syndrome is a more benign, limited form of systemic sclerosis (scleroderma) in which fibrosis and tightening of the skin is common. She has esophageal dysmotility. However, she has not developed respiratory or renal complications."a[89] = f + "(D) Incorrect. Dermatomyositis is characterized by inflammation and a rash, not fibrosis."a[90] = f + "(E) Incorrect. Amyloid is pink, amorphous material, not collagen."a[91] = f + "(A) Incorrect. IL-1 is more involved with the appearance of fever with inflammation."a[92] = f + "(B) Incorrect. Bradykinin mediates the appearance of pain and of vascular permeability."a[93] = f + "(C) Incorrect. Complement C5a is a chemotactic factor."a[94] = f + "(D) CORRECT. She is having a type 1 hypersensitivity reaction with systemic anaphylaxis, in part mediated by histamine released from mast cells, which causes vascular permeability. About 20% of all persons will have some reaction to penicillin or similar drugs, and a few of these will be severe."a[95] = f + "(E) Incorrect. Thromboxane generated by the cyclo-oxygenase pathway of arachidonic acid metabolism mediates platelet aggregation and vasoconstriction to promote hemostasis."a[96] = f + "(A) Incorrect. The bacterial infections suggests hypogammaglobulinemia, not a primary or secondary T cell defect as could occur with human immunodeficiency virus infection."a[97] = f + "(B) Incorrect. Anti-Jo1 is more specific for polymyositis."a[98] = f + "(C) Incorrect. Bruton's disease manifests early in childhood with a marked reduction in all serum globulins."a[99] = f + "(D) CORRECT. He has common variable immunodeficiency (CVID), with hypogammaglobulinemia that predisposes him to bacterial infections and also <I>Giardia</I> infection. Patients with CVID can have rheumatoid arthritis and hemolytic anemia. Most patients have normal to slightly reduced numbers of B lymphocytes that cannot differentiate into immunoglobulin-secreting plasma cells."a[100] = f + "(E) Incorrect. Though persons with diabetes mellitus are prone to bacterial infections, they do not get a Giardia diarrhea or hemolytic anemia or arthritis."a[101] = f + "(A) Incorrect. Atherosclerosis, if anything, is probably less severe in a patient with systemic lupus erythematosus."a[102] = f + "(B) CORRECT. Systemic lupus erythematosus manifestations are often mediated through a type III hypersensitivity reaction with antigen-antibody complexes. These complexes can lodge in the glomeruli. The immune complexes tend to to be trapped along basement membranes in epithelial and mesothelial surfaces."a[103] = f + "(C) Incorrect. Although chronic inflammatory diseases may lead to amyloidosis, this is less common than renal disease due to antigen-antibody complexes with SLE."a[104] = f + "(D) Incorrect. Although chronic inflammation may be present with vasculitis with SLE, immune deposition plays a greater role in development of renal disease."a[105] = f + "(E) Incorrect. Goodpasture's syndrome, not systemic lupus erythematosus, is associated with anti-GBM."a[106] = f + "(A) Incorrect. Thrombocytopenia leads to appearance of petechiae and purpuric areas on the skin."a[107] = f + "(B) CORRECT. The rash is typical for graft versus host disease (GVHD). The donor lymphocytes engraft and attack host tissues, including epidermal keratinocytes, causing apoptosis. The two other tissues often affected by GVHD include liver and GI tract."a[108] = f + "(C) Incorrect. Gangrenous necrosis is very extensive and involves an entire body part, including soft tissues."a[109] = f + "(D) Incorrect. Contact dermatitis is a form of type IV hypersensitivity and produces erythema and swelling of skin in affected areas exposed to the antigen."a[110] = f + "(E) Incorrect. Abrasions produce a scraping type of injury."a[111] = f + "(A) CORRECT. This patient has the classic malar rash of lupus, but without a positive ANA or other findings, discoid lupus limited to the skin is the most likely diagnosis. Some patients with DLE (particularly those with a positive ANA) may go on to develop SLE."a[112] = f + "(B) Incorrect. Scleroderma leads to dermal fibrosis, without vasculitis and without erythema."a[113] = f + "(C) Incorrect. Contact dermatitis does not usually have a pattern of involvement over the malar regions and is related to antigen exposure, not sun exposure."a[114] = f + "(D) Incorrect. SLE would typically have a positive ANA."a[115] = f + "(E) Incorrect. Dermatomyositis