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2020 Vasculitis McGowan > Vasculitis DSA and CIS > Flashcards

Vasculitis DSA and CIS Flashcards

1
Q

What is Pregnancy or Neonatal Lupus?

A

Affects children born to mothers with

Anti Ro (SS-A) or La (SS-B)

1-2%

Maybe seen in Sjogren’s as well

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2
Q

What is the serology workup for Sjogren’s? Which markers are the most imporant ones and why?

A

(+) ANA

(+) Rheumatoid Factor (RF)

High ESR

Polyclonal Hypergammaglobulinemia

(+) Anti SSA/Ro

Presence may lead to newborn complete heart block

(+) Anti SSB/La

(never present without Ro)

Low C4 complement
Anemia of chronic disease

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3
Q

Pemphigus vulgaris, Goodpasture syndrome, Acute rheumatic fever, Insulin-resistant diabetes, and pernicious anemia are examples of what type of hyerpsensitivity?

A

Type II Hypersensitivity

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4
Q

Autoantibody specific for Rheumatoid Arthritis?

A

anti-CCP (cyclic citrullinated peptides)

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5
Q

Drugs to treat DM and PM

A
  • Glucocorticoids
  • Methotrexate (MTX)
  • Azathioprine
  • Cyclophosphamide
  • Cyclosporine
  • Intravenous immunoglobulin (IVIG)
  • Mycophenolate mofetil
  • Rituximab
  • Hydroxychloroquine
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6
Q

What types of antibodies do you look for in scleroderma serology?

A

Diffuse (dcSSc)

Anti-Scl70 aka Anti-DNA Topoisomerase I

Anti-RNA polymerase III

Limited (lcSSc) or CREST

Anti-centromere

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7
Q

What is the basis of an immune complex-mediated (type III) hypersensitivity reaction?

What type of antigens involved?

A
  • IgM and IgG antibodies bind antigens usually in circulation and the antigen-antibody complex deposits in vessel walls and induce inflammation (vasculitis)
  • Antigens can be exogenous and endogenous
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8
Q

How are self-reactive T cells able to undergo apoptosis in the process of peripheral tolerance?

A
  • Self-reactive T cells express pro-apoptotic member of Bcl family (Bim), w/o antiapoptotic members of the family like Bcl-2 or Bcl-x
  • If self-antigens engage antigen receptors of self-reactive T cells, FasL and Fas are co-expressed inducing extrinsic pathways of apoptosis
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9
Q

What is the treatment for Wegener’s Granulomatosis?

A

Quit smoking

Cyclophosphamide

High dose glucocorticoids

Rituximab

Methotrexate okay only if renal fxn is normal

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10
Q

How does Chronic Discoid Lupus Erythematosus present?

A
  • Skin manifestations mimicking SLE, rarely systemic involvement
  • Well-defined imflammatory skin plaques w/ edema, erythema, scaliness, follicular plugging
  • Localized, deep, and scarring
  • Usually only involving the face and scalp
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11
Q

Population most impacted by autoimmune diseases?

A

F > M

Minority populations are at increased risk of rheumatolgoic diseases

cANCA

pANCA

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12
Q

What is the morphology of the blood vessels like in patients with SLE?

How about in the chronic stages?

A
  • Acute necrotizing vasculitis involving capillaries, small arteries, and arterioles
  • Chronic stages = vessels undergo fibrous thickening with luminal narrowing
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13
Q

MSK complications as a result of Systematic Sclerosis

A

Carpal tunner syndrome

Tendon friction rubs

Fibrosis and adhesion of tendon sheaths

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14
Q

What are the salient feautes of Neonatal Lupus?

A

Transient

  • rashes
  • thrombocytonpenia
  • hemolytic anemia
  • arthritis

–> risk for Permanent complete heart block

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15
Q

What induces the pathologic lesions of immune complex disorders?

A

Complement-fixing Abs (IgG and IgM) and Abs that bind to leukocyte Fc receptors

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16
Q

Differences between primary and secondary Raynaud

A

Primary: benign, exeggerated response to cold, affects more females between 15-30yo. Nailfold capillaries are normal

Secondary: unilateral, mor severe –> ischemia. Nailfolds with irregular loops, dilated lumen, and areas of vascular ‘dropout’

Episodic: pallor (vasoconstriction) –> cyanosis (ischemia) –> erythema (reperfusion)

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17
Q

How are antigens restricted to peripheral tissues able to be expressed in the thymus during the process of central tolerance?

A

A protein called AIRE

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18
Q

What is ANA and what is it specific for?

A

Anti-nuclear Ab

NOT specific Indirect immunofluorescence

1:40 is normal, higher may be of clinical significance

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19
Q

Centromere Pattern

A

30-60 Specles in the nucleus

  • Localize to chromosomes in the metaphase
  • CREST, systematic sclerosis
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20
Q

What is the earliest histological finding in both the major and minor salivary glands in patients with Sjorgen Syndrome?

