4 immunology-csbrp
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4 immunology-csbrp
- 1. Disorders of Immunity - 4 Autoimmunity Dr.CSBR.Prasad, M.D. APR-2015-CSBRP
- 2. Autoimmune diseasesAutoimmune diseases Immune reaction against self antigen DEFINITION Autoimmunity can be defined as breakdown of mechanisms responsible for self tolerance and induction of an immune response against components of the self. Such an immune response may not always be harmful (e.g., anti-idiotype antibodies). However, in numerous autoimmune diseases it is well recognized that products of the immune system cause damage to the self. APR-2015-CSBRP
- 3. Pathogenesis of autoimmune diseases 1 Sex is a key factor in autoimmune disease, ie a number of autoimmune diseases are much more common in women than in men. 2 There is a genetic background to autoimmune diseases and a strong correlation with particular MHC haplotypes. MHC associations differ in different ehtnic groups. 3 T helper cells play a major role in autoimmunity through TH1 clones triggering a DTH response. Auto-reactive cytotoxic T cells develop more rarely but are found in IDDM. 4 At least in experimental models and in certain human auto-immune diseases somatic hypermutation is an important process leading to auto-reactive antibodies. 5 Infection by viruses (HCMV, EBV) or bacteria results in polyclonal B cell activation. This may constitute a mechanism for activation of anergic, self reactive B clones. 6 Exposure of segregated antigens can lead to autoimmune diseases. 7 Molecular mimicry, ie the sharing of identical are highly similar antigenic determinants between viral /bacterial antigens and self antigens, plays little if any role in autoimmunity. APR-2015-CSBRP
- 4. Regulatory T cellsRegulatory T cells • Regulatory T cells (Formerly called suppressor cells) • Express CD4+ and CD25+. • They express forkhead family transcription factor Foxp3. Expression of Foxp3 is required for regulatory T cell development and function. • They supress immune activation by the production of immunosuppressive cytokines such as TGF-β and IL-10. • Genetic mutations in Foxp3 in humans leads to development of a severe and rapidly fatal autoimmune disorder known as Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) syndrome. • This disease provides the most striking evidence that regulatory T cells play a critical role in preventing autoimmune disease.APR-2015-CSBRP
- 5. Some notable pointsSome notable points • AI diseases are more common in females (75% of pts) • Autoantibodies appears normally with increasing age • Hypergammaglobulnemia is usually present • Autoantibodies against Adrenals, Parathyroids, Pancreatic islets and mitochondria are uncommon at any age • There is strong genetic predisposition • There may be familial clustering of AID • A patient with one type of organ specific AID is quite likely to develop another** • The course of AID is variable and not always progressive APR-2015-CSBRP
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- 14. Systemic Lupus Erythematosis - SLE APR-2015-CSBRP
- 15. Systemic Lupus Erythematosis • Autoimmune disease • Multisystem affection • Characterized by presence of Antinuclear Antibodies (ANA) • Acute or insidious in onset • Chronic remitting & relapsing febrile illness • Principally affects skin, joints, kidney & serosal membrane APR-2015-CSBRP
- 16. Systemic Lupus Erythematosis • American Rheumatism Association • 1997 revised 11 criteria for SLE APR-2015-CSBRP
- 17. SLE – 1997 Revised criteria 1. Malar rash – Fixed erythema over malar eminence 2. Discoid rash – Erythematous raised patches with adherent keratotic scaling & follicular plugging 3. Photosensitivity – Skin rash after UV light exposure 4. Oral ulcers –Painless oral / nasopharyngeal ulcers 5. Arthritis – Non-erosive arthritis involving 2 or more joints 6. Serositis – Pleuritis / Pericarditis 7. Renal disorder – Persistent proteinuria >0.5 g/dl Cellular cast APR-2015-CSBRP
- 18. SLE – 1997 Revised criteria 8. Neurological disorder – Seizures, Psychosis 9. Hematological disorder Hemolytic anemia with reticulocytosis Leukopenia < 4000/mm3 Lymphopenia <1500/mm3 Thrombocytopenia <1.0 lakh /mm3 10. Immunological disorder Anti–dsDNA, anti–sm & / or antiphospholipid 11. Antinuclear antibody (ANA) Abnormal titre of ANA in the absence of drug induced SLE APR-2015-CSBRP
