Screening for the immunological diseases

Immunodeficiency disorders usually result from use of a medication or from a long-lasting serious disorder (such as cancer) but occasionally are inherited.
People usually have frequent, unusual, or unusually severe or prolonged infections and may develop an autoimmune disorder or cancer.
Doctors suspect immunodeficiency based on symptoms and do blood tests to identify the particular disorder.
People may be given antimicrobial drugs (such as antibiotics) to prevent and treat infections.
Immune globulin may be given if there are too few antibodies (immunoglobulins) or they are not functioning normally.
For some severe immunodeficiency disorders, stem cell transplantation is sometimes done.
Immunodeficiency disorders impair the immune system’s ability to defend the body against foreign or abnormal cells that invade or attack it (such as bacteria, viruses, fungi, and cancer cells). As a result, unusual bacterial, viral, or fungal infections or lymphomas or other cancers may develop.

Another problem is that up to 25% of people who have an immunodeficiency disorder also have an autoimmune disorder (such as immune thrombocytopenia). In an autoimmune disorder, the immune system attacks the body's own tissues. Sometimes the autoimmune disorder develops before the immunodeficiency causes any symptoms.

There are two types of immunodeficiency disorders:

Primary: These disorders are usually present at birth and are genetic disorders that are usually hereditary. They typically become evident during infancy or childhood. However, some primary immunodeficiency disorders (such as common variable immunodeficiency) are not recognized until adulthood. There are more than 100 primary immunodeficiency disorders. All are relatively rare.
Secondary: These disorders generally develop later in life and often result from use of certain medications or from another disorder, such as diabetes or human immunodeficiency virus (HIV) infection. They are more common than primary immunodeficiency disorders.

1. DIFFERENTIAL DIAGNOSIS By Catherine Cavallaro Goodman

2. OUTLINES Screening for Immunologic Disease

3. INTRODUCTION Immunity denotes protection against infectious organisms. The immune system is a complex network of specialized organs and cells that has evolved to defend the body against attacks by "foreign" invaders.

4. Immunity is provided by lymphoid cells residing in the immune system. This system consists of central and peripheral lymphoid organs

5. TYPES OF IMMUNITY Immunologic responses in humans can be divided into two broad categories: Humoral immunity , which takes place in the body fluids (extracellular) and is concerned with antibody and complement activities, Cell-mediated or cellular immunity , primarily intracellular, which involves a variety of activities designed to destroy or at least contain cells that are recognized by the body as being alien and harmful

6. USING THE SCREENING MODEL How long have you had this problem? (acute vs. chronic) Has the problem gone away and then recurred? • Have additional symptoms developed or have other areas become symptomatic over time?

7. 1. Past Medical History Persons with fibromyalgia or chronic pain often have a family history of alcoholism. depression, migraine headaches, gastrointestinal (GI) disorders, or panic attacks.

8. Clients with systemic inflammatory disorders may have a family history of an identical or related disorder such as rheumatoid arthritis (RA), SLE, autoimmune thyroid disease, multiple sclerosis, or myasthenia gravis.

9. A recent history of surgery may be indicative of bacterial or reactive arthritis, which requires immediate medical evaluation.

10. 2. Risk Factor Assessment Unknown PMH with positive family history is the only red flag sign

11. 3. Clinical Presentation soft tissue and/or joint pain, stiffness, swelling, weakness, constitutional symptoms, Raynaud's phenomenon, and sleep disturbances. prolonged stiffness Specific arthropathies have a predilection for involving specific joint areas.

12. especially single-joint swelling, it is necessary to distinguish whether this swelling is articular (as in arthritis), is periarticular (as in tenosynovitis ), involves an entire limb (as with lymphedema ), or occurs in another area (such as with lipoma or palpable tumors)

13. Generalized weakness is a common symptom of individuals with immune system disorders in the absence of muscle disease. If the weakness involves one limb without evidence of weakness elsewhere, a neurologic disorder may be present.

14. Nail bed changes are especially indicative of underlying inflammatory disease. For example, small infarctions or splinter hemorrhages

15. 4. Associated Signs and Symptoms 5. Review of systems

16. IMMUNE SYSTEM PATHOPHYSIOLOGY Immunodeficiency disorder Hypersensitivity disorder Autoimmune disorder Immunoproliferative disorder

17. Immunodeficiency Disorders AIDS What it is CAUSES WHO GETS IT? DIAGNOSIS PROGNOSIS

18. • What it is: AIDS, acquired immunodeficiency syndrome, is a contagious disease that destroys the T cells, a key component of the body's immune system. • What causes it: AIDS is caused by the human immunodeficiency virus (HIV), spread through sexual contact, needles , or syringes shared by injection drug users (IDUs); transfusion of infected blood or blood products; or perinatal transmission (from infected birthing or breastfeeding mother to her infant). •

19. Who gets it: Primary persons infected with HIV have been homosexual men MSMs who have sex with women) and injection drug users IDUs. The Centers for Disease Control and Prevention (CDC) estimate that heterosexual contact is responsible for 3% of male cases and 34% of female cases.

