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RAD 266 Pathology Correlation for CT/MRI Autoimmune Disease Module B Topics Included in Module B Immune system responses and disorders Congenital and acquired Immunodeficiency Acquired Immunodeficiency Treatment of Immunodeficiency Immunodeficiency complications Autoimmune Disorder Types of Autoimmune disorders Types of effects caused from autoimmune disorder Treatment of autoimmune disorder Acquired immunodeficiency syndrome Systemic lupus erythematosus Student Objectives 1. Name the common pathological conditions affecting the autoimmune system. 2. For each common pathological condition identified: a.describe the disorder b.list the etiology c.name the associated symptoms d.name the common means of diagnosis e.list characteristic CT and MR manifestations of the pathology Continue… Student Objectives 3. Identify common pathology on CT and MRI images. 4. Identify pathology common only in pediatric patients. 5. Describe the MR tissue characteristics of pathologic processes. Continue… Student Objectives 6. Identify how field strength affects the ability to visualize select pathology. 7. Recognize various differences in pathologic conditions between children and adults. Student Learning activities 1. Read Chapter One, pages 1 – 36. Pathophysiology, Gutierrez and Peterson 2. Name the immune system cell types, page 2, Pathophysiology, Gutierrez and Peterson 3. Review and answer all “Do You UNDERSTAND” questions in Chapter one, Pathophysiology, Gutierrez and Peterson pages 5, 8, 11, 13, 19, 20, 22, 25, 29, 30, 33, 35 and 36. Continue….. Student Learning activities 4. Read the following pages, CT and MRI Pathology A Pocket Atlas, Gray and Ailinani – – – – – – – Pages Pages Pages Pages Pages Pages Pages 42, 43 Brain Abscess 46, 47 Multiple Sclerosis (Brain) 72, 73 Multiple Sclerosis (Spinal cord) 86, 87 Vertebral Osteomyelitis 116, 117 Sinusitis 152, 153 Hepatoma 202, 203 Splenomegaly Continue….. Student Learning activities 5.Review Pathology Terms and Appendix A Immune System The human body has three main types of defenses against injury and disease. These defenses include the following: normal functioning mucosal membranes normal immune responses intact skin -(Pathophysiology, Gutierrez, Peterson) The two types of immune responses are nonspecific and specific. Nonspecific immune responses Nonspecific immune response – the response of the innate immunity system or what we are born with. Specific immune response Specific immune response – the response based on our ability to develop antibodies against foreign substances. Because the immune system is nonspecific and specific, the body must be able to distinguish between foreign substances and its own tissues. Immune System Disorders Immunodeficiency Disorder and Autoimmune Disorder “Immune system disorders occur when the immune system responds in an inappropriate way (excessive or lacking)” -Medline Plus,2005 Lymphoid Tissue thymus lymph nodes tonsils parts of the spleen and gastrointestinal tract bone marrow Lymphocytes There are two groups of lymphocytes called: T-lymphocytes and B-lymphocytes cellular immunity - T lymphocytes are sensitized lymphocytes that directly attack antigens humoral immunity - B lymphocytes produce antibodies that attach to the antigen and make phagocytes and complement proteins much more efficient in the destruction of the antigen -Medline Plus,2005 Human immune system There are two broad categories for human immune system disorders: immunodeficiency disorders (lacking) autoimmune disorders (excessive) Immunodeficiency disorders can be congenital or acquired can affect any part of the immune system -UMM, 2005 Congenital Immunodeficiency Disorders Most commonly, this involves decreased functioning of T or B lymphocytes (or both), or deficient antibody production Congenital Immunodeficiency Disorders The causes include congenital (inherited) defects and acquired immunodeficiency caused by a disease that affects the immune system” -UMM, 2005 Congenital Immunodeficiency Disorders Congenital immunodeficiency - disorders of antibody production Congenital disorders affecting the T lymphocytes Inherited combined immunodeficiency affects both T and B lymphocytes Acquired Immunodeficiency -is a result or a