A

Periductal and perivascular lymphocytic infiltration

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21
Q

What plays a role in acceptance/tolerance of a fetus while inside the placenta?

A

CD4+ CD25+ FOXP3 Treg cells

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22
Q

Some fun facts about Lupus-Like Syndrome

aka

Drug-induced SLE

A

Presents with SLE like symptoms but it is not a Type III hypersensitivty rxn.

T-cell DNA demethylation in older folks (Type IV)

Positive for ANA

95% will test positive for Anti-histone Abs

Affects M and F equally (unlike SLE)

No oral mucosa, CNS, or kidney involvemnt

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23
Q

Differentail DDx for THROMBOSIS

A

APS (Antiphospholipid Ab Syndrome)

Protein C deficiency

Protein S deficiency

Anti-thrombin deficiency

Factor V Leiden deficiency

Heparin Induced thrombocytopenia

Sepsis

Systemic vasculitis

DIC (disseminated intravascular coagulation)

TTP (thrombotic thrombocytopenic purpura)

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24
Q

What can be used to monitor disease activity of Type III Hypersensitivity?

A
  • Complement proteins can be detected at site of injury, and during active phase of disease consumption of complement will lead to decrease in serum C3
  • Serum levels of C3 can be used to monitor the disease activity
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25
Q

Differentail DDx for COTTON WOOL spots

A
  • Hypertension
  • Diabetes
  • Immune-mediated

◦ SLE/APS

  • Ischemia
  • Embolic

◦ DVT

◦ Long bone fracture (white cell emboli)

◦ Traumatic pancreatitis (white cell emboli)

  • Infectious
  • Toxic
  • Radiation-induced
  • Neoplastic
  • Traumatic
  • Idiopathic
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26
Q

What group of people is most common to develop SLE?

A
  • Women
  • African American and Hispanic > White
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27
Q

What are the main clinical features of a patient presenting with SLE?

A
  • Butterfly rash over the face
  • Fever
  • Pain but no deformity in one or more peripheral joints (feet, ankles, knees, hips, fingers, wrists, elbows, shoulders)
  • Pleuritic chest pain
  • Photosensitivity
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28
Q

Mutations in the AIRE gene cause?

A

Autoimmune polyendocrinopathy

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29
Q

Venous and arterial thromboses, which may be associated with recurrent spontaneous miscarriages and focal cerebral or ocular ischemia is characteristic of?

A

Antiphospholipid antibody syndrome

  • Can be primary = by itself
  • Or secondary = in association with lupus
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30
Q

Differential DDx for DLE

A

Discoid lupus
Tinea infection (Ring worm)
Psoriasis
Morphea (Localized scleroderma)

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31
Q

How do deposited antibodies in fixed tissues, such as basement membranes and ECM cause injury due to inflammation?

A

1) Activate complement, generating by-products, including chemotactic agents (C5a), which brings polymorphonuclear leukocytes and monocytes to tisse
2) Activate anaphylatoxins (C3a and C5a), which increase vascular permeability

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32
Q

What sort of renal involvement is seen in Sjorgen Syndrome vs. SLE?

A
  • Defects of tubular function (renal tubular acidosis, uricosuria, and phosphaturia)
  • Glomerular lesions are extremely rare (like those seen in SLE)
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33
Q

List 6 scenarios/diseases that involve immune-complex mediated (type III) hypersensitivity?

A

1) Systemic lupus erythematous
2) Poststreptococcal glomerulonephritis
3) Polyarteritis nodosa
4) Reactive arthritis
5) Serum sickness
6) Arthus reaction (experimental)

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34
Q

Which organs are most commonly involved in Systemic Sclerosis (Scleroderma)?

A
  • Skin is most common with secondary Raynaud phenomenon
  • Raynaud is typically first sx in Caucasians
  • Skin pigmentation changes as the first sx are more common in African Americans than Raynaud
  • GI tract
  • Kidneys
  • Heart
  • Muscles
  • Lungs
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35
Q

Mutations in the FAS gene cause what disease?

A

Autoimmune Lymphoproliferative Syndrome (ALPS)

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36
Q

Homogeneous Staining Pattern

A

Entire nucleus is diffusely stained

  • Drug-induced SLE
  • Sjogren’s syndrome
  • SLE
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37
Q

What are 2 methods by which infections may induce autoimmunity?