does not usually have a malar rash. The eyelids are most often affected."a[116] = f + "(A) Incorrect. The anti-glomerular basement membrane antibody of Goodpasture's produces a linear pattern on immunofluorescence in glomeruli and alveoli, but not skin."a[117] = f + "(B) Incorrect. Though patients with SLE may have arthralgias, there is no significant erosion of joint surfaces and no bony anklyosis or deformity, such as may be seen with rheumatoid arthritis."a[118] = f + "(C) Incorrect. Nodular glomerulosclerosis, typically seen with diabetes mellitus, does not have immune complex deposition."a[119] = f + "(D) Incorrect. Significant pulmonary fibrosis is a feature of diffuse scleroderma, not SLE."a[120] = f + "(E) CORRECT. A granular pattern of staining with C1q is characteristic for systemic lupus erythematosus with immune complex deposition with type III hypersensitivity. Immune complexes tend to deposit where basement membranes beneath epithelial and mesothelial surfaces trap them."a[121] = f + "(A) CORRECT. Severe combined immunodeficiency (SCID) may be due to adenosine deaminase (ADA) deficiency in about half of cases. This disorder leads to malfunction of both humoral (B-cell) and cell-mediated (T-cell) immunity. Viral, fungal, and bacterial infections occur frequently in the first year of life."a[122] = f + "(B) Incorrect. The findings with common variable immunodeficiency resemble those of X-linked agammaglobulinemia, but have their onset in late teenage years. Giardiasis is common."a[123] = f + "(C) Incorrect. HIV infection could lead to multiple opportunistic infections but would not explain the thymic hypoplasia."a[124] = f + "(D) Incorrect. One mechanism for development of common variable immunodeficiency is autoantibody formation, with targeting of lymphocytes."a[125] = f + "(E) Incorrect. Thymic hypoplasia in DiGeorge syndrome leads to T-cell deficiency with symptoms related to failure of cell-mediated immunity -- viral, fungal, and protozoal infections."a[126] = f + "(A) Incorrect. Neutrophils release proteases that can produce local destruction of microbes and host cells."a[127] = f + "(B) CORRECT. The primary mediator of the stuffy nose with hay fever (from allergies to plant pollens) is histamine released from mast cells. Why do you think so many anti-histaminic drugs, such as the ones he received, are sold? Up to 10% of persons have some degree of atopy."a[128] = f + "(C) Incorrect. CD4+ and CD8+ cells are more important in cell-mediated immune responses."a[129] = f + "(D) Incorrect. NK cells help in destruction of antibody-coated cells and in cells infected with intracellular microbes such as viruses."a[130] = f + "(E) Incorrect. Macrophages help to clean up an inflammatory mess, but are not part of an immediate response."a[131] = f + "(A) CORRECT. The history suggests exposure to an allergen at home to which she is not exposed elsewhere. Bird fancier's disease is similar to farmer's lung. Both are forms of extrinsic allergic alveolitis, a form of localized antigen-antibody complex disease (Arthus reaction). Persistence of exposure may eventually lead to granulomatous inflammation as well."a[132] = f + "(B) Incorrect. Smoking does not typically produce an Arthus reaction, and smokers usually do not quit only when they are away from home."a[133] = f + "(C) Incorrect. Chemical irritants do not typically produce immune complex disease with extrinsic allergic alveolitis."a[134] = f + "(D) Incorrect. Cocaine is not associated with immune complex disease."a[135] = f + "(E) Incorrect. SLE does not come and go with vacation scheduling."a[136] = f + "(A) Incorrect. These opportunistic infections are more severe than would be expected for an autoimmune disease such as SLE."a[137] = f + "(B) Incorrect. Though some persons with AIDS may develop thrombocytopenia, it is typically not as severe as in persons with Wiskott-Aldrich syndrome with predominantly IgM deficiency."a[138] = f + "(C) Incorrect. Adults with a selective IgA deficiency are bothered by mild respiratory tract infections and diarrhea, not life-threatening opportunistic infections."a[139] = f + "(D) CORRECT. The opportunistic infections are characteristic for development of AIDS in a person with human immunodeficiency virus infection. Manifestations of clinical AIDS generally occur once the CD4 count drops below 200/microliter."a[140] = f + "(E) Incorrect. cANCA is a feature of vasculitides such as