20. Diagnosis: Screening for AIDs is conducted by testing a fingerstick sample of blood for the presence of antibodies to HIV-1. The test indicates only if a person has been exposed to the virus. A new "quick" test called OraQuick Rapid HIV Antibody Test is almost 100% accurate, and results are available within 20 minutes. A positive test requires additional confirmation testing. •

21. Prognosis: At present, there is no cure, but many people in the U.S. remain healthy and active with combination antiretroviral medications designed to attack HIV in various stages of its life cycle; when death occurs, it is usually as a result of " opportunistic" infections or cancers that the immunosuppressed body cannot resist . IDUs are four times more likely to die of AIDS than individuals infected through sexual contact .

22. E a r l y S y m p t o m a t i c H I V I n f e c t i o n Fever Night sweats Chronic diarrhea Fatigue Minor oral infections Headache Cough Shortness of breath Cutaneous changes

23. A d v a n c e d S y m p t o m a t i c HIV I n f e c t i o n Kaposi's sarcoma ( Multiple purple blotches and bumps on skin Continued ) Hypertension (pulmonary and/or cardiac) Dyspnea , syncope, fatigue, chest pain, nonproductive cough Opportunistic diseases (e.g., tuberculosis, Pneumocystis carinii , pneumonia, lymphoma, thrush; herpes I and II; toxoplasmosis; candidiasis) Persistent dry cough • Fever , night sweats

24. Easy bruising Thrush (thick, white coating on the tongue or throat accompanied by a sore throat) • Muscle atrophy and weakness Back pain Side effects of medication (see text) Poor wound healing HIV-related dementia (memory loss, confusion, behavioral change, impaired gait) Distal symmetric polyneuropathy (pain, numbness, tingling, burning, weakness, atrophy)

25. HYPERSENSITIVE DIORDERS When the immune system becomes overactive, or hyperactive, a state of hypersensitivity exists, leading to immunologic diseases such as allergies.

26. TYPE I TYPE II TYPE III TYPE IV

27. Type I Anaphylactic Hypersensitivity ("Allergies") ALLERGY AND ATOPY Allergy refers to the abnormal hypersensitivity that takes place when a foreign substance (allergen) is introduced into the body of a person likely to have allergies. The body fights these invaders by producing the special antibody immunoglobulin E ( IgE ). This antibody (now a vital diagnostic sign of many allergies), when released into the blood, breaks down mast cells, which contain chemical mediators, such as histamine, that cause dilation of blood vessels and the characteristic symptoms of allergy.

28. Atopy differs from allergy because it refers to a genetic predisposition to produce large quantities of IgE , causing this state of clinical hypersensitivity . The reaction between the allergen and the susceptible person (i.e., allergy-prone host) results in the development of a number of typical signs and symptoms usually involving the GI tract, respiratory tract, or skin.

29. Clinical signs and symptoms vary from one client to another according to the allergies present. With the Family/Personal History form used The therapist can then be alert to any of these warning signs during treatment and can take necessary measures, whether that means grading exercise to the client's tolerance, controlling the room temperature, or appropriately using medications prescribed.

30. Type II Hypersensitivity ( Cytolytic or Cytotoxic) A type II hypersensitivity reaction is caused by the production of autoantibodies against self cells or tissues that have some form of foreign protein attached to them. The autoantibody binds to the altered self cell, and the complex is destroyed by the immune system. Typical examples of this type of hypersensitivity are hemolytic anemias , idiopathic thrombocytopenia purpura (ITP ), hemolytic disease of the newborn, and transfusion of incompatible blood. Blood group incompatibility causes cell lysis , which results in a hemolytic transfusion reaction.

31. Clinical Signs and Symptoms of Type II Hypersensitivity Headache • Back (flank) pain • Chest pain similar to angina • Nausea and vomiting • Tachycardia and hypotension • Hematuria • Urticaria (skin reaction

32. Type III Hypersensitivity (Immune Complex) Immune complex disease results from formation or deposition of antigen-antibody complexes in tissues . For example, the antigen-antibody complexes may form in the joint space, with resultant synovitis , as in RA . Antigen-antibody complexes are formed in the bloodstream and become trapped in capillaries or are deposited in vessel walls, affecting the skin ( urticaria ), the kidneys (nephritis), the pleura ( pleuritis ), and the pericardium (pericarditis).