A

1) Infections may upregulate the expression of co-stimulators on APCs; if these cells are presenting self-antigens result may be a breakdown of anergy and activation of self-reactive T cells
2) Molecular mimicry: microbe expresses Ag w/ same AA sequence as self-Ags

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38
Q

Occult malignancies in Dermatomyocytitis include…

A
  • Ovarian - Check transvaginal US, CT abd/pelvis with IV contrast, CA-125
  • Lung (CT > CXR)
  • Pancreatic (Abdominal CT with IV contrast or MRCP)
  • Stomach (EGD)
  • Colorectal (Colonoscopy)
  • Non-Hodgkin Lymphoma (CBC with diff, lymph node biopsy, bone marrow biopsy, imaging CT/MRI/PET)
  • Cervical (pap smear)
  • Prostate (digital rectal exam and PSA, biopsy)
  • Breast (mammogram)
  • Monoglonal gammopathies [multiple myeloma] serum protein electrophoresis
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39
Q

What do antibodies do in Myathenia gravis?

A

React with ACh receptors in the motor end plates of skeletal muscles and block (act as antagonists) neuromuscular transmission –> muscle weakness

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40
Q

Sjorgen Syndrome can occur as a primary form (sicca syndrome) or most commonly as a secondary dz to something else with systemic symptoms. Which disease is it most commonly associated with?

A
  • Rheumatoid arthritis
  • Can also be associated with SLE, polymyositis, scleroderma, vasculitis, mixed CT disease, or thyroiditis
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41
Q

What are some important features of Scleroderma?

A

No course altering tx

ONLY manage/limit organ sx and damage

Education: Quit smoking and warm clothing

Ca2+ channel blockers (Raynaud)

ACE inhibitors for HTN

Anti-refulx for esophegeal pathology

Glucocorticoids: only indicated for inflammatory myositis or pericarditis. High doses –> renal crisis

*Cyclophosphamide improves lung fxn and survival

_*Phosphodiesterase Type5 inhibito_r –> tx pulm HTN

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42
Q

Thromboangiitis Obliterans

aka

Buerger Disease

A

Medium vessels

Young males, <35yo

ONLY in smokers

Distal –> Proximal

Thrombosis –> Loss of digits –> hands/feet

Angiograph: corkscrew

TX: Quit smoking

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43
Q

What is the mechanism responsible for tissue injury in some forms of Glomerulonephritis and vascular rejection in organ grafts among others?

A

Antibody-mediated inflammation

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44
Q

Clinically antibody-mediated cell destruction and phagocytosis occur in what 4 situations?

A

1) Transfusion rxns: cells from incompatible donor react with and are opsonized by preformed Ab in host
2) Hemolytic disease of newborn (erythroblastosis fetalis): antigenic difference between mom and baby; IgG antierythrocyte Abs from mom cross placenta and destroy fetal red cells
3) Autoimmine hemolytic anemia, agranulocytosis, and thrombocytopenia: pt makes Abs to their own blood cells
4) Certain drug rxns: drug acts as a “hapten” by attaching to plasma membrane proteins of red cells and Abs are produced against the drug-protein complex

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45
Q

In type II hypersensitivities what are the mechanisms for which antibodies destroy cells?

A
  • Opsonization and phagocytosis: cells opsonized by IgG recognized by phagocyte Fc receptors; IgG and IgM on cell surfaces may also activate classical pathway
  • Antibody-dependent cellular cytotoxicity (ADCC): cells coated with IgG are killed by variety of effector cells, mainly NK and macrophages; cell lysis W/O phagocytosis
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46
Q

What are the presenting sx of inflammatory myopathies?

A

F>M

AA>Caucasians

Myalgias

Weakness, most often symmetrical proximal msucles (difficulty rising from chair or bathtub)

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47
Q

What environmental factors are involved in SLE?

A
  • UV light: may induce apoptosis in cells and alter DNA to become immunogenic
  • Gender bias: partly related to genes on the X chromosome
  • Drugs: such as hydralazine, procainamide, and D-penicillamine
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48
Q

What are the joints like in patients with SLE?

Opposite of what?

A
  • Non-erosive synovitis with little deformity
  • Opposite of RA
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49
Q

Kawasaki Disease

aka

Mucocutaenous lymph node syndrome

A

Medium vessel

Acute Febrile illness

Worldwide, but Japan more common

1) fever
2) lymphadenopathy
3) rash on hands and feet
4) strawberry tongue

Morbidity d/2 coronary involvement may happen years later

TX: IVIG and Aspirin (typically not given if child presents with virual-like sx, this is an exception…b/c KD is not viral)

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50
Q

Recommended preventitive measures in SLE

A
  • Reduce known risk factors assoicated with atherosclerosis
  • Quit smoking
  • Influenza vaccination - annually
  • Pneumococcal vaccination - 5yrs
  • Malignancy screening
  • Monitor bone dz with corticosteroid use (hips, knees, etc) and osteoporosis
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51
Q

Antibody-mediated (type II) hypersensitivity involve what specific antibodies and can target what antigens?

A
  • IgG and IgM
  • Can be autoantibodies: specific for normal cell or tissue Ags
  • Antibodies to exogenous Ags, such as chemical or microbial proteins
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52
Q

Diagnostic feautes of IBM?