complication of an external causative source. common side-effect of chemotherapy complication of diseases such as HIV and AIDS malnutrition, particularly with lack of protein many cancers people who have had a splenectomy people with diabetes increased age -(UMM, 2005) Immunodeficiency complications (symptoms, signs): persistent or recurrent infections -(Merck, 2003) severe infections, by organisms, that are normally mild -(Merck, 2003) incomplete recovery from illness, poor response to treatment -(Adam Encyclopedia, 2004) increased incidence of cancer or other tumor growth -(UMM, 2005) enlarged liver and spleen -(Merck, 2003) Treatment of Immunodeficiency Drug induced immunodeficiency – “…..maintain a balance between suppression of parts of the immune system and the ability to fight disease and infection -(Medline Plus, 2005)”. protection against infection and other disease processes -(UMM, 2005) drugs that increase the efficiency of the immune system -(UMM, 2005) Continue….. Treatment of Immunodeficiency drugs to reduce the amount of virus in their immune system -(UMM, 2005) transplantation of thymus tissue stem cell transplantation -(Merck, 2003) -(Merck,2003) prophylactic antibiotic before surgeries or dental procedures -(Merck,2003) Prognosis of Immunodeficiency is variable based on the type of immunodeficiency disorder. Autoimmune Disorder Autoimmune Disorder is an overactive immune response where the body attacks its own cells. Continue …… Autoimmune Disease Although the cause of autoimmune disease is not known, most autoimmune diseases are most likely the result of multiple circumstances. There are more than 40 human diseases classified as definite or probable autoimmune diseases. Examples of autoimmune (or autoimmune-related) disorders include -(MedlinePlus, 2006): Hashimoto’s thyroiditis pernicious anemia Addison’s disease type 1 diabetes rheumatoid arthritis systemic lupus erythematosus dermatomyositis Sjogren’s syndrome lupus erythematosus multiple sclerosis myasthenia gravis Reiter’s syndrome Grave’s disease Autoimmune diseases affect women more often than men. Almost 79% of autoimmune disease patients in the United States are women -(Wikipedia, 2006). Symptoms for Autoimmune disorders fatigue vertigo malaise – (nonspecific feeling of not being well) fever or low grade temperature Tests for Autoimmune Because symptoms are different according to the specific disorder, and the organ or tissue affected, there are a variety of testing methods. Treatment of autoimmune disorder (Medline Plus, 2005): treat symptoms according to the type and severity control the autoimmune process while maintaining the ability to fight disease hormones or other substances normally produced by the affected organ may need to be supplemented assist mobility or other functions may be needed for disorders that affect the bones, joints or muscles. Acquired Immunodeficiency syndrome (AIDS) Acquired Immunodeficiency syndrome (AIDS) AIDS (acquired immunodeficiency syndrome) is the final stage of HIV disease. The Center for Disease Control and Prevention (CDC) has classified AIDS as “beginning when a person with HIV infection has a CD4 cell count below 200”. -(Medline Plus, 2006) A patient is also considered to have AIDS when numerous opportunistic infections and cancers appear to the person with the HIV infection. AIDS “Although AIDS was not recognized as a new clinical syndrome until 1981, researchers examining the earlier medical literature, identified cases appearing to fit the AIDS surveillance definition as early as the 1950s and1960s” -(HIVInSite, 2003) HIV infection is transmitted by: sexual intercourse (heterosexual and homosexual) by blood and blood products perinatally from infected mother to child (prepartum, intrapartum, and postpartum via breast milk) ……..