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IHO 5 > Autoimmune diseases > Flashcards

Autoimmune diseases Flashcards

1
Q

Autoimmune disease

A
  • Tissue and cell injury due to immune reactions to self-antigen
  • Can be mediated by autoantibodies, immune complexes, or T lymphocytes
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2
Q

Does the presence of autoantibodies alway indicate the presence of an autoimmune disease?

A

NO

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3
Q

What is the primary cause of autoimmune disease?

A

Loss of self-tolerance. Can be a loss of central or peripheral tolerance.

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4
Q

Central tolerance

A

Killing of immature self-reactive T and B lymphocytes that recognize self-antigens in the central lymphoid organs

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5
Q

Peripheral tolerance

A

Anergy, suppression by Treg cells, deletion of self-reacting lymphocytes by apoptotic cell death.

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6
Q

What are 2 tissues that are not exposed directly to blood and lymphocytes?

A

Eye and testes. Antigen can hide out here and cause an autoimmune reaction if tissues damaged due to an infection or trauma

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7
Q

2 factors that when combined together lead to autoimmune disease

A
  • Inheritance of susceptibility genes

- Environmental triggers

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8
Q

Do the presence of specific HLA alleles lead to autoimmunity?

A

No. Complex multigenic disorders usually. The greatest contribution is with HLA genes though.

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9
Q

What ways can infections mediate autoimmunity?

A
  • Can upregulate expresssion of co-stimulators of APCs–can cause breakdown in anergy process
  • Molecular mimicry
  • Some infections cause polyclonal B lymphocyte activation which can lead to autoantibody production.
  • Tissue injury from infection may release self antigens and structurally alter self-antigen
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10
Q

Molecular mimicry

A

Offending organism expresses antigens that have the same amino acid sequences of self-antigens. Can result in immune response to self-antigens

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11
Q

Are infections always negative in regard to autoimmunity?

A

No. Infections may also protect against some autoimmune diseases. (Hygiene hypothesis)

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12
Q

Typical clinical course of untreated autoimmune disease

A
  • Progressive once initiated. Always slowly progressive even if waxing and waning of symptomology.
  • May be directed at specific organ or directed at widespread antigens
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13
Q

Collagen vascular diseases

A

Systemic autoimmune diseases that tend to involve blood vessels and connective tissues

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14
Q

Pathogenic mechanism for SLE

A

-Susceptibility genes interfere with maintenance of self-tolerance and external triggers–>persistence of nuclear antigens–>Ab response against self-nuclear antigens–>amplified by action of nucleic acids on DCs and B cells and production of type 1 IFNs, TLRs.–>formation of antigen-antibody compexes that are deposited in tissues–> leads to inury

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15
Q

Primary test done for SLE

A

Anti-nuclear antibodies! If positive, anti-DNA and anti-Sm tests to help verify

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16
Q

What can cause ANA to be +

A
  • SLE
  • All connective tissue diseases
  • Many medications give false +
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17
Q

What is the primary hypersensitivity type of SLE?

A

Type III. Immune complex-mediated disease

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18
Q

What part of SLE can lead to a type II hypersensitivity?

A

Abs directed against RBCs, platelets, white cells–> cytopenias. Antibody-mediated hypersensitivity

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19
Q

Potential complication of anti-phospholipid antibodies in SLE

A
  • False postive for syphilis test
  • Can prolong partial throboplastin time (lupus anticoagulant)
  • Can cause venous and arterial thrombosis (hypercoaguable state)
  • Spontaneous miscarriages, cerebral ischemia (secondary anti-phospholipid Ab syndrome)
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20
Q

Key pathological findings in SLE

A

Serositis, oral ulcers, photosensitivity, pulmonary fibrosis, blood cells, renal, Raynauds, ANA, Immunologic, neuropsych, malar rash, discoid rash. (SOAP BRAIN MD). Granular formation in glomerulus

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21
Q

Chronic discoid lupus erythematosus

A
  • Predominantly limited to skin
  • Chronic photosensitive dermatosis with atrophy and scarring.
  • Don’t have systemic manifestiations of classic SLE
  • Usually negative for antibodies to double stranded DNA
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22
Q

Subacute cutaneous lupus erythematosus

A
  • Predominantly limited to skin
  • Mild systemic lesions may be present
  • Association to antibodies to SS-A and HLA-DR3 gene
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23
Q

Drug induced lupus erythematosus

A

Some drugs (procainamide and hydralazine) bind histones and cause them to be immunogenic. Anti-histone antibodies develop and lupus-like syndrome produced

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24
Q

Rheumatoid arthritis pathogenic mechanism

A

Triggered by exposuer to arthritogenic (arthritis causing) antigen in genetically predisposed indival that leads to breakdown of self-tolerance and a chronic inflammatory reaction (T and B cell response to self antigens leading to release of collagease, stromelysin, elastase, PGE2, other enzymes)

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25
Q

Rheumatoid arthritis

A

Primarily attacks the joints, may affect many tissues and organs. Produces nonsuppurative prolf=iferative and inflammatory synovitis that often progresses to destruction of articular cartilage and ankylosis of joints

26
Q

What are many of the autoantibodies produced in RA specific for?

A

Citrullinated peptides (CCPs). Can be used as diagnostic test for RA.

27
Q

CCPs

A
  • produced during inflammation
  • insults such as infection and smoking may promote citrullination of self proteins, triggering the autoimmune reactions in genetically susceptible individuals.
28
Q

Is rheumatoid factor specific for RA?

A

No. Can be seen in 1-5% of healthy people

29
Q

What is a moer specific test for RA?