A

Biopsy - endomyosial inflammation with rimmed vacuoles, and invastion of non-necrotic fibers

CK may be normal, or mildly elevated

Anti-cN1A antibodies

TREATMENT REFRACTORY version of myositis

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53
Q

What is the classic example of molecular mimicry?

Causes what?

A
  • Rheumatic heart disease; Abs against streptococcal proteins cross-react w/ myocardial proteins
  • Cause Myocarditis
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54
Q

How do you dx Dermamyositis?

A

Biopsy - perifascicular inlfammation and atrophy

Elevated CK and Aldolase

Anti-Jo-1, anit Mi2, and MDA5, andti Pl55/Pl40

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55
Q

What is Mikulicz syndrome?

Seen in what conditions?

A
  • Enlargement and fibrosis of salivary and lacrimal glands
  • Sjorgen syndrome and IgG4 related disease
56
Q

What antibodies are diagnostic for Sjogren syndrome?

A
  • anti-Ro/SS-A
  • anti-La/SS-B
57
Q

Pink-colored urine after a blood transfusion indicates?

A

Hemolysis of RBCs occuring due to antibody mediated activation of complement

58
Q

What is essential for the diagnosis of Sjorgen Syndrome?

A

Biopsy of the lip to examine minor salivary glands

59
Q

Systemic Sclerosis (Scleroderma) is characterized by excessive?

A

Fibrosis throughout the body

You may see:

  • thinckening and hardening of the skin
  • microangiopathy and fibrosis of the skin and visceral organs
  • Obliteration of eccerince sweat glands –> dry itchy skin
60
Q

Whats the skinny on APS?

A
  • Pt doens’t need to have SLE to have APS (primary), but 1/3 of SLE Pts will also have APS (secondary to SLE)
  • Three types of anti-phospholipid antibodies
    • RPR and VRDL –> false positive for syphillis
    • Lupus anticoagulatn - risk factor for venous and arterial thrombosis and miscarriage
    • Anti-cardiolipin - serium cofactor Beta2GPI ​​
  • Tx is anticoagulation therapy
61
Q

How does the clinical presentation of Inclusion Body Myocytitis differ from DM and PM?

A

Younger adults, >40-50

M>F

Caucasians > African American

Weakness is in fingers or quadriceps

62
Q

What cardiovascular system effects are present with SLE?

A
  • Symptomatic or asymptomatic pericardial involvement (50% of pt’s)
  • Myocarditis
  • Valvular abnormalities (mitral and aortic)
  • Valvular (Libman-Sack) endocarditis (see photo)
  • CAD (angina, MI) owing to coronary atherosclerosis
63
Q

Polymorphisms in the genes encoding the IL-2R (CD25) and IL-7 receptor α chains are associated with what 2 diseases?

A

MS

Type I diabetes

64
Q

What is the diagnostic work up for Systematic Sclerosis?

A

BLOOD PRESSUE heralding renal failure

Labs: ESR, ANA, Serology, UA

Radiographs: CXR, Barium swallow, Hand x-ray for resorption and calcinosis

Others: ECG, echo, right heart cath, PFT,

Skin biopsy?

65
Q

What drugs induce lupus syndrome?

A

Hydralazine

Procainamide

Isoniazid (INH)

Minocycline

D-penicillamine

TNF inhibitors used to tx RA

Quinidine

Chlorpromazine

Methyldopa

Sulfa antibiotics may cause flare

66
Q

What are the major causes of short- and long-term mortality in SLE?

A

Short-term:

  • Opportunitistic infections d/2 immune system bugged and
  • Kidney or CNS dzs d/2 primary dz progression

Long-term:

  • Accelerated atherosclerosis leading to MI, and
  • thromboembolitic events
67
Q

Most chronic inflammatory diseases are caused by abnormal and excessive?

A

TH1 and TH17 responses

*Psoriasis, MS, and some types of IBD

68
Q

Specific alleles of which HLA has been linked to production of anti-dsDNA, anti-Sm, and anti-phospholipid antibodies seen in SLE?

A

HLA-DQ

69
Q

What is a Non-atopic allergy?

A
  • Triggered by non-antigenic stimuli: temperature extremes and exercise
  • Does NOT involve TH2 cells or IgE
  • Thought to be mast cells that are abnormally sensitive to acitvation by nonimmune stimuli
70
Q

Systemic Anaphylaxis is characterized by?

What is the immediate response and is then followed by?