(McGraw-Hill, 2005) sharing needles with an infected person The associated symptoms for AIDS: Symptoms are primarily the result of infections that do not normally develop in people with healthy immune systems. These infections are called opportunistic infections. The associated symptoms for AIDS: “cough and shortness of breath” “seizures and lack of coordination” “difficult or painful swallowing” “mental symptoms such as confusion and forgetfulness” “severe and persistent diarrhea” -(Medline Plus, 2006) Continue….. The associated symptoms for AIDS: “fever” “vision loss” “nausea, abdominal cramps, and vomiting” “weight loss and extreme fatigue” “severe headaches with neck stiffness” “coma” -(Medline Plus, 2006) The common means for diagnosis of AIDS –ELISA – (enzyme-linked immunoassay) This is the screening test. –Western blot – This is the confirmatory test. continue….. AIDS Newly infected people may have symptoms which include: fever headache enlarged lymph glands in the neck Malaise These symptoms can disappear on their own, within a few weeks. continue….. AIDS “Science has determined when each progressive opportunistic infections and cancers appear, based on the CD4 count”. -(Medline Plus, 2006) “CD4 count below 350/ml (Medline Plus, 2006)”: – “herpes simplex virus occurs more frequently than ever before – tuberculosis – oral or vaginal thrush – herpes zoster – Non-Hodgkins lymphoma” -(Medline Plus, 2006) “CD4 count below 200/ml (Medline Plus, 2006)” – “Pneumocystis carinii pneumonia, PCP pneumonia – candida esophagitis- yeast infection of the esophagus -(Medline Plus, 2006)” “CD4 count below 100/ml (Medline Plus, 2006)” – – – – – “Cryptococcal meningitis- infection of the brain by this fungus AIDS dementia toxoplasmosis encephalitis – infection of the brain by this parasite, which is frequently found in cat feces progressive multifocal leukoencephalopathy – viral disease of the brain wasting syndrome – extreme weight loss and anorexia caused by HIV” -(Medline Plus, 2006) “CD4 count below 50/ml (Medline Plus, 2006)” – “mycobacterium avium – blood infection by a bacterium related to tuberculosis – cytomegalovirus infection – a viral infection that can affect almost any organ system, especially the eyes” -(Medline Plus, 2006) Complications as a result of Trauma-AIDS Pathology resulting for trauma can undergo any number of pathologic processes. Infection is always a concern. When treating a patient with HIV/AIDS an attempt to “…reduce co-factors that may contribute to immune compromise” -(Pathophysiology, Gutierrez, Peterson) Co-factors that may contribute to immune compromise “protein-calorie malnutrition… pregnancy…. stress…. alcohol… intravenous and recreational drug use…..” -(Pathophysiology, Gutierrez, Peterson)” Characteristic CT and MRI manifestations - AIDS CT and MRI manifestations of the AIDS pathology depend on the system affected by the syndrome. – Multiple systems can be affected. – “The role of imaging methods in the diagnosis of diseases associated with HIV infection depends on such factors as cost, availability, and expertise of the clinician or radiologist interpreting the image.” -(HIVInsite, Goodman, 2006) – Abscesses can be located in any area. Gastrointestinal “CT scanning may provide information about intraluminal as well as solid organ or lymph node disease. -(HIVInsite, Goodman2006)” The most common CT findings are: – enlarged retroperitoneal and mesenteric lymph nodes (42%) – hepatomegaly (50%) – splenomegaly (46%) – small bowel wall thickening (14%) -(HIVInsite, Goodman, 2006) Copyright General Electric Company 1997-2006 | GE Healthcare Copyright General Electric Company 1997-2006 | GE Healthcare Copyright General Electric Company 1997-2006 | GE Healthcare Copyright General Electric Company 1997-2006 | GE Healthcare Copyright General Electric Company 1997-2006 | GE Healthcare Respiratory “Pneumocystis carinii is a common organism found both in soil and in living spaces that does not cause illness in people with a normal immune system, this organism multiplies rapidly and causes pneumonia”. -(Pathophysiology, Gutierrez, Peterson) Radiology, Feb 2000; 214: 427 - 432. Radiology, Feb 2000; 214: 427 - 432. Radiology, Feb 2000; 214: 427 - 432. Radiology, Aug 2002; 224: 493 - 502. Radiology, Aug 2002; 224: 493 - 502. Radiology, Aug 2002; 224: 493 - 502. Central Nervous System “In patients with advanced HIV disease, neurologic signs and symptoms are caused by a variety of central nervous system (CNS) infections, tumors, and direct effects of HIV on neural tissue” -(HIVInsite, Goodman, 2006) RadioGraphics, Jul 2004; 24: 1029 1049 Central Nervous System Continue…… “MRI appears more sensitive than CT scanning in the detection of HIV related CNS pathology. Some researchers