Diseases Of The Immune System Flashcards
Anaphylaxis is what type of hypersensitivity reaction?
Type I ( Immediate) Hypersensitivity
Erythroblastosis fetalis is what type of hypersensitivity?
Type II ( Antibody-mediated) Hypersensitivity
Type III Hypersensitivity reactions are generally systemic , except for?
Arthus reaction
Arthus reaction : acute, localized inflammatory response that typically occurs after vaccination.
Psoriasis is what type of hypersensitivity?
Type IV ( T-cell mediated) hypersensitivity
What is the best screening test for SLE and is correlated with disease activity?
Anti-dsDNA
What is the triad that comprises Sjorgen Syndrome?
- Dry eyes ( keratoconjunctivitis sicca)
- Dry mouth (xerostomia)
- Other connective tissue disorders ( rheumatoid arthritis)
Name the components of CREST Syndrome?
- Calcinosis
- Raynaud phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
Which antibody is present in 10- 20% of diffuse scleroderma
Anti-Scl 70
Which disease is characterized by recurrent bacterial and enteroviral infections after 6 months?
X-linked (Burton) agammaglobulinemia
Which disease is characterized by a triad of thrombocytopenia, recurrent infections, and eczema?
Wiskott-Aldrich Syndrome
Which disease is characterized by a triad of thrombocytopenia, recurrent infections, and eczema?
Wiskott- Aldrich Syndrome
What is the antibody associated with mixed connective tissue disease?
Anti-U1-ribonucleoprotein (anti-U1-RNP)
Which organ is the most commonly affected in amyloidosis and represents the most serious form of organ involvement?
Kidney
What facilitates the attachment of the HIV virus to host cells? What facilitates fusion?
gp120-attachment;
gp41-fusion
Vascular dilatation, edema, smooth muscle contraction, mucus production, tissue injury, and inflammation are characteristic of this type of hypersensitivity?
Type I ( Immediate IgE- mediated)
Phagocytosis and lysis of cells, inflammation, and functional derangements without evidence of cell tissue injury ( in some disease) are characteristic of this type of hypersensitivity?
Type II ( Antibody mediated)
Inflammation and necrotizing vasculitis ( fibrinoid necrosis) are characteristic of this type of hypersensitivity?
Type III ( Immunecomplex mediated)
Perivascular cellular infiltrates, edema, granuloma formation, and cell destruction are characteristic of this type of hypersensitivity?
Type IV ( T-cell mediated / Delayed)
32/F with difficulty in breathing , bilateral elbow joint pains , and rash after sun exposure. CBC shows low hemoglobin and reticulocytosis. ANA titer is positive with peripheral rim pattern . What is the diagnosis?
SLE Systemic Lupus Erythematosus
Fundamental defect in SLE?
Failure of mechanisms of self-tolerance
Mechanism of organ damage in SLE?
Type III (most) .
Type II - opsonization and phagocytosis , hematologic manifestations)
Main pathogenic mechanism of autoantibody formation in SLE?
Inadequate clearance of apoptotic bodies that increase nuclear antigen burden.
Most common autoantibody in SLE?
ANA
Most specific autoantibodies for SLE ?
anti-Sm (Smith)
anti-dsDNA: correlates with disease activity
Type of LE that associated with hydralazine , INH , Procainamide, and D-penicillamine intake; rarely involves kidneys and brain , rarely associated with anti-dsDNA ; associated with anti-histone antibodies.
Drug - induced lupus
34/F with dry eyes and dry mouth. Lip biopsy shows acinar atrophy, fibrosis , and hyalinization of the minor salivary glands . What is the diagnosis?
Sjogren (late) Syndrome
Please also study the early histologic findings in Sjogren Syndrome
Most common and most important autoantibody detected in Sjogren Syndrome?
Anti-Ro (SS-A) and Anti-La (SS-B)
45/ F with chronic GERD, History of progressive dyspnea, and chronic kidney disease with thickening of the skin. What is the diagnosis.
