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OS4-MS/Derm/Rheum > Autoimmune Disorders - RA, Lupus, Scl, SS, Spondylo, etc. > Flashcards

Autoimmune Disorders - RA, Lupus, Scl, SS, Spondylo, etc. Flashcards

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Dermatology Board Prep flashcards
1
Q

If a pt is experiencing back pain related to RA, it is most likely to be isolated to which vertebrae?

A

C1, C2, C3
*in OA, the spine is much more heavily involved than in RA; and damage to the cervical spine usually occurs after several years of disease

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2
Q

What is rheumatoid arthritis?

A

A chronic multi-system disease mediated by auto-immune mechanisms. Characterized by persistent inflammatory synovitis (peripheral joints, symmetric distribution) which erodes cartilage and bone.
The disease has a variable course.

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3
Q

What constitutes the diagnosis of rheumatoid arthritis?

A

> =6 points on the classification scale, which includes points for the following categories:

  • Joint distribution (how many large and how many small joints involved)
  • serology (negative/low positive/high positive of RF and ACPA)
  • symptom duration (>6wks)
  • acute phase reactants (abnormal CRP and/or ESR)
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4
Q

Describe the characteristic laboratory features of rheumatoid arthritis.

A
  • Rheumatoid Factor (RF): usually IgM type Ab directed against the Fc portion of IgG; not specific for RA
  • Anti-CCP Ab: anti-cyclic-citrullinated peptide antibody produced by synovial B cells; more specific for RA
  • ANA: seen in 20% of RA pts
  • Abnormal CRP and/or ESR
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5
Q

Describe the characteristic systemic clinical features of rheumatoid arthritis.

A

fatigue, anorexia, weight loss, weakness, generalized aching and stiffness, low-grade fever

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6
Q

Describe the characteristic articular (local) clinical features of rheumatoid arthritis.

A
  • swelling, warmth, erythema, and pain in the affected joints
  • stiffest for at least 30min in the morning
  • insidious onset, can begin as intermittent
  • not usually migratory but appears in one joint (typically large peripheral joints like the knee), persists, and develops into persistent polyarthritis (characteristically involving wrist and proximal hand)
  • synovial fluid analysis reveals exudative yellow fluid, elevated WBCs/neutrophils, and reduced viscosity
  • weakened tissues and supporting structures such as ligaments, joint capsules
  • may lead to fibrosis of tissues including muscles
  • muscle atrophy from inflammation and disuse
  • joint deformities under normal forces of muscular traction
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7
Q

Describe the characteristic extra-articular clinical features of rheumatoid arthritis.

A

skin - rheumatoid nodules, vasculitis
ocular - keratoconjunctivitis sicca (dry eyes), xerostomia (dry mouth), episcleritis and scleritis (like skin nodules but on sclera) (due to lymphocytic infiltration of glands)
Cardiorespiratory - fibrosis, pulm nodules, pleuritis with pleural effusion (common), pericarditis, valve nodules
Neurologic - cervical myelopathy, carpal tunnel syndrome, ulnar tunnel syndrome (Guyon’s canal)

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8
Q

Summarize current thoughts on what factors contribute to development of rheumatoid arthritis.

A

Genetics (60%) and Environmental (rest). Gene-environment interactions may be multiplicative or additive in RA risk.
Genetics: there are >35 risk loci, with HLA-DRB1 (DR4) contributing ~30% genetic risk, other inflammatory genes (TNF-AIP3, STAT4, IL2RB) ~5% risk

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9
Q

Describe the role of HLA-DRB1/DR4 in the pathogenesis of RA.

A

HLA-DR4 selectively binds and presents antigen (arthritogenic peptide) to T cells, results in selection of autoreactive T cells in thymus

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10
Q

Describe the autoimmune nature of RA, including the role of antibodies, T cell cytokines, and MP/fibroblast cytokines.

A

Autoabs may include RF, ACPA which may attack joint antigens like type 2 collagen, or systemic antigens like glucose phosphate isomerase. These Abs contribute to synovitis by local complement activation.
T cell cytokines including IFN-gamma and IL-17 are present in low levels in RA synovium. T cells can contribute to synovial inflammation via antigen- independent mechanisms such as direct cell-cell contact with macrophages.
Macrophage and fibroblast cytokines are abundant in RA synovium. These include TNF, IL-1, IL-6, IL-8, IL-15, IL-18, GM-CSF, and IL-33 which serve to recruit inflammatory cells to the joint.

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11
Q

RA classically involves which joints?

A

Most characteristic is WRIST and PROXIMAL HAND followed by:
Shoulder
Elbow
Hip
Knee
* joint involvement tends to be symmetrical

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12
Q

Describe catastrophic antiphospholipid syndrome.

A

Multiple thromboses of medium and small arteries occurring over several days

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13
Q

What distinguishes drug induced Lupus from SLE?