believe that MRI is the method of choice for all HIV related CNS pathology, and obviates CT scanning in most cases”. Goodman, 2006 -HIVInsite, Primary CNS lymphoma is an entity that was rarely encountered previously, and it accounted for only 1%–3% of CNS neoplasms prior to 1978. However, incidence has increased drastically since the onset of the acquired immunodeficiency syndrome, or AIDS, epidemic, and CNS lymphomas now represent up to 15% of all brain tumors at some institutions. Radiology, Jul 2002; 224: 177 - 183. Radiology, Jul 2002; 224: 177 - 183. ogy, Jul 2002; 224: 177 - 183. Radiology, Oct 2005; 237: 265 - 273. Radiology, Oct 2005; 237: 265 - 273. Hepatobiliary System “Infections of the liver and biliary system with any of a number of organisms may result in solitary or multiple hepatic abscesses or different forms of biliary involvement, including papillary stenosis and sclerosing cholangitis”. -(HIVInsite, Goodman, 2006)”. Liver disease may be diagnosed with: CT Ultrasound Biliary disease may be diagnosed with: CT, Ultrasound or Endoscopic Retrograde Cholangiopancreatography (ERCP) -(HIVInsite, Goodman, 2006) Peliosis hepatis is the term used for liver infection by B henselae. This condition occurs almost exclusively in patients with acquired immunodeficiency syndrome (AIDS). The mechanism of transmission is unknown, but involvement by animal or insect vectors has been postulated. -RadioGraphics, Jul 2004; 24: 937 - 955. RadioGraphics, Jul 2004; 24: 937 - 955. RadioGraphics, Jul 2004; 24: 937 - 955. RadioGraphics, Jul 2004; 24: 937 - 955. Radiographic Procedures and Interventional Techniques CT and HDCT (High Definition Computer Tomography) are often beneficial when diagnosing opportunistic diseases, caused by AIDS. Follow up may sometimes be done with routine chest x-ray or CT. “In some cases, needle aspiration or excisional biopsy may be necessary to rule out lymphoma, KS, mycobacterial disease, or other infection” -HIVInsite, Goodman, 2006 Systemic Lupus Erythematosus (SLE) Systemic Lupus Erythematosus (SLE) Systemic Lupus Erythematosus (SLE) is classified with the group of diseases involving connective tissues; formerly termed collagen vascular disease. -McGraw-Hill, 2000 Systemic Lupus Erythematosus (SLE) Connective tissue diseases have many overlapping symptoms which makes identifying the specific disease difficult. -McGraw-Hill, 2000 Connective tissue diseases include: Lupus erythematosus systemic vasculitis Scleroderma Systemic sclerosis Polymyositis Sjogren’s syndrome -McGraw-Hill, 2000 Each of the preceding diseases can involve multiple organ systems and is often coupled with various immunologic abnormalities. -(McGraw-Hill, 2000). Cause of Systemic Lupus Erythematosus – 1. In most cases of SLE, the individual has antibodies present in the blood serum which are not normally present. -McGraw-Hill, 2000 2. A genetic predisposition to the development of SLE exists. -eMedicine, 2006 CONTINUE..... 3. Except for drug-related lupus, the cause of systemic lupus and its variants is not known; thus, a rational basis for cure does not exist. -McGraw-Hill, 2000 4. If a mother has SLE, her daughter’s risk of developing the disease is 1:40, and her son’s risk is 1:250 -eMedicine, 2006 continue…. 5. “The antibodies may be produced that can react against the body’s: – blood cells – organs – and tissues -MedlinePlus, 2004 continue…. 6. “The number and variety of antibodies that can appear in lupus are greater than those in any other disorder” -Merck, 2003 7. Many researchers suspect it occurs following infection with an organism that looks similar to particular proteins in the body, which are later mistaken for the organism and wrongly targeted for attack”. -MedlinePlus, 2004 CONTINUE…… 8. Drug induced Lupus can occur from medications used to treat heart disease and tuberculosis. 9. Drug induced lupus usually disappears after the drug is discontinued. -McGraw-Hill, 2000 Associated signs and symptoms 1.”The symptoms for SLE vary from person to person.” -Merck, 2003 2. 90% of the people with Lupus are young women in their late teens to 30’s. 3. Lupus occurs in all parts of the world but may be more common in African Americans and in Asians. 