Systemic sclerosis
Most common autoantibody associated with diffuse cutaneous systemic sclerosis?
Anti-DNA topoisomerase I (anti-Scl 70)
Common autoantibody associated with limited scleroderma ( limited cutaneous systemic sclerosis) ?
Anti-centromere antibodies
Most common fungal infection in patients with AIDS?
Candidiasis
Most common type of associated with HIV infection lymphoma?
B-cell lymphomas
AIDS- defining sarcoma caused by Human Herpes Virus 8 (HHV-8), Clinically typified by appearance of purple patches , plaques or nodules on the skin?
Kaposi Sarcoma
Arm of immune system that is more specific, has more potent response , and has memory , but requires previous exposure to the agent?
Adaptive immunity
Allergic symptoms at one place with resolution after relocation. Drug of choice?
Antihistamine
38/F with proximal muscle weakness , joint pains , and anemia. (+) U1 ribonucleoprotein (U1RNP). Diagnosis?
Mixed connective tissue disease
When a Helper T cell binds to a class II MHC protein on a displaying cell this process os known as?
Co-stimulation
Patient with a short course of an URTI but with purulent exudate are typical features with acute inflammation with what type of response?
Neutrophilic
Granuloma formation with reactivation is due to what type of hypersensitivity?
Type IV
Young female complaints of flu-like symptoms, accompanied by multiple joint pains and tenderness. Noted presence of rashes which develops after exposure to sunlight. Abnormal ANA titer and pesistent proteinuria. What antigen is involved in this immune complex mediated disease?
Nuclear antigen ( Systemic Lupus Erythematousus)
Disease associated with Warthin-Finkeldey Cells ?
Kimura Disease : is a rare chronic inflammatory disorder of unknown etiology. It usually presents as subcutaneous mass in the head and neck region and is frequently associated with regional lymphadenopathy or salivary gland involvement.
Which syndrome presents with dry eyes, dry mouth which is due to the destruction of lacrimal gland and salivary glands?
Sjogren Syndrome
Type of hypersensitivity reaction after developing difficulty of breathing with marked inspiratory stridor from laryngeal edema with mild urticaria and swelling within 5 mins of bee sting on her hand?
Type I
Type of cell that plays a part in type I hypersensitivity reaction?
Lymphocytes
Characteristics of epinephrine for treating Type 1 hypersensitivity reaction?
- Can be given parenterally , easily available emergency drug.
- The reaction is triggered by mast cell bound IgE releasing histamine.
- Fastest acting agent to treat life threatening condition.
Clinical findings of a child with sudden onset fever with pain initially on both knees later involving the ankles, and occasional movements of upper and lower extremities. Had sore throat 2 weeks ago.
Rheumatic fever Arthritis is the most common presentation.
Sydenham’s chorea- involuntary movements of the limbs.
Pathogenesis of rheumatic fever?
Antigen antibody reaction
Most characteristic lesions of SLE result from immune complex deposition in which of the least area?
Skin
Factors contributing to the pathogenesis of SLE?
Drugs, Sex Hormones, UV light exposure
Hallmark of SLE ?
Production of autoantibodies
Immune cells that rapidly recognize and destroy virally infected cells ?
NK cells
Intracellular pathway that mediates EGF binding to the epidermal cell surface receptors which is followed by transcription factor translocation and DNA transcription?
Cyclin- dependent Kinase
General features of autoimmune diseases?
- Damaged caused by autoimmune diseases is progressive Chronic with relapses and remissions.
- Clinical and pathologic manifestations are determined by nature of underlying immune response.
Basic patterns seen on indirect immunofluorescence in patients with Systemic Sclerosis? (2)
Nucleolar Pattern & Centromeric Pattern
Drugs that can induce SLE-like response to humans? (3)
Hydralazine
Procainamide
D-penicillamine