33. Serum sickness is another type III hypersensitivity response that develops 6 to 14 days after injection with foreign serum (e.g., penicillin, sulfonamides, streptomycin, thiouracils , hydantoin compounds). Deposition of complexes on vessel walls causes complement activation with resultant edema, fever, inflammation of blood vessels and joints, and urticaria .

34. Clinical Signs and Symptoms of Type III Hypersensitivity Fever Arthralgias ; synovitis Lymphadenopathy Urticaria Visceral inflammation (nephritis, pleuritis , pericarditis

35. Type IV Hypersensitivity (Cell-Mediated or Delayed) In cell-mediated hypersensitivity, a reaction occurs 24 to 72 hours after exposure to an allergen . For example, type IV reactions occur after the intradermal injections of TB antigen. Graft-versus-host disease ( GVHD) and transplant rejection are also type IV reactions. In GVHD, immunocompetent donor bone marrow cells (the graft) react against various antigens in the bone marrow recipient (the host), which results in a variety of clinical manifestations, including skin, GI, and hepatic lesions. Contact dermatitis is another type IV reaction that occurs after sensitization to an allergen, commonly a cosmetic, adhesive, topical medication, drug additive (e.g., lanolin added to lotions, ultrasound gels, or other preparations used in massage or soft tissue mobilization), or plant toxin (e.g., poison ivy).

36. Clinical Signs and Symptoms of Type IV Hypersensitivity • Itching • Erythema • Vesicular skin lesions • Graft-versus-host disease (GVHD

37. Autoimmune Disorders Organ specific autoimmune diseases include thyroiditis , Addison's disease, Graves' disease, chronic active hepatitis, pernicious anemia, ulcerative colitis, and insulin-dependent diabetes

38. Generalized autoimmune diseases involve reactions in various body organs and tissues (e.g., fibromyalgia, rheumatoid arthritis, systemic lupus erythematosus , and scleroderma).

39. FIBROMYALGIA SYNDROME A non inflammatory condition appearing with generalized musculoskeletal pain in conjunction with tenderness to touch in a large number of specific areas of the body and a wide array of associated symptoms.

40. Anatomic location of tender points Low cervical: Bilateral, at C5-C7 Second rib: Bilateral, at second costochondral junctions Lateral epicondyle: Bilateral, 2 cm distal to epicondyles Knees: Bilateral, at medial fat pad proximal to joint line Occiput: Bilateral, at suboccipital muscle insertions Trapezius: Bilateral, at midpoint of upper border Supraspinatus : Bilateral, above scapular spine near medial border Gluteal: Bilateral, upper outer quadrants of buttocks in anterior fold of muscle Greater trochanter: Bilateral, posterior to trochanteric prominence

41. Differentiating Myofascial Pain Syndrome from Fibromyalgia Syndrome MPS FMS Trigger points Localized musculoskeletal condition No associated signs and symptoms Etiology: overuse, repetitive motions, reduced muscle activity Tender points Systemic condition Wide array of associated signs and symptoms Etiology: neurohormonal imbalance; autonomic nervous system dysfunction

42. CLINICAL SIGNS AND SYMPTOMS aches and pains stiffness, Swelling in soft tissue tender points, muscle spasms or nodules

43. Myalgia (generalized aching) Fatigue (mental and physical) Sleep disturbances, nocturnal myoclonus , nocturnal bruxism Tender points of palpation Chest wall pain mimicking angina pectoris Tendinitis, bursitis Temperature dysregulation

44. Dyspnea , dizziness, syncope Headache (throbbing occipital pain) Morning stiffness (more than 15 minutes) Paresthesia (numbness and tingling Mechanical low back pain with sciatica-like radiation of pain Subjective swelling Irritable bowel symptoms

45. Urinary urgency; irritable bladder syndrome Dry eyes/mouth Depression/anxiety Cognitive difficulties (e.g., short-term memory loss, decreased attention span) Premenstrual syndrome (PMS) Weight gain from physical inactivity because ofpain and fatigue

46. DD OF FMS Hypothyroidism Adult growth hormone deficiency (e.g., pituitary tumors, head trauma, AIDS) Polymyalgia rheumatica /giant cell arteritis Rheumatoid arthritis, seronegative Polymyositis / dermatomyositis Systemic lupus erythematosus Myofascial pain syndrome Metabolic myopathy (e.g., alcohol)

47. Lyme disease Neurosis (depression/anxiety) Metastatic cancer Chronic fatigue syndrome Temporomandibular joint dysfunction Disc disease Myalgic side effect of medication (e.g., statins ) Parasitic infection Depression, anxiety

48. SUMMARY SCREENING OF IMMUNOLOGIC DISORDERS