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Pemphigus complex Flashcards

1
Q

Prevention of auto-immunity by auto-reactive T cells (and B cells):

A
  1. Clonal deletion (central tolerance)
  2. Peripheral deletion (T cells, perhaps B cells): recognition of self-antigen → Fas-FasL interaction → apoptosis
  3. Immunological ignorance (T cells): hidden antigens
  4. Anergy (T cells, B cells): antigen presentation without co-stimulatory signals
  5. Suppression (T cells): Treg
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2
Q

Factors involved

A
  1. Genetics: genes for MHC, cytokines, Treg, IgA deficiency
  2. Infections: production of INF-γ (MHC-II up-regulation), superantigens, molecular mimicry
  3. IgA deficiency: exposure to mucosal microbes → cross-reactivity
  4. Vaccination
  5. Environmental: UV
  6. Hormonal: female predisposition in humans and rodents; not in D, C
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3
Q

Auto-immune- definition

A

adaptive immune response to autoantigens

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4
Q

Which part of the body have normal Ig deposition

A

nasal planum and footpads

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5
Q

Demonstration of serum auto-antibodies: indirect IF
-wich tissues are the best for PF,PV, PNP, AISBD

A

Canine PF: substrate of choice is neonatal mouse skin; in C feline footpad and oral mucosa
Canine PV: substrate of choice is canine gingival mucosa
Canine PNP: bladder epithelium
AISBD: substrate of choice is canine lip (especially intact and salt-split)

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6
Q

Phases of treatment:

A
  1. Induction: days to weeks
  2. Transition: gradual tapering until recurrence or discontinuation
  3. Maintenance: if recurrence during tapering; usually x 8-12 months
  4. Determination of cures: at transition or after maintenance phase
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7
Q

MOA of tetracyclines

A

a) inhibition of lymphocyte blastogenesis, Ab production;
b) inhibition of WBC chemotaxis;
c) inhibition of C3;
d) inhibition of PG, lipases and MMPs (collageneases);
e) downregulation of cytokines;
f) inhibition of angiogenesis,
g) inhibition of apoptosis

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8
Q

MOA of niacinamide

A

a) inhibition of mast cell degranulation;
b) inhibition of PDE;
c) inhibition of enzymes (proteases);
d) photoprotectant;
e) AcR agonist (cholinomimetic)

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9
Q

MOa of hydroxychloroquine

A

Increase pH → interference with MHC and antigen complex formation → blocks stimulation of Th cells; reduced presentation of auto-antigens (but not of foreign antigens)

Block TLR7 and TLR9 activation of DCs → inhibition of inflammatory cytokines
Protection from UV-induced cell damage and inflammation

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10
Q

Indications for hydroxychloroquine

A

generalized DLE (D), ECLE (D)

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11
Q

MOA of methotrexate

A

antimetabolite (cell cycle specific; folic acid antagonist) → alteration of DNA and RNA synthesis

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12
Q

Causes of Pemphigus

A
  1. Genetic factors
  2. UV light
  3. Drugs:
    1) sulfydril (penicillamine),
    2) amide (captopril, penicillins-also in H, cephalosporins),
    3) thiol
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13
Q

Th of Pemphigus

A
  1. Glucocorticoids
  2. azathioprine
  3. mycophenolate mofetil
  4. chlorambucil,
  5. chrysotherapy;

In selected cases: 1) dapsone, 2) tetracycline-niacinamide, 3) vitamin E, 4) ω3/ω6 fatty acids, 5) PTX; 6) rituximab: monoclonal anti-CD20 antibody; 7) IV human immunoglobulin; 8) plasmapheresis and imunoabsorption
In refractory cases: restricted diet trial
+ Sun avoidance

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14
Q

Drug induced PF - def

A

-self-cure
-due to pharmacologic acantholysis,

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15
Q

drug-triggered PF- def

A

=continues
-due to auto-antibody production

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15
Q

Concurrent autoimmune diseases (19%) in PF

A
  1. immune-mediated polyarthritis,
  2. KCS,
  3. immune-mediated thrombocytopenia
16
Q

Clinical peculiarities in H with PF

A

Urticaria may be a prodromal sign
Oedema of limbs and ventral abdomen: vasculitis, hypoalbuminemia

17
Q

IHC - immunofluoresecnce of PF

A
  1. Direct: IgG (IgG2, IgG4) > C3 > IgM, IgA;
  2. Indirect: IgG1, IgG4;
18
Q

polysulfated glycosaminoglycan- in wich disease we use

A

PF
Adequan 4.4 mg/kg IM or SC every 4 days (or less or more frequently as needed) as adjunctive treatment in refractory cases

19
Q

wich drugs we add to PF in horses

A

1) fatty acids,
2) PTX,
3) sun avoidance,
4) polysulfated glycosaminoglycan (Adequan)

20
Q

Causes of PV

A
  1. Idiopathic
  2. Drug-induced: thiabendazole (D), procainamide (D), phenytoin (D), sulfasalazine (D), polymyxin B (D)
20
Q

Targeted Ag in PV

A
  1. Dsg III : H, D (60%),
  2. Dsg I (150 kDa): D (40%), possible in H
21
Q

Antigens vs clinical subtype in PV

A
  1. Mucosal-dominant: dsg III
  2. Mucocutaneous: dsg III (mucosal lesions) + dsg I (skin lesions)
  3. Cutaneous: dsg I + dsg III (but the latter Abs are pathogenetically weak)
22
Q

What is c-Myc and in wich disease is important

A

-pro-oncogen
-up-regulation in lesional and perilesional skin and mucosae → prolonged basal cell proliferation → delayed strengthening of desmosomes

PV

23
Q

Specific clinical forms of PV

A
  1. Follicular PV: only hair follicles are affected; no mucosal or mucocutaneous lesions; truncal alopecia
  2. Nasal PV: only nasal planum (Dx from DLE, MMP and epitheliotropic lymphoma)
24
Q

Ag in PNP

A

1) Desmoplakin I/II,
2) Envoplakin,
3) Periplakin,
4) Dsg III,
5) BPAG-1

25
Q

Wich tumors are connected with PNP

A
  1. lymphoma,
  2. thymoma (also in C, H),
  3. sarcoma (spleen),
  4. mammary carcinoma,
  5. Sertoli cell tumour
26
Q

Clinical forms in humans P Veg

A
  1. Hallopeau type: pustules → proliferative wart-like lesions, plaques, oral lesions; benign course
  2. Neumann type: pustules, vesicles, bullae → widespread erosions, oral lesions; refractory to treatment
26
Q

P Veg is correlated with autoimmune

A

It represents panepidermal pustular pemphigus (some clinical differences; PPV does not have mucosal lesions
It is a more benign or abortive form of pemphigus vulgaris

27
Q

Targeted Ag in P veg

A

Dsg I (D)

28
Q

Clinical manifestations of P veg

A
  1. Vesicles, pustules
  2. Vegetations-papillomatous proliferations with pustules
  3. Mucosal lesions
29
Q

IHC- immunofluorescence of Pemphigus erythematosus

A

Direct: IgG > C3; epidermis and basement membrane (D, C)
Indirect: negative (D, C)
ANA: low titres in 50% (D); a positive test is necessary for the diagnosis

30
Q
A

MS (20 decks)

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