A

No nephritis usually seen in Drug induced lupus.

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14
Q

Describe the histological manifestation of SLE in the skin.

A

Superficial and deep perivascular inflammation and mucin in the reticular dermis. Also seen is edema. Vasculitis with fibrinoid necrosis may also be present.

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15
Q

Describe the immunofluorescent manifestations of SLE in the skin.

A

Shows deposition of immunoglobulin and complement along the dermoepidermal junction. This is not diagnostic of SLE, and is also found in scleroderma and dermatomyositis.

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16
Q

You see moderate lupus on a kidney biopsy. What do you see that tells you it is lupus?

A

Wire-loop thickening of glomerular capillaries.

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17
Q

Describe the immunofluorescent pattern of lupus nephritis.

A

IgG deposits in a granular pattern

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18
Q

Describe the earliest pathological changes in Systemic Sclerosis, which also persist throughout the disease.

A

changes in endothelial cell function with increased apoptosis, upregulation of MHC class II and ICAM-1 expression on endothelial cells.

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19
Q

Describe a serious GI complication of SSc.

A

Gut dismotility* and telangectasias. Upper GI dysmotility more common. Lower dysmotility has bad prognosis.

*Collagen replacement of vascular and enteric smooth muscle results in hypomotility, lumen dilatation, tensile rigidity and eventual loss of organ functions.

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20
Q

What two conditions contribute to the majority of deaths in SSc?

A

Interstitial lung disease and PAH together account for 60% of mortality in pts with SSc.

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21
Q

Describe linear scleroderma.

A

Linear scleroderma is a limited cutaneous manifestation of SSc (not systemic). that presents as a linear streak that crosses dermatomes and is associated with atrophy of muscle, underlying bone and rarely the brain called “ en coup de sabre”.

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22
Q

Mixed connective tissue disease is most commonly associated with antibodies to:

A

U1-RNP

In general, this Ab predicts lack of severe CNS and renal involvement.

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23
Q

The absence of this condition, highly typical of MCTD, would suggest the need to consider another Dx.

A

Raynaud’s phenomenon

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24
Q

25% of MCTD pts develop this type of renal involvement:

A

Membranous glomerulopathy

Proliferative glomerulonephritis is uncommon in MCTD.

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25
Q

Describe the commonest CNS problems seen in MCTD.

A

trigeminal neuralgia & sensorineural hearing loss

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26
Q

What is the commonest cause of death in MCTD pts?

A

PAH

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27
Q

Describe the histology of SSc.

A

**Dead giveaway: Loss of Dermal-epidermal papillary projections (rete pegs). Dermal-epidermal interface is a straight line. This is due to underlying excessive deposition of collagen, deep fibrosis, and perivascular lymphohistiocytic infiltration. Also, loss of hair follicles and glands.

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28
Q

Describe the histological appearance of a renal glomerulus in SSc.

A

Shrunken, lacking inflammatory cells or proliferative changes. Also seen histologically will be interstitial fibrosis presenting as increases spacing between renal tubules in transverse section.

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29
Q

Describe the clinical hallmarks of Sjogren’s syndrome (SS).

A

xerostomia, keratoconjunctivitis sicca, and parotid gland swelling.

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30
Q

What Abs are we going to test for when Dxing Sjogren’s?

A

serum ANAs including: anti-Ro/SS-A and anti-La/SS-B

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31
Q

Autoimmune diseases are characteristically found more commonly in men or women?

A

Women.

32
Q

Sjogren’s syndrome (SS) carries a 44-fold risk of acquisition of this malignancy:

A

Non-Hodgkin’s Lymphoma, MALT lymphomas
Risk factors for NHL include:
length of time with disease, low C4, type II cryoglobulins, anemia, lymphocytopenia, hypergammaglobulinemia, cutaneous vasculitis (palpable purpura), lymphadenopathy, splenomegaly, parotid gland enlargement, & peripheral neuropathy.

33
Q

How does SS present renaly?

A

Infrequently: tubular interstitial nephritis, type I renal tubular acidosis, glomerulonephritis, and nephrogenic diabetes insipidus.

34
Q

1/3 of pts with Sjogren’s experience this GI symptom:

A

esophageal dysmotility,

35
Q

SS mimics this neurological disease on presentation:

A

multiple sclerosis

36
Q

Describe the pulmonary manifestations of SS.

A

Xerotrachea, xerobronchitis, nonspecific interstitial pneumonitis, lymphocytic interstitial pneumonitis, usual interstitial pneumonitis, bronchiolitis, & lymphoma.

37
Q

Describe the laboratory findings (Ab screen) or SS.

A

75-95% (+) for rheumatoid factor
85% (+) for ANAs
1/3-1/2 (+) for anti-Rho/SS-A and anti-La/SS-B
5-10% have low blood levels of C3 & C4
5-15% have leukopenia or thrombocytopenia
5-10% with primary SS have Type II or III cryoglobulinemia or monoclonal gammopathy

38
Q

Describe the two types of SS.

A

Primary:
Chronic autoimmune inflammatory disorder
Lymphocytic infiltration and proliferation
destroys exocrine glands
Infiltrates other organs to a variable extent
Malignant transformation possible

Secondary:
A complication of another autoimmune connective tissue disease
Commonly seen in SLE and RA

39
Q

Describe the histopathology or SS.

A

mononuclear inflammatory infiltrates, interstitial fibrosis. Clusters of lymphocytes.

40
Q

Is rheumatoid factor present in the spondyloarthropathies?

A

No, rheumatoid factor is ABSENT in the “seronegative” spondyloarthropathies

41
Q

Describe the HLA haplotype association found in spondyloarthropathies.

A

HLA-B27 very commonly found in pts w/ spondyloarthropathies. It is an MHC class I molecule that binds antigenic peptides and presents them to CD8+ T cells.

42
Q

Is arthritis of the spondyloarthropathies found predominantly in the upper or lower extremities?

A

Lower

43
Q

Describe the frequency of HLA-B27 in pts with ankylosing spondylitis compared to the general population.

A

Pts with AS: 90% have HLA-B27

Gen Pop: 8% have HLA-B27

44
Q

Describe the epidemiology of Ankylosing Spondylitis.

A

Age: adolescence - 35 yo
Sex: 3x likelihood in males

45
Q

Describe the pathology of ankylosing spondylitis.

A

Inflammatory cell infiltrate
Synovial inflammation similar to RA
TNF-a excess- pts respond well to humira (adalimumab)

Unknown cause.

46
Q

Describe the clinical features of ankylosing spondylitis.

A

Insidious onset
Chronic low back pain/stiffness
Symptoms are worse in morning and improve w/ exercise
Symptoms gradually ascend up the spine

47
Q

Describe the extra-articular features of ankylosing spondylitis.

A

Anterior uveitis 25-30%
Cardiac: aortic regurg, heart block, pericarditis, MI
Pulm: apical lung fibrosis, thoracic cage restriction due to ankylosis of costovertebral joints

48
Q

Describe the “classic triad” associated with reactive arthritis.

A

Arthritis, urethritis, conjunctivitis. Triad not always complete.
Reactive arthritis is an inflammatory arthritis following an infectious process

49
Q

What are the 4 organisms classically associated with Reactive Arthritis?

A

Chlamydia, Salmonella, shigella, C. difficile

50
Q

Describe the epidemiology of reactive arthritis.

A

Uncommon (3-5/100,000)
Mostly males
75% HLA-B27 (+)
ReA more common in HIV/AIDS- more severe, resistant to therapy

51
Q

Describe the clinical features of reactive arthritis.

A

Asymmetric oligoarthritis
Dactylitis
Axial disease: sacroiliitis and spondylitis
Enthesitis (inflamm of tendon-bone interface) —pathognomonic for reactive arthritis (Reiter’s)
Uretritis
Oral ulcers
Conjunctivitis
circinate balanitis- shit builds up around glans of penis

52
Q

Describe the laboratory findings of reactive arthritis.

A

Inflammatory markers (ESR, CRP)
Culture is usually negative
Consider HIV
HLA-B27

53
Q

Arthritis mutilans and “sausage fingers” are condition characteristics of:

A

Psoriatic arthritis. Digits contract and “telescope” because bones are being chewed up by the arthritis.

54
Q

What feature of psoriatic arthritis helps distinguish it from RA?

A

increased vascularity and the presence of neutrophils in synovial tissues in psoriatic arthritis.

55
Q

Describe the immunopathology found in psoriatic arthritis.

A

Elevated plasma levels of immunoglobulins
T cells express: HLA-DR, receptors for IL-2 and adhesion molecules. T cells secrete IL-6 and other proinflammatory cytokines.
Fibroblasts from skin and synovium proliferate and secrete IL-1 beta, IL-6, and PDGF.

This is all similar to RA
However, more TNF-a, IL-1B, IL-2, IL-10, and IFN-g
No pannus like in RA, but high vascularity
Also increased levels of IL-18 that contributes to angiogenesis and mononuclear cell recruitment.

56
Q

Describe tx of psoriatic arthritis.

A

Infliximab or adalimumab

57
Q

Describe the pathophysiology of enteropathic arthritis.

A

Gut wall is leaky to microbes. Microbes get into circulation, and interact with lymphocytes, cause autoimmunity via molecular mimicry and damaging circulating immune complexes.

58
Q

Inflammatory arthritis is associated with these 3 inflammatory gut pathologies.

A

Crohn’s Disease
Ulcerative colitis
Whipple’