A
  • Vascular shock, widespread edema, and difficulty in breathing
  • Within minutes: itching, hives, and skin erythema
  • Followed by: striking contraction of bronchioles and respiratory distress
  • Laryngeal edema –> hoarsness and further compromises breathing
  • Vomiting, diarrhea, abdominal cramps, and laryngeal obstruction follow
71
Q

Henoch-Schonlein Purpura

A

Etiology: IgA Vasculitis

Demographics: Kids

1) Palpable purpura w/o thrombocytopenia
2) Abdominal pain
3) Arthralgia
4) Renal Diseas

DX: IgA deposits

TX: supportive care + steroids

72
Q

What are possible complications of Systematic Scleraderms on the Cardiovascular system?

A

Myocardial fibrosis

Cardiomyopathy

Pericarditis

Myocarditis

Pericardial effusion

Arrhythmia

73
Q

What is the most common and also the most severe pattern of glomerular disease seen in SLE?

Characteristics/morphology?

A
  • Diffuse lupus nephritis (Class IV)
  • >50% involvement of Glomeruli
  • Proliferation of epithelial cells —> cellular crescents that fill Bowmans space
  • Circumferential thickening of capillary wall, forming “wire loop” strucutres on light mircoscopy (see attached photo)
74
Q

What are the Clinical features of Systemic Sclerosis (Scleroderma)?

Who’s affected most often (gender and age)?

A
  • Female-to-male ratio of 3:1
  • More severe in blacks
  • Peak incidence in the 50-60 yo group
  • Distinctive features = cutaneous changes, skin thickening, Raynaud phenomenon
  • Dysphagia, respiratory difficulty, myocardial fibrosis, mild proteinuria
75
Q

Some patients with the limited form of Systemic Sclerosis (Scleroderma) also develop?

A

CREST syndrome

Cutaneous calcinosis

Raynaud phenomenon (ischemic ulceration may be present) often the first symptom

Esophageal dysmotility (GERD)

Sclerodactyly

Telangiectasia

Complication –> Pulmonary HTN

Good prognosis with indolent course

76
Q

Autoantibody associated with diffuse systemic scleroderma?

Limited systemic scleroderma?

A

Diffuse = anti-DNA topoisomerase I (anti-Scl 70)

Limited (CREST) = anti-centromere

77
Q

How may infections actually protect against some autoimmune diseases?

A

Promoting low levels of IL-2 production which is essential for maintaining Treg cells

78
Q

In addition to antibody testing (whic his a must) in SLE, what others labs do you need to conisder and why?

A

CBC (neutropenia, and thrombocytopenia)

ESR (red blood cell distruction)

Complement (downstream affects of autoimmune dz, expect C3 and C4, CH50 low)

Antiphospholipid Ab

  • Anti-cardiolipin Ab
  • Lupus anticoagulant

Urinanalysis (not sure why, but I’m assuming that with Type III deposition causing renal/glomerular damange you’d see protein in UA)

79
Q

What are the clinical features and presenting signs of a patient presenting with Sjorgen Syndrome?

Most common gender and age group affected?

A
  • Women between ages 50-60
  • Keratoconjunctivitis = Blurring of vision, burning, and itching, and thick secretions accumulate in the conjunctival sac
  • Xerostomia = Difficulty swallowing solids, decreased taste, cracks and fissures in mouth
  • Parotid gland enlargement
  • Epistaxis
  • Recurrent bronchitis and pneumonia
80
Q

Polyarteritis Nodosa

A

Medium vessels - Segmental

Hepatitis B Vrius (HBV)

NO LUNG INVOLVEMENT

General: fatigue, fever, weight loss

Skin: livedo, reticularis, nodules, ulcers, digital grangrene

Neuro: Peripheral neuropathy (80% will have)

GI: posprandial abdominal pain

Renal: renin-mediated HTN, renal infarct

81
Q

Behcet Syndrome

A

Variable vessel size

Demographic: East and Asia

Aneurysms

DVT

1) recurrent mouth ulcers

2) genital ulcers

3) eye inflammation

MSK: large join arthralgia

Neuro: MS-like sx

GI: ulcers throughout the tract (DDX Chron’s)

DX: H&P, HLA-B51 serology

TX: low dose corticosteroids

82
Q

Polymorphisms in the gene for NOD2 are associated with what disease?

A

Chron’s disease

83
Q

What are the two diagnostic antibodies in SLE?

A

Abs against dsDNA - correlates with dz progression

and

Abs for Smith (Sm) - will NOT correlate with dz progression

84
Q

Complement activation can lead to the formation of the MAC, which disrupts membrane integrity of cells by “drilling holes,” which cells is the MOA most effective against?

A

Cells that have thin walls —> Neisseria bacteria

85
Q

Systemic Sclerosis (Scleroderma) is characterized by what 3 things?

A

1) Chronic inflammation though to be result of autoimmunity
2) Widespread damage to small blood vessels
3) Progressive interstitial and perivascular fibrosis in the skin (early) and multiple organs (later)

86
Q

Renal complications in Systematic Sclearderma are due to what risk factors?