- 19. APR-2015-CSBRP
- 20. Other antibodies Antinuclear antibody (ANA) Anti - ds DNA, Anti - ss DNA Anti – Sm Ag Anti ribonucleoprotiens( anti RNA) Anti histone antibodies Anti-Nucleolar antibodies Antiphospholipid antibodies APR-2015-CSBRP
- 21. Diagnosis of SLE A person is said to have SLE if any 4 or more of the 11 criteria are present serially or simultaneously during any interval of observation APR-2015-CSBRP
- 22. Systemic Lupus Erythematosis Etiology & pathogenesis Failure of self tolerance mechanisms 1.Genetic factors 2.Environmental factors 3.Immunological mechanism APR-2015-CSBRP
- 23. Systemic Lupus Erythematosis Genetic factors • Family members have increased risk of developing SLE • Upto 20 % of clinically unaffected I0 relatives show ANA • High rate of concordance in monozygotic twins • MHC genes regulate production of auto-Ab APR-2015-CSBRP
- 24. Systemic Lupus Erythematosis Environmental factors • Drugs • Hydralazine, • Procainamide, • D-Pencillamine can induce SLE like lesions • UV light – exacerbate the disease • Sex hormones – SLE 10x more common in females than males APR-2015-CSBRP
- 25. Systemic Lupus Erythematosis Immunological factors • Suppression of regulatory T cells • Inherited defect in B cells • Most of visceral lesions – Type III reactions • Auto antibodies against red cells, WBC & platelet mediate their effect by -Type II reaction APR-2015-CSBRP
- 26. APR-2015-CSBRP
- 27. SLE – Clinical features & pathologic manifestation • Hematologic – 100 % • Arthritis – 90% • Skin – 85% • Fever – 83% • Fatigue – 81% • Weight loss – 63% • Renal – 50% • CNS – 50% • Pleuritis – 46% • Pericarditis – 33%APR-2015-CSBRP
- 28. SLE - Morphology • Immune complex disease – Blood vessel, kidney, connective tissue & skin • Acute necrotizing vasculitis – small vessels & arterioles • Chronic stage – fibrous thickening with luminal narrowing APR-2015-CSBRP
- 29. SLE - KidneySLE - Kidney All SLE patient show lesion in kidney WHO – 5 types of lupus nephritis • Class I –Normal by light M/s, EM & IF • Class II – Mesangial lupus GN • Class III – Focal proliferative GN • Class IV – Diffuse proliferative GN • Class V - Membranous GN • Class VI – Sclerosing nephropathy APR-2015-CSBRP
- 30. Mesangial lupus GN • Increase in mesangial matrix & cells • Granular mesangial deposits of Ig & complement are always present • Mild hematuria/transient proteinuria APR-2015-CSBRP
- 31. Focal proliferative GN • Focal lesion, affecting less than 50 % of the glomeruli • Swelling & proliferation of endothelial & mesangial cells • Neutrophils • Fibrinoid deposits & intra capillary thrombi • Hematuria & proteinuria APR-2015-CSBRP
- 32. APR-2015-CSBRP
- 33. Diffuse proliferative GN • Proliferation of endothelial, mesangial cells & epithelial cells producing epithelial crescent • Fibrinoid necrosis & leukocyte infiltration • Most or all glomeruli are involved • Gross hematuria & proteinuria • Nephrotic syndrome – 50% APR-2015-CSBRP
- 34. Figure 20-16 Acute proliferative glomerulonephritis. A, Normal glomerulus. B, Glomerular hypercellularity is due to intracapillary leukocytes and proliferation of intrinsic glomerular cells. C, Typical electron-dense subepithelial "hump" and a neutrophil in the lumen. APR-2015-CSBRP
- 35. Membranous GN • Thickening of capillary wall • Severe proteinuria - nephrotic syndrome • EM – subepithelial deposits of immune complex APR-2015-CSBRP
- 36. APR-2015-CSBRP
- 37. SLE - SKIN • Malar rash – 50-60% cases • Liquefactive degeneration of basal layer • Edema, perivascular infiltrate • Vasculitis & fibrinoid necrosis of vessels • Ig & complement deposition along dermo- epidermal junction APR-2015-CSBRP
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- 45. SLE - Joints • Non erosive synovitis with little deformity • Neutrophilic infiltration & fibrin – synovium • Perivascular mononuclear infiltrate in subsynovial tissue APR-2015-CSBRP
- 46. SLE - CVSSLE - CVS • Vegetations on tricuspid & mitral valve – Libman- Sacks endocarditis APR-2015-CSBRP
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- 48. APR-2015-CSBRP
- 49. Diagnosis of SLEDiagnosis of SLE APR-2015-CSBRP