A

ACCP (anti-cyclic citrullinated peptide antibodies)

30
Q

Specific immunopathogeneis of RA

A

Activation of CD4+ T cells as well as B cells results in the formation of a pannus (mass of inflamed synovium) which grows over the joint cartilage and results in inflammatory destruction of the joint.

31
Q

Characteristic features of RA

A
  • Chronic papillary synovitis

- dense chronic inflammatory infiltrate rich in plasma cells

32
Q

Rheumatoid nodules

A

areas of central fibrinoid necrosis surrounded by a palisade of macrophages and scattered chronic inflammatory cells

33
Q

Sjogren syndrome clinical findings

A
  • Dry eyes
  • Dry mouth
  • Typically middle-aged women
  • May have extraglandular disease (synovitis, diffuse pulmonary fibrosis, peripheral neuropathy)
  • Can primary or secondaary (in association with another immune disorder)
34
Q

Sjogren syndrome pathogenesis

A
  • Autoimmune, immunologically mediated destruction of lacrimal and salivary glands.
  • Likely related to abherrent B and T cell activation in genetically susceptible ind.
35
Q

Sjogren syndrome pathology

A

lymphocytic inflammation involving lacrimal and salivary glands, followed by fibrosis and gland atrophy as the disease develops. May also see parotid gland enlargement due to inflammation (Mikulicz disease)

36
Q

What type of cancer are patients with Sjogren syndrome more likeley to develop?

A

Lymphoma (often MALT lymphoma)

37
Q

What do patients w/Sjogren syndrome typically have autoantibodies to?

A

Ribonucleoproteins SS-A and SS-B.

38
Q

Diagnosis of Sjogren syndrome

A

Measure SS-A and SS-B

Lip biopsy

39
Q

Scleroderma (systemic sclerosis) clinical features

A

3:1 F:M ratio.
Adults
Skin most commonly effectled, also GI tract, kidneys, heart, lungs.

40
Q

Scleroderma pathology

A

widespread damage to small blood vessels and progressive interstitial and perivascular fibrosis of the skin and multiple organs.
Pathogenic findings secondary to ischemic damage and fibrosis in the affected organs

41
Q

Diffuse scleroderma

A

widespread skin involvement at onset, with rapid progression and early visceral involvement.

42
Q

Limited scleroderma

A

skin involvement is confined to the fingers, forearms, and face, with late visceral involvement (more indolent form). Some patients with the limited form develop the CREST syndrome

43
Q

CREST Syndrome*****

A

Can occur in patients with limited scleroderma

calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia

44
Q

Antibodies present in scleroderma (systemic sclerosis)

A

SLA-70

Patients with CREST syndrome may have anti-centromere antibodies

45
Q

Scleroderma skin findings

A

clerotic atrophy and sclerosis (sclerodactyly), beginning in the distal fingers and extending proximally.
Changes can also involve the face; extensive dystrophic calcification in the subcutaneous fat can also be present.

46
Q

Scleroderma GI tract findings

A

involved in 90% of patients; esophageal fibrosis results in dysmotility, with dysphagia and reflux; small bowel involvement can result in loss of villi and dysmotility with malabsorption, cramps, and diarrhea.

47
Q

Raynaud’s phenomenon

A

Most common intital complaint in systemic sclerosis

48
Q

Scleroderma lung findings

A

interstitial fibrosis (respiratory failure is the most common cause of death).

49
Q

Scleroderma musculoskeletal system findings

A

non-destructive arthritis; 10% of patients can develop an inflammatory myositis indistinguishable from polymyositis.

50
Q

Scleroderma kidney findings

A

vascular thickening; patients may develop hypertension.

51
Q

Inflammatory myopathies

A

Dermatomyositis and polymyositis

52
Q

Dermatomyositis

A

autoimmune disease with immunologic injury and damage to small blood vessels and capillaries in the skeletal muscle, along with skin involvement and characteristic skin rash

53
Q

Dermatomyositsis clinical manifestations

A

Typically involve musle weakness (proximal muscles first, symmetric) and a rash (violaceous discoloration of upper eyelids with periorbital edema, dusky red pathes over knuckles, elbows and knees–Gottron papules).
Can have interstitial lung disease, dysphagia, myocarditis.

54
Q

Dermatomyositis pathology

A

Muscle biopsy shows lymphocytic inflammation around small blood vessels and in the perimysial connective tissue, along with perifascicular myocyte atrophy secondary to ischemia. Necrotic muscle fibers with regeneration can also be seen. Activated B and T cells and antibodies with complement activation are involved in the capillary damage.

55
Q

What do you need to check for in patients newly diagnosed with dermatomyositis

A

Malignancy! (15-25% of cases have underlying malignancy)

56
Q

Dermatomyositis juvenile form (more common clinical manifestations that occur)

A

Abdominal pain and GI involvement

57
Q

Treatment for dermatomyositis and polymyositis

A

Immunosuppressive agents

58
Q

Lab results in dermatomyositis and polymyositis

A

Elevated creatine kinase

59
Q

Polymyositis

A
  • Muscle and systemic involvement is similar to that seen in dermatomyositis, except for the lack of skin involvement.
  • mainly adults
60
Q

Polymyositis pathology

A

Immunologic injury to muscle by activated CD8+ cytoxic T cells.
-lymphocytic inflammation surrounding and invading muscle fibers, without the perifascicular atrophy seen in dermatomyositis. Necrotic and regenerating muscle fibers are found throughout the fascicle. No vascular injury is seen.

61
Q

Mixed connective tissue disease

A

Overlap features of 6 main systemic connective tissue diseases and presence of ANTI-U1-RNP antibody

62
Q

Secondary Sjogren syndome

A

Sjogren syndrome associated with 1 of these: SLE, RA, systemic sclerosis, polymyositis, dermatomyositis

IHO 5 (5 decks)

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