A

malignat HTN,

hemolytic anemia, and

progressive renal insufficency

87
Q

Type III Hypersensitivity preferentially involve what 3 organs/sites?

A
  • Kidney (glomerulonephritis)
  • Joints (arthritis)
  • Small blood vessels (vasculitis)

*Organs where blood is filtered at high pressure to form other fluids, like urine and synovial fluid.

88
Q

What organ systems are involved in SLE and how are they affected?

A

General: fatigue, fever, malaise, weight loss

Skin: butterfly rash, photosensitivity, vasculitis, alopecia, oral ulcers

Arthritis: inflammatry, symmetric, non-erosive

Blood: LAD, neutropenia, thrombocytopenia, splenomegaly, thrombosis

Cardio: Pericarditis*

  • chest pain worse with cough, also worse when supine, better with sitting or leaning forward
  • pericardial friction rub
  • Diffuse ST elevation of ECG
  • Libman-Sacks endocarditis
  • Pleuritis, myocarditis
  • Increased risk of MI due to accelerated atherosclerosis

Nephritis: no clinical signs, only histologic dx

GI: peritonitis, vascultitis

Neuro: organic brain syndromes, seizures, psychosis, cerebritis

89
Q

What leads to the fibrosis in Systemic Scleroderma?

A

Activation of fibroblasts by cytokines produced by T cells and alternatively activated macrophages (M2)

90
Q

How does Mixed CT disease typically present?

Good response to what tx?

A
  • Synovitis of the finger
  • Raynaud phenomenon and mild myositis
  • Renal involvement is modest
  • Good response to corticosterioid, in the short term
91
Q

Polymyalgia Rheumatica (PMR)

A

Associated with Giant Cell Arteritis (GCA)

Proximal severe symmetrical stiffness, soreness, muscle pain. Start in the am –> lasts ALL day long

Subjecive weakness with ADL as a result of pain

No objective muscle weakness

ONLY dx is elevated ESR and CRP

TX: glucocorticosteroids

92
Q

What is Sjorgren characterized by?

Result of?

A

SICCA Symptoms:

  • Dry eyes
  • dry mouth (xerostomia) with incrased incidence of candida infection,
  • vaginal dryness,
  • tracho-bronchial dryness
  • Parotid or other slaivary gland enlargement

keratoconjunctivitis SICCA: sensation of foreing body in eye

  • Schirmer test - measures tears secreted

All d/2 Immunologically mediated destruction of lacrimal and salivary glands

Strong Association with B-cell Non-Hodgkin lymphoma (MALT)

20-30% will have BOTH sicca + systemic sx

93
Q

What are the three categories of inflammatory myopathies?

A

Dermatomyositis

Polymyositis

Inclusion Body Myositis

94
Q

Nucleolar Pattern

A

Blobby staining of the nucleus

  • Abs against RNA
    • fibrillarin, RNA plymerase I and III, Th, PM-Scl and RNA helicase
  • Diffuce Systematic Sclerosis - most commonly
  • SLE
95
Q

Autoantibodies associated with Goodpasture Syndrome?

A

anti-basement membrane autoantibodies

anti-type IV collagen

–> affects small vessels, such as glomerular or pulmonary capillaries (death if not treated)

TX: plasmapheresis

96
Q

What are the major connective tissue diseases?

Aka “collagen vascular disorders”

A

SLE

Scleroderama (systematic sclerosis)

Sjogren Syndrome

Dermatomyositis (DM)

Polymyositis (PM)

Inclusion Body Myositis (IBM)

RA

97
Q

Differential DDx of PROXIMAL MUSCLE WEAKNESS

A

Inflammatory myopathies/myositis ◦ DM/PM/IBM

HYPOthyroidism - Still can see elevated CK

Hyperthyroidism - NormalCK

Cushing disease - NormalCK

Polymyalgia rheumatic (PMR) - Pain but NO weakness, CK normal

Neurological - Multiple sclerosis, Myasthenia gravis, Amyotrophic lateral sclerosis, Lambert-Eaton Syndrome

Vasculitides (distal and asymmetric) – Polyarteritisnodosa, Churg-Strauss,Granulomatosiswithpolyangiitis

Drugs/Medication Corticosteroids,alcohol,penicillamine,hydroxychloroquine,colchicine (long term), Zidovudine, HMG-CoA reductase inhibitors

98
Q

How do majority of patients with Systemic Sclerosis (Scleroderma) die?

A

- Renal failure –> may see hemolityc anemia during renal crisis

  • Cardiac failure
  • Pulmonary insufficiency
  • Intestinal malabsorption
99
Q

Treatment for DLE?

A

Photoprotection with topical anti-inflammatory agents and systematic antimalarial drugs

Tends to scar, early treatment can prevent scarring

100
Q

Injury in SLE is caused by?

What type of hypersensitivity?