- 50. SLE - ANA • ANA Major pathogenetic significance • Four types 1.Ab to DNA 2.Ab to Histone 3.Ab to non-histone proteins bound to RNA 4.Ab to nucleolar Ag APR-2015-CSBRP
- 51. SLE - ANA • Detected by Indirect Immunoflurorescence • Four basic staining pattern 1.Homogenous / diffuse nuclear staining Ab to chromatin & histone 1.Peripheral staining Ab to double stranded DNA 1.Speckled pattern – least specific 2.Nucleolar pattern – Ab to nucleolar RNA APR-2015-CSBRP
- 56. SLE - ANA • IIF test for ANA is positive in all SLE patients • Highly sensitive / Not specific • Anti ds DNA & anti Sm Ag – Diagnostic of SLE • Others – Anti histone, nuclear RNP, SS-A, SS-B, scl-70 APR-2015-CSBRP
- 57. SLE – other tests LE cell Phagocytic leukocyte (neutrophil) that engulfed the denatured nucleus of an injured cell APR-2015-CSBRP
- 58. Diagram of sequence of formation of LE cells in vitro Phase-I Phase-II Phase-III APR-2015-CSBRP
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- 61. APR-2015-CSBRP
- 62. LE prepLE prep • Traumatize leucocytes by rotating 10ml of defibrinated blood with glass beads • Incubating the prep for 1hour at 37°C • Separate the buffy coat and make a smear • Stain with leishman stain • Observe under oil immersion APR-2015-CSBRP
- 63. LE cells may also be seen inLE cells may also be seen in • Pericardial effusions • Pleural effusions • CSF • Synovial fluid APR-2015-CSBRP
- 64. Morphological variants of LE cells • LE body • LE rosette APR-2015-CSBRP
- 65. “Tart cellTart cell” • It’s a monocyte / histiocyte with engulfed viable nuclear material • It’s not to be equated with LE cell • It has no diagnostic value • Importance: It should not be mistaken for LE cell APR-2015-CSBRP
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- 90. Pernicious anemia Abs against gastric parietal cells causes atrophic gastritis, achlorhydria and reduced secretion of IF APR-2015-CSBRP
- 91. APR-2015-CSBRP
- 92. Goodpasture syndromeGoodpasture syndrome Autoantibodies directed to components of the basal membranes of lung alveoli and kidney glomeruli induce complement activation and a local inflammatory response which leads to tissue damage APR-2015-CSBRP
- 93. How is Goodpasture's Syndrome Diagnosed? When a patient presents with the initial indicators of Goodpasture's Syndrome (hemoptysis and hematuria), an anti-GBM antibody titer, bronchoscopy and renal biopsy may provide a definitive diagnosis of GS (Avella et al., 1999). The anti-GBM antibody titer is indicated when patients have acute renal failure, pulmonary hemorrhage and/or rising serum creatine concentrations with hematuria (Hellmark et al., 1997). The Anti-GBM antibody titer may eliminate other disorders, as these symptoms may be indicative of other diseases (Hellmark et al., 1997). A definitive diagnosis of GS will include an anti-GBM Ab titer test of greater than 20 units (normal = 0-9 units), bloody secretions in the bronchoscopy, and a linear distribution of IgG on the basement membrane of the renal biopsy (Avella et al., 1999). APR-2015-CSBRP
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- 96. Insulin dependent diabetes mellitus 50% may have islet cell Abs (ICAbs) at the time of diagnosis ICAbs in family members indicates a high risk of subsequent diabetes APR-2015-CSBRP
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- 102. systemic sclerosis- hands showing tight shiny skin sclerodactyly, flexion contracture of fingers and thickening of tendon sheath.APR-2015-CSBRP
- 103. APR-2015-CSBRP
- 105. Grave’s diseaseGrave’s disease Abs to TSH receptors on thyroid acinar cells (TSA) TSA is stimulatory TSA is absent in nodular goitre / adenoma APR-2015-CSBRP
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- 107. Marty Feldman (1934 – 1982) English writer, comedian Notable for his bulging eyes He wrote situation comedies such as The Army Game He also appeared in ‘The Adventure of Sherlock Holmes’ APR-2015-CSBRP
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Editor's Notes
- DEFINITION: Autoimmunity can be defined as breakdown of mechanisms responsible for self tolerance and induction of an immune response against components of the self. Such an immune response may not always be harmful (e.g., anti-idiotype antibodies). However, in numerous autoimmune diseases it is well recognized that products of the immune system cause damage to the self.