ANCA-associated granulomatous vasculitis."a[141] = f + "(A) Incorrect. PCP is a complication of immune deficiency diseases such as AIDS."a[142] = f + "(B) CORRECT. As in other autoimmune diseases, but particularly with her Sjogren syndrome,  malignancy is more common, and non-Hodgkin lymphoma in particular."a[143] = f + "(C) Incorrect. This complication is more typical for scleroderma."a[144] = f + "(D) Incorrect. This complication is more typical for scleroderma."a[145] = f + "(E) Incorrect. Glomerulonephritis is more a feature of SLE."a[146] = f + "(A) Incorrect. This suggests severe combined immunodeficiency (SCID)."a[147] = f + "(B) Incorrect. This pattern of infection implies a problem with cell-mediated immunity, and the hypocalcemia suggests DiGeorge syndrome with thymic hypoplasia. The thymus, parathyroids, aorta, and heart can be involved. T-cell function is deficient, resulting in recurrent and multiple fungal, viral, and protozoal infections."a[148] = f + "(C) Incorrect. Congenital HIV would be associated with decreased CD4 lymphocytes and problems with cell-mediated immunity without hypocalcemia."a[149] = f + "(D) Incorrect. Chronic granulomatous disease is manifested by recurrent bacterial infections, particularly with S. aureus, but IgG levels are normal."a[150] = f + "(E) CORRECT. He has features of X-linked agammaglobulinemia of Bruton. In this condition, B-cell maturation stops after the rearrangement of heavy-chain genes, and light chains are not produced. Thus, complete immunoglobulin molecules with both heavy and light chains are not assembled and transported to the cell membrane. The lack of immunoglobulins predisposes the child to recurrent bacterial infections. Since T-cell function remains intact, viral, fungal, and protozoal infections are not common."a[151] = f + "(A) Incorrect. Local immune complexes are a feature of type III hypersensitivity with farmer's lung."a[152] = f + "(B) Incorrect. Macrophages have a major role with type IV hypersensitivity reactions."a[153] = f + "(C) Incorrect. Myasthenia gravis is an example of an anti-receptor disease of an autoimmune nature (type II hypersensitivity). Antibody is directed against acetylcholine receptors. Many myasthenia gravis patients have a thymoma or thymic hyperplasia."a[154] = f + "(D) CORRECT. Exposure to the allergens of a bee sting is a cause for systemic anaphylaxis for some people, and this form of type I hypersensitivity can be life-threatening. The epinephrine counteracts the effects of the histamine release. An antihistaminic H1 blocker such as diphenhydramine can be useful treatment as well."a[155] = f + "(E) Incorrect. Complement activation is a feature of type III hypersensitivity reactions."a[156] = f + "(A) CORRECT. However, not every person with HLA B27 present will develop ankylosing spondylitis, one of the spondyloarthropathies."a[157] = f + "(B) Incorrect. A positive ANCA suggests an autoimmune disease with vasculitis as a prominent feature."a[158] = f + "(C) Incorrect. Her joint deformities occurred as a complication of rheumatoid arthritis. Rheumatoid nodules typically form in subcutaneous locations on extensor surfaces, though they may involve visceral organs such as lung less commonly."a[159] = f + "(D) Incorrect. Decreased serum complement is typical for autoimmune diseases such as SLE."a[160] = f + "(E) Incorrect. Amyloid deposition typically does not involve bone or joints. Myeloma can produce lytic lesions of bone."a[161] = f + "(A) CORRECT. Such persons with markedly decreased to absent IgA can live a normal life, but they can have antibodies to IgA, which set off an anaphylactic reaction when they encounter IgA in the serum in transfused blood products."a[162] = f + "(B) Incorrect. Amyloidosis is a chronic disease not marked by hypersensitivity reactions."a[163] = f + "(C) Incorrect. SLE is more likely to have a polyclonal gammopathy; there is no tendency to type 1 hypersensitivity reactions."a[164] = f + "(D) Incorrect. There is an adult form of Niemann-Pick disease, but immunologic reactions are not part of this disease."a[165] = f + "(E) Incorrect. GVHD is rarely possible from a transfusion, but the results take weeks to manifest."a[166] = f + "(A) Incorrect. Scleroderma is not complicated by risk for renal cell carcinoma."a[167] = f + "(B) Incorrect. Nodular glomerulosclerosis is most often seen as a complication of long term diabetes mellitus."a[168] = f + "(C) Incorrect. Glomerulonephritis is more typical for SLE than for other autoimmune diseases."a[169] = f + "(D) CORRECT. These findings are typical for diffuse scleroderma, and malignant hypertension may result."a[170] = f + "(E) Incorrect. Polycystic change in adults can be an autosomal dominant inherited condition, but is not an autoimmune phenomenon."a[171] = f + "(A) Incorrect. Allergic conditions and anaphylaxis are typical for type 1 hypersensitivity reactions."a[172] = f + "(B) Incorrect. Type 2 reactions are diverse and include antibody dependent cell-mediated cytotoxicity, complement mediated cell lysis, and anti-receptor diseases."a[173] = f + "(C) Incorrect. Type 3 reactions are antigen-antibody complex mediated diseases."a[174] = f + "(D) CORRECT. This tuberculin skin test relies upon an intact cell-mediated immune response. It is positive with prior exposure to mycobacterial organisms so that memory T cells are present and can be activated."a[175] = f + "(E) Incorrect. Cell-mediated immune reactions do not depend upon complement."a[176] = f + "(A) Incorrect. CD4 lymphocytes play a role in cell-mediated immune responses."a[177] = f + "(B) Incorrect. Degranulation of mast cells occurs with allergic responses, part of type 1 hypersensitivity reactions."a[178] = f + "(C) CORRECT. This pattern is typical for a type III hypersensitivity reaction. Antigen-antibody complexes tend to be trapped along basement membranes."a[179] = f + "(D) Incorrect. Macrophages mainly take part in cell-mediate immune responses and in some type II hypersensitivity reactions."a[180] = f + "(E) Incorrect. Neutrophils release proteases non-specifically and can be attracted with complement activation, but their effects are not localized."a[181] = f + "(A) Incorrect. The pancreas is not typically affected by GVHD."a[182] = f + "(B) CORRECT. There is cholestasis in the liver as a consequence of his graft versus host disease (GVHD), with donor lymphocytes attacking host tissues that don't have an exact HLA antigen match."a[183] = f + "(C) Incorrect. Complement tends to be used up with antigen-antibody reactions, not GVHD."a[184] = f + "(D) Incorrect. Neutrophilia is seen with inflammation and infection, but GVHD tends to be mediated more by apoptosis."a[185] = f + "(E) Incorrect. Muscle tends not to be affected by GVHD."a[186] = f + "(A) Incorrect. CD4 lymphocytes are helper cells that are part of rejection, but they tend to recognize class II HLA antigens."a[187] = f + "(B) Incorrect. Natural killer cells can recognize Fc receptors as well as lyse IgG coated cells as part of an antibody-dependent cell mediated cytotoxicity reaction. NK cells have receptors that recognize self class I MHC molecules."a[188] = f + "(C) CORRECT. These are cytotoxic lymphocytes that participate in acute cellular rejection of solid organ transplants. Immunosuppressive agents are generally good at combating this type of transplant rejection."a[189] = f + "(D) Incorrect. Macrophages clear up debris after cell death after other inflammatory cells have destroyed tissues."a[190] = f + "(E) Incorrect. Plasma cells make immunoglobulins and have no lytic or phagocytic properties."a[191] = f + "(A) Incorrect. CD8 cells are not infected by HIV. CD4 cells are infected by HIV and are lysed. During the period of clinical latency, there is continual turnover of CD4 cells."a[192] = f + "(B) Incorrect. High endothelial venules have endothelial cells in lymph nodes that aid in trafficking of cells because of their expression of carbohydrate ligands to which L-selectins of naive T cells can bind."a[193] = f + "(C) CORRECT. Macrophages can become infected with HIV but can survive and serve as a reservoir for spread to other cells. Follicular dendritic cells can also capture HIV and spread it to other cells."a[194] = f + "(D) Incorrect. Memory B cells play a role in humoral responses. Unfortunately, vaccines which produce antibody are not protective of HIV."a[195] = f + "(E) Incorrect. NK cells do not play a major role in HIV infection."a[195.1] = f + "(F) Incorrect. Plasma cells secrete antibodies, and such antibodies may be directed against components of HIV, which is useful for serologic testing for HIV."a[196] = f + "(A) Incorrect. Macrophages are mainly part of the process of resolution of inflammation."a[197] = f + "(B) CORRECT. The signs and symptoms are suggestive of an acute bacterial pneumonia. The decreased serum immunoglobulins at