4. “The manifestations and course of systemic lupus vary greatly from one individual to another and even in one person over time.” -(McGraw-Hill, 2000)”. 5. SLE can involve: – joints (arthralgias-intermittient joint pain or acute polyarthritis) – kidneys – mucous membranes (gastrointestinal system) – blood vessel walls (including those of the musculoskeletal system) – skin – nervous system – heart – lungs Continue…... Associated Signs and Symptoms 6. Symptoms may occur gradually or they may begin suddenly with fever, resembling an acute infection. -Merck, 2003 7. Additional symptoms which may be associated with this disease: – – – – – – – blood in the urine nose bleed coughing up blood swallowing difficulty patchy skin color red spots on skin fingers that change color upon pressure or in the cold – numbness and tingling – mouth sores hair loss -MedlinePlus, 2004 CONTINUE…. Continue…... Associated Signs and Symptoms 8. abdominal pain 9. visual disturbances -MedlinePlus, 2004 -MedlinePlus, 2004 10. “Widespread enlargement of the lymph nodes is common, particularly in children, young adults, and in African Americans of all ages”. -Merck, 2003 11. “For many women, lupus flare ups occur during the second half of the menstrual cycle, with symptoms disappearing when menstruation starts”. 12.“Migraine-type headaches, epilepsy, or severe mental disorder (psychoses) may be the first abnormalities that are noticed”. 13. “Skin rashes include a butterfly-like redness across the nose and cheeks (malar butterfly erythema), raised bumps or patches of thin skin, and red, flat or raised areas on the face and sun exposed areas of the neck, upper chest, and elbows”. Merck, 2003 14. Ulcers occur on mucus membranes, such as the gums, inside the nose, roof of the mouth, inside the cheeks. Merck, 2003 15. Alopecia 16. pain when breathing deeply (recurring inflammation of the pleura) 17. chest pain due to pericarditis (more serious but rare effects on the heart include angina due to coronary artery vasculitis, fibrosing myocarditis) 18. sensitivity to sunlight (photosensitivity) Common Means of Diagnosis SLE is can not be diagnosed by doing any one laboratory test. Several types of laboratory testing can be done to help confirm a diagnosis of Systemic Lupus Erythematosus. “The diagnosis of SLE is based upon the presence of at least four out of eleven typical characteristics of the disease” “antinuclear antibody (ANA) panel” “rheumatoid factor” “urine protein” “ESR” “chest x-ray showing pleuritis or pericarditis” “CBC” “WBC count” “serum globulin electrophoresis” “serum protein electrophoresis” “cryoglobulins” “Coombs’ test” MedlinePlus, 2004) “The diagnosis of SLE is based upon the presence of at least four out of eleven typical characteristics of the disease” Continue: “complement component 3 (C3)” “characteristic skin rash or lesions” “complement” “antithyroid microsomal antibody” “chest sounds which reveal heart friction rub or pleural friction rub” “antithyroglobulin antibody” “antimitochondrial antibody” “anti-smooth muscle antibody” “neurological examination” “kidney biopsy” -MedlinePlus, 2004 Complications of SLE: Complications associated with SLE depend on the affected systems and on the severity of the disease. “Surgical procedures and pregnancy are more complicated for people who have lupus, and they require close medical supervision”. “If inflammation of the blood vessels at the back of the eye occurs, doctors give prompt treatment with an immunosuppressive drug because of the high risk of blindness”. Merck, 2003 Complications of SLE Complications associated with SLE depend on the affected systems and on the severity of the disease. Surgical procedures and pregnancy are more complicated Retinitis-promt treatment necessary to prevent blindness Blockage of arteries in the brain or lung due to thrombosis Complications of SLE continue… Kidney involvement Myocarditis Lupus pneumonitis Thrombocytopenia Ischemia Complications of high dose glucocorticoid therapy Complications of cytotoxic agents -(Merck, 2003) Characteristic Imaging Manifestations Imaging methods are chosen according to the area of investigation SLE presents with various radiologic manifestations: – – – – – – Respiratory Cardiovascular Gastrointestinal Genitourinary Musculoskeletal Neurological -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Characteristic Imaging Manifestations continue…….. there are no universally accepted imaging criteria for the diagnosis of SLE not all SLE patients need imaging because many will present with systemic findings may help direct management when there are complications related to therapy -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Characteristic Imaging Manifestations continue…….. In a patient with a known diagnosis of SLE, imaging is often performed to determine: 1. the extent and severity of disease and to, 2. monitor the myriad complications that arise from the disease and, 3. it’s therapy. 