- Regulatory T cells (Formerly called suppressor cells) Recently, a distinct population of T cells has been discovered called regulatory T cells. Regulatory T cells come in many flavors, but the most well characterized include those that express CD4+ and CD25+. Because activated normal CD4 T cells also express CD25, it was difficult to distinguish regulatory T cells and activated T cells. The latest research suggests that regulatory T cells are defined by expression of the forkhead family transcription factor Foxp3. Expression of Foxp3 is required for regulatory T cell development and function. The precise mechanism/s through which regulatory T cells suppress other T cell function is not clear. One of the mechanisms include the production of immunosuppressive cytokines such as TGF-β and IL-10. Genetic mutations in Foxp3 in humans leads to development of a severe and rapidly fatal autoimmune disorder known as Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked (IPEX) syndrome. This disease provides the most striking evidence that regulatory T cells play a critical role in preventing autoimmune disease.
- ** Over 1/3rd of patients with primary Addison’s disease suffer from some of the other organ specific AID. A 1/3rd of Caucasians cause of thyrotoxicosis are seropositive for gastric parietal cell antibodies; and over ½ of the cases of Sjogren’s syndrome have rheumatoid arthritis.
- Figure 20-16 Acute proliferative glomerulonephritis. A, Normal glomerulus. B, Glomerular hypercellularity is due to intracapillary leukocytes and proliferation of intrinsic glomerular cells. C, Typical electron-dense subepithelial &quot;hump&quot; and a neutrophil in the lumen.
- Figure 20-19 Membranous glomerulonephritis. A, PAS stain. Note the marked diffuse thickening of the capillary wall without an increase in the number of cells. B, Electron micrograph showing electron-dense deposits (arrow) along the epithelial side of the basement membrane (B). Note the obliteration of foot process overlying deposits. CL, capillary lumen; End, endothelium; Ep, epithelium. C, Characteristic granular immunofluorescent deposits of IgG along GBM. D, Diagrammatic representation of membranous glomerulonephritis.
- LUPUS ERYTHEMATOSUS, DISCOID / LUPUS ERYTHEMATOSUS CASE-2 This 43 year old woman had an 11 year history of slowly progressive red scaly plaques with central scarring and hair loss in sun exposed areas. A biopsy showed changes typical of discoid lupus. She was otherwise well and complained of itching of her feet where she had new lesions. She also had some asymptomatic erosions on the tongue and buccal mucosa.
- LUPUS ERYTHEMATOSUS, DISCOID / LUPUS ERYTHEMATOSUS CASE-2 This 43 year old woman had an 11 year history of slowly progressive red scaly plaques with central scarring and hair loss in sun exposed areas. A biopsy showed changes typical of discoid lupus. She was otherwise well and complained of itching of her feet where she had new lesions. She also had some asymptomatic erosions on the tongue and buccal mucosa.