A
  • Deposition of immune complexes and binding of antibodies to various cells and tissues

- Type III hypersensitivity

101
Q

What is IgG4 related disease characterized by?

A
  • Tissue infiltrates dominated by IgG4 anti-body producing plasma cells and lymphocytes (mainly T cells)

- Storiform fibrosis

- Obliterative phlebitis

  • Increased serum IgG4
102
Q

What disease often affects middle-aged and older men and is 1st characterized in autoimmune pancreatitis?

A

IgG4 Related Disease

103
Q

What is the major cause of death in patients with Systemic Sclerosis (Scleroderma)?

A

Pulmonary disease is the major cuase of mortality.

  • Interstitial lung disease in DIFFUSE variant with chronic cough, dyspnea and ‘velcro’ crackles
    • Dx: via pulm fxn test
  • Pulmonary artery hypertention (PAH) in LIMITED
    • Dx: right heart catheterization
  • There is also an increased incidence of bronchoalveolar carcinomal
104
Q

What is absolutely essential for Sjogren’s diagnosis?

A

Lip biopsy

Note: parotid gland biopsy is reserved for Pts with atypical presentation (e.g. unilateral enlargement - cancer?)

105
Q

Which autoantibody found in patients with Sjorgen syndrome is associated with early disease onset, longer disease duration, and extraglandular manifestations, such as diffuse pulmonary fibrosis, cutaneous vasculitis and nephritis?

A

SS-A

106
Q

How can Dermatomyositis be differentiated from Polymyositis?

A

Poly has no skin involvement

Different age group: 30-50yo

subacute (weeks to months)

107
Q

Patients with SLE that have the presence of antiphosphlipid antibodies will have increased?

These antibodies cause what kind of state?

A
  • Increased PTT
  • Hypercoagulable state (excessive clotting); leading to thrombosis
108
Q

Churg-Strauss Syndrome

aka

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

A

Small to medium vessels

Respiratory tracts

Asthma and eosinophilia are hallmarks of this dz

Three phases: allergic –> eosiniophilia tissue infiltration –> vasculitis with systematic necrotizing heart/lung/nerves/skin and palpable purpura

Tx: steroids, no smoking

DX: palpable purpura and Granulomatous Polyangitis

109
Q

What are the 3 immune-privileged sites?

A
  • Brain
  • Eyes
  • Testis
110
Q

Wegener’s Ganulomatosis

aka

Granulomatosis with Polyangiitis

A

Small vessel involvement

M>F

>40 yo

1) oropharynx (90%), saddle nose, obstruction

2) lungs - infiltrates, alveolar hemhorrage, cavitary lesions

3) kidneys

granulomatous inflammation with necrotizing vasculitis and segmental glomerulonephritis

111
Q

Important considerations in the treatment of Sjogren’s

A

AVOID atropinic drugs and decongestants

NO immunology drug has been proven to be helpful

Glucocorticoids are NOT effective for sicca sx

Supportive care and survaillance for mouth, dry eyes

Arthralgias may improve with hydroxychloroquine

112
Q

Diagnostic workup for Polyartheritis Nodosa

A

Biopsy - infiltration and destruction of vessles by inflammatory cells –> fibroid necrosis

NO granulomas

Angiogram: micro aneurysm

Serology: hardly every positive for cANCA

Check for HBsAg, ABsAb, HBeAg

Tx: glucocorticosteroids

113
Q

Speckled Staining Pattern

A

Numerous fine spots

  • UI RNP, Sm, and La antigents
  • Sjogren’s syndrome - more common
  • Mixed Connective Tissue Dz
  • Diffuse Systematic Sclerosis
  • SLE
114
Q

Immunofluorescence microscopy of the skin in patient with SLE will show?

Are these finding diagnostic?

A
  • Deposition of immunoglobulin and complement along dermoepidermal junction
  • May also be present in uninvolved skin
  • This finding is NOT diagnostic of SLE and is sometimes seen in scleroderma or dermatomyositis
115
Q

Treatment considerations for SLE

A

No sun (avoid and/or wear sunscreen)

NSAIDS - tame pain and inflammation

Steroids - calm immune system

Hydroxychloroquine - tx skin pigment and texture

116
Q

Mixed Connective Tissue disease is characterized serologically by high titers of antibodies to?

A

Ribonucleotide particle-containing U1 ribonucleoprotein (anti-U1-ribonucleotide)

117
Q

Some viruses like EBV and HIV cause __________which may result in the production of autoantibodies

A

Polyclonal B cell activation

118
Q

Glomerular lesions in SLE are the result of what?

A

Immune Complex Deposition

119
Q

Briefly describe the 3 phases of the pathogenesis of systemic immune complex disease (main players and time line of events).