his age suggest common variable immunodeficiency, which puts him at risk for bacterial infections. Such infections promote an acute inflammatory response dominated by neutrophils, which gives the sputum the yellowish, purulent appearance."a[198] = f + "(C) Incorrect. Mast cells degranulate and release vasoactive substances as part of type 1 hypersensitivity reactions."a[199] = f + "(D) Incorrect. Lymphocytes and monocytes and plasma cells are the 'round cells' of chronic inflammatory infiltrates."a[200] = f + "(E) Incorrect. Langhans giant cells are typically seen with infectious granulomas."a[201] = f + "(A) Incorrect. This mechanism of autoimmunity is not part of rheumatic fever."a[202] = f + "(B) Incorrect. A hypergammaglobulinemia can be seen in autoimmune diseases such as SLE, suggesting that this mechanism is operative."a[203] = f + "(C) CORRECT. Sympathetic ophthalmia, with released of antigens that incite a granulomatous response, even in the opposite eye, can occur by this mechanism."a[204] = f + "(D) Incorrect. Streptococcal M proteins cross react with cardiac glycoproteins, resulting in rheumatic heart disease, a form of autoimmunity."a[205] = f + "(E) Incorrect. Derangements in T-cell regulation can lead to immunologic disorders and neoplasia, but this is not part of rheumatic fever."a[206] = f + "(A) Incorrect. Wiskott-Aldrich syndrome has atopy with eczema, thrombocytopenia, and immune defects in both cell mediated and humoral arms. The protein is involved with bridging cell surface receptors to actin filaments."a[207] = f + "(B) CORRECT. This pattern of infection implies a problem with cell-mediated immunity, and the hypocalcemia suggests DiGeorge syndrome with thymic hypoplasia. The thymus, parathyroids, aorta, and heart can be involved. T-cell function is deficient, resulting in recurrent and multiple fungal, viral, and protozoal infections."a[208] = f + "(C) Incorrect. Congenital HIV would be associated with decreased CD4 lymphocytes and problems with cell-mediated immunity without hypocalcemia."a[209] = f + "(D) Incorrect. Chronic granulomatous disease is manifested by recurrent bacterial infections, particularly with S. aureus, but IgG levels are normal."a[210] = f + "(E) Incorrect. With X-linked agammaglobulinemia of Bruton, B-cell maturation stops after the rearrangement of heavy-chain genes, and light chains are not produced. Thus, complete immunoglobulin molecules with both heavy and light chains are not assembled and transported to the cell membrane. The lack of immunoglobulins predisposes the child to recurrent bacterial infections. Since T-cell function remains intact, viral, fungal, and protozoal infections are not common."a[211] = f + "(A) Incorrect. Local immune complexes are a feature of type III hypersensitivity with farmer's lung."a[212] = f + "(B) Incorrect. Macrophages have a major role with type IV hypersensitivity reactions."a[213] = f + "(C) CORRECT. Myasthenia gravis is an example of an anti-receptor disease of an autoimmune nature (type II hypersensitivity). Antibody is directed against acetylcholine receptors. Many myasthenia gravis patients have either a thymoma or thymic hyperplasia."a[214] = f + "(D) Incorrect. Exposure to the allergens of a bee sting is a cause for systemic anaphylaxis for some people. Antigen linking to IgE coating mast cells causes degranulation with release of vasoactive substances such as histamine to cause anaphylaxis."a[215] = f + "(E) Incorrect. Cross-reactivity is a feature of some infections such as those with group A strep producing either a glomerulonephritis or carditis."a[216] = f + "(A) Incorrect. Not every person with HLA B27 present will develop ankylosing spondylitis, one of the spondyloarthropathies."a[217] = f + "(B) Incorrect. A positive ANA suggests an autoimmune disease such as SLE or scleroderma."a[218] = f + "(C) CORRECT. Her joint deformities occurred as a complication of rheumatoid arthritis. Rheumatoid nodules typically form in subcutaneous locations on extensor surfaces, though they may involve visceral organs such as lung less commonly."a[219] = f + "(D) Incorrect. Decreased serum complement is typical for autoimmune diseases such as SLE."a[220] = f + "(E) Incorrect. Most patients with rheumatoid arthritis have hypergammaglobulinemia. Some patients with common variable immunodeficiency with hypogammaglobulinemia develop rheumatoid arthritis, but they have a problem with infections."