- LUPUS ERYTHEMATOSUS, DISCOID / LUPUS ERYTHEMATOSUS CASE-2 This 43 year old woman had an 11 year history of slowly progressive red scaly plaques with central scarring and hair loss in sun exposed areas. A biopsy showed changes typical of discoid lupus. She was otherwise well and complained of itching of her feet where she had new lesions. She also had some asymptomatic erosions on the tongue and buccal mucosa. Well demarcated crusted pink plaques with depigmentation, atrophy, and hair loss
- This 65-years-old woman developed multiple, well demarcated, red to violaceous papules and plaques associated with mild pruritus and photosensitivity involving her nose and malar areas 6 months earlier. The small papules became confluent to form large plaques. She also developed superficial pustules from secondary bacterial infection probably due to scratching one month earlier. She had associated arthralgia of the small joints of her hands and positive ANA and Anti Ro autoantibodies. NOTES: Lupus erythematosus has two predominant forms, cutaneous, otherwise known as discoid lupus (DLE) and systemic (SLE). The former is found in sun-exposed sites with persistent, sharply demarcated, raised, scaly erythematous papules or plaques less than 1cm in diameter with distinctly palpable infiltrate. Through peripheral growth, the lesions become disc shaped (discoid) and may become confluent. The hyperaesthesia of the lesions is also of diagnostic importance; passing the edge of the fingernail over the lesions causes pain. Whereas the lesions slowly spread out from the margins, regression commences in the centre, resulting in atrophy. The rash of SLE is photosensitive but classically affects the interphalangeal area and spares the skin over the PIP / MCP joints. &quot;Butterfly&quot; rashes are only seen in 5-10% of cases of SLE.
- A healthy 2 month old boy developed round and annular red papules and plaques on his scalp, face, and trunk. His mother had a history of subacute cutaneous lupus erythematosus, and both mother and baby had positive serology for anti-Ro (ssA) and anti-La (ssB) antibodies. He had no other signs of lupus, and his rash disappeared by 9 months of age. round and annular red papules and plaques with subtle atrophy.
- The LE cell. The large homogeneous areas adjacent to these polymorphs&apos; nuclei each contain the nucleus of another being digested. Free lysed nuclear material can also be seen in the lower left example
- It was named after a patient in whom it was identified.
- RHEUMATOID GRANULOMA / RHEUMATOID NODULE This 64 year old woman with longstanding rheumatoid arthritis developed nontender rubbery nodules on her elbows. She also had chronic synovitis with severe involvement of the wmall joints of her hands. A number of her nails demonstrate chronic distal subungual onychomycosis with yellowing, onycholysis, and scaling of the finger tips.
- firm rubbery subcutaneous nodule.
- IRITIS (ANTERIOR UVEITIS).Causes include:- Autoimmune disorder (Juvenile rheumatoid arthritis, ankylosing spondylsis, Reiter&apos;s syndrome, inflammatory bowel disease, psoriasis). - Infections (Syphilis, TB, Herpes Zoster, Herpes Simplex, Adenovirus).- Malignancy ( Masquerade syndrome-Retinoblastoma, Leukemia, Lymphoma, Malignant melanoma,).- Trauma.- Vision becomes gradually blurred.- Mild to marked pain.- Slight to marked photophobia.- Diffusely red with slight to marked circumcorneal flush.- Small and irregular shape if posterior synechiae are present.- Keratic precipitates on posterior surface of cornea.- Flare and cell in the anterior chamber can be seen with the slit lamp.- Course may be acute or chronic.- Recurrences are common sometimes.
- This 9 year old boy developed a generalized recalcitrant vesiculobullous eruption with involvement of the oral pharynx, nasal pharynx, larynx, conjunctivae, and perianal skin. He failed to stay in remission with oral and parenteral steroids, methotrexate, and dapsone. He eventually cleared on plasmaphoresis and pulse parenteral cyclophosphamide give in conjuction with a 6 month tapering course of oral steroids, and a year of oral cytoxan. FIG-1 generalized vesicles and bullae with many on the border of annular eroded plaques.
- Pemphigus vulgaris can present with oral lesions. A suprabasal split is characteristic, and acantholysis of individual cells is variable. The epithelium above the basal layer often detaches in an intact manner as is seen in this biopsy. The detached epithelium tends to slough away very soon within the mouth, so a biopsy of a very early lesion or of a fresh, induced lesion (local trauma) may be necessary to demonstrate the diagnostic pathology. Medium power view. The section is overstained. There is a layer of basal cells in the base of the cleft. The inflammatory infiltrate is composed mostly of lymphocytes with a few plasma cells mixed in.
- Figure 3. Histopathology and glandular manifestations of Sjögren&apos;s syndrome. Tongue with atrophic papillae and dry and deeply fissured epithelium that replaces functional glandular epithelium; cheilitis is evident bilaterally. A possible cause of these manifestations might be xerostomia, either alone or in combination with fungal infection. (B) Missing teeth and multiple caries. (C) Persistent enlargement of parotid glands. Figure 4. Extraglandular features of Sjögren&apos;s syndrome. (A) Palpable purpura in the lower extremities. (B) Multiple necrotic cutaneous ulcers of the lower extremities in a patient with primary Sjögren&apos;s syndrome and cryoglobulinemia. (C) Annular urticarial lesions of the trunk.
- Histopathologic image of focal lymphoplasmacytic infiltration in the minor salivary gland associated with Sjögren syndrome. Lip biopsy. H & E stain.
- An example of the linear distribution of IgG on the glomerular basement membrane.
- This 68-year-old woman developed progressive fibrotic atrophic plaques with hemorrhagic vesicles and bullae on her abdomen, chest, and extremities. Skin biopsies showed changes consistent with morphea and lichen sclerosis et atrophicus. She had no systemic disease and failed to improve with topical and oral steroids and phototherapy (PUVA). Diffuse atrophy, fibrosis, scaling and scattered hemorrhagic vesicles and bullae.
- systemic sclerosis- hands showing tight shiny skin sclerodactyly, flexion contracture of fingers and thickening of tendon sheath.
- Scleroderma- notice cachexia due to dysphagia(from an oesophageal motility disturbance) or malabsorption )due to bacterial overgrowth.Sclerodactyly, tethered smooth skin, calcinosis and ulceration. Atrophy of the finger pulps due to Raynaud’s phenomenon, and fixed flexion deformities of the fingers.
- Marty Feldman (1934 – 1982) was an English writer, comedian and BAFTA award winning actor, notable for his bulging eyes, which were the result of a thyroid condition known as Grave’s Disease. He wrote situation comedies such as The Army Game, Bootsie and Snudge for the BBC and later had a series of his own on the BBC called Marty. He also appeared in The Adventure of Sherlock Holmes’ Smarter Brother and Mel Brooks’ Silent Movie, as well as directing and starring in The Last Remake of Beau Geste. He guest-starred in the “Arabian Nights” episode of The Muppet Show.
- DERMATOMYOSITIS/HELIOTROPE A 38 year old woman developed persistent violaceous edema of the upper eye lids. A skin biopsy showed vacuolar change at the dermal-epidermal junction and a mild lymphocytic infiltrate consistent with collagen vascular diseae. She had no other cutaneous findings or systemic symptoms. A full medical evaluation was pending.Symmetric violaceous edematous plaques with scattered distinct telangiectasias.
- DERMATOMYOSITIS / TELANGIECTASIA A 45 year old woman who complained of chronic fatigue developed a heliotrope rash with violaceous erythema of the eye lids associated with periorbital edema. She was also noted to have edema of her hands associated with periungual erythema and telangiectasias. Electromyography and a muscle biopsy showed no evidence of myositis, and she was diagnosed with amyopathic dermatomyositis without underlying malignancie. Oral prednisone (1 mg/kg/d) was given in a single daily dose, and her fatigue and skin findings improved. Edema of the fingers associated with periungual erythema and telangiectasias.
- DERMATOMYOSITIS / TELANGIECTASIA A 45 year old woman who complained of chronic fatigue developed a heliotrope rash with violaceous erythema of the eye lids associated with periorbital edema. She was also noted to have edema of her hands associated with periungual erythema and telangiectasias. Electromyography and a muscle biopsy showed no evidence of myositis, and she was diagnosed with amyopathic dermatomyositis without underlying malignancie. Oral prednisone (1 mg/kg/d) was given in a single daily dose, and her fatigue and skin findings improved. Edema of the fingers associated with periungual erythema and telangiectasias.
- Dermatomyositis. Sheet-like calcifications seen in patients withdermatomyositis is called calcinosis universalis because of its wide-spread distribution. This is more likely to occur in younger patients with dermatomyositis.
- Vitiligo-associated with other autoimmune diseases (Ex: Pernicious anemia, DM, Thyroiditis et.c.)