A
  1. Formation of immune complex: intro of protein Ags triggers immune response resulting in formation of Abs, typically 7 days after injection of the protein. Secreted into blood where they react with Ag still present and form complexes
  2. Deposition of complex: Ag-Ab complex (medium sized/slight Ag excess = most pathogenic) deposit in tissues/organs
  3. Inflammation/tissue injury: acute inflammatory rxn, about 10 days after Ag was introduced producing fever, urticaria, arthralgia, lymphadenopathy, and proteinuria
120
Q

Thyroid complication of Systematic Sclerosis?

A

HYPOthyroidsim d/2 fibrosis

121
Q

What kind of complications does Systematic Sclerosis (diffused or limited) have on the GI system?

A

Entire GI can be affected

Malabsorption: Fat, protein, B12, VitD

Xerostomia

Esophagus

  • GERD
  • Dysphagia
  • Strictures
  • Barrett esophagus

Gastroparesis

Gastric antral vascular ectasi (GAVE syndrome)

Chronic diarrhea from bacterail overgrowth

Pseudo-obstruction

Primary biliary cirrhosis/cholangitis (anti-Mitochondrial Ab)

122
Q

What are the defining features of Dermatomyositis?

A

Bimodal age distribution 7-15 and 30-60

Proximal muscle weakness w/o sensory deficits

Gottron’s patches/papules (raised violaceous lesions on DIP, PIP and MCP)

Heliotrope rash with or w/o periorbital edema

Periungual erythema

V-neck erythema (Shawl sign)

Increased risk of MALIGNANCY

123
Q

Function of CTLA-4 and PD-1?

A
  • Structurally homologous to CD28 and act as inhibitory receptors
  • CTLA-4 has a higher affinity for B7 molecules on APC’s
  • Act to induce anergy in a lymphocyte upon recognizing a self antigen
124
Q

What do antibodies do in Graves disease?

A

Act as agonists on the TSH receptor of thyroid epithelial cells and stimulate the cells to make thyroid hormones —> hyperthyroidism

125
Q

What are the diagnostic features of Polymyelositis?

A

Endomysial inflammation with invasion of non-nectrotic muscle fibers

Elevated CK

Anti-Jo-1

126
Q

What other organs are involved in SLE?

A

CNS

Spleen (onion-skin lesions from smooth muscle cell hyperplasia)

Lungs (effusions), interstitial fibrosis and secondary pulmonary HTN

127
Q

What kind of lab results do you expect to see in inlfammatory myopathies?

A

Serum elevation of CK

CK is normal or only mildly elevated in IBM

ESR and CRP often normal

ECG and muscle biopsy have characteristic findings

derma features in DM

128
Q

Autoantibodies associated with drug-induced SLE?

A

anti-histone antibodies

129
Q

Which cytokine is essential for the maintenance of Treg cells?

A

IL-2

130
Q

What are the most common causes of death in patients with SLE?

A
  • Renal failure
  • Infections
  • CAD
131
Q

Which gene is said to be most frequently implicated in autoimmunity; encodes what?

Associated with what disorders?

A
  • PTPN22; encodes a protein tyrosine phosphatase
  • Type I DM, Rheumatoid arthritis, IBD
132
Q

What are signs of renal involvement in SLE?

A

Hematuria, proteinuria, red cell casts, nephrotic syndrome

133
Q

What occurs in the Arthus reaction?

How can it be produced experimentally and what is the end result?

A
  • Localized area of tissue necrosis resulting from acute immune complex vasculitis, usually elicited in skin
  • Experimentally by intracutaneous injection of Ag in a previously immunized animal that contains circulating Abs against the Ag.
  • As Ag diffuses into the vascular wall, binds preformed Ab and large immune complexes are formed locally. Precipitate in vessel walls and cause fibrinoid necrosis
134
Q

What organs are NOT affected in drug-induced SLE?

A

Renal

CNS

135
Q

Patients with Sjorgen Syndrome are at a high risk for developing?

A

Dominant B-cell clone and marginal zone lymphoma

136
Q

How is Subacute Cutaneous Lupus distinguished from Chronic Discoid LE?

A
  • Skin rash is widespread, superficial, and non-scarring
  • Most have mild systemic symptoms consistent with SLE
  • Strong association with antibodies to the SS-A antigen and w/ HLA-DR3 genotype

*Basically an intermeditate between SLE and Chronic Discoid LE

137
Q

ANA Patterns

A

Homogeneous - reflects Abs against chormatin, histones, and dsDNA

Speckled (very small, uniform) - most often seen, least specific. Reflects Abs against non-DNA nuclear constituents Sm, SS-A, SS-B, and ribonucleotides

Centromere (30-60 spots) - Pts with Systematic Sclerosis have Abs against centromeres

Nucleolar (few large blobs) - Antibodies for RNA, most often seen in Systematic Sclerosis

2020 Vasculitis McGowan (1 decks)

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