4. …imaging may be important for prompt diagnosis of and initiation of therapy for complications of SLE -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Gastrointestinal The most common CT finding in patients with SLE and acute abdominal pain is ischemic bowel disease Pancreatitis in SLE may be due to vasculitis, ischemia of small pancreatic vessels, immune complex deposition, or a combination of these entities. Gastrointestinal continue….. CT images of patients with SLE, are typically evaluated for the following radiological signs: ischemia, bowel wall changes mesenteric changes fluid collection retroperitoneal lymphadenopathy peritoneal enhancement hepatomengaly changes in other abdominal organs pancreatitis Gastrointestinal continue……. “SLE may involve any portion of the gastrointestinal system. Nonspecific and vague abdominal pain is seen in 10%–37% of patients with SLE and may be due to vasculitis, obliterative vascular thrombosis resulting in ischemic bowel, or immune complex deposition of autoantibodies in tissues, eliciting an inflammatory response. Immunosuppressants such as azathioprine and prednisone can also induce abdominal symptoms and have been implicated in pancreatitis.” -RadioGraphics 2004; 24:1069-1086 © RSNA, 2004. -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Copyright © 2005, Radiological Society of North America, Inc. -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Respiratory “The risk of pulmonary infection is three times higher in patients with SLE than in the general population due to intrinsic immunologic abnormalities…..” “Recurrent pulmonary infections can lead to bronchiectasis and respiratory compromise.” “Imaging is often performed in patients with a known diagnosis of SLE to determine the extent and severity of disease, which depend on the extent of organ involvement, and to monitor complications. -RadioGraphics 2004;24:1069-1086© RSNA, 2004 Respiratory continue……. “Pleural effusions are the most common manifestation of SLE in the respiratory system and are bilateral in approximately 50% of patients.” -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 “Alveolar hemorrhage is a potentially catastrophic complication of systemic lupus erythematosus.” -(American College of Chest Physicians, Chest journal, 2006) -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 Central Nervous System Neurologic changes can occur secondary to to lupus. 15% of patients experience CVA’s, (cerebral vascular accident) and 20% of all patients develop some type of seizure disorder. These CNS manifestations can limit the patient’s ability to care for themselves. Lupus therefore has a significant effect on the quality of life for those with this disease. Central Nervous System continue…… Brain imaging is most often conclusive when using MRI in comparison to CT. “All lesions seen on CT were also visible on MR; however, MR proved to be substantially more sensitive. Many lesions not visible on CT were noted on MR, and in general, MR demonstrated the abnormalities with greater clarity”. -(American College of Physicians, Chest journal, 1985) Central Nervous System continue…… “Focal lesions were demonstrated on MR imaging in eight patients with systemic lupus erythematosus and recent onset of neuropsychiatric symptoms. Corresponding findings were visible in only two of seven patients who had CT scans”. -(American College of Physicians, Chest journal, 2006) Immunosuppressive therapy for the treatment of SLE can reduce the immune response leaving the patient vulnerable for cerebral abscesses. -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004 -RadioGraphics 2004;24:1069-1086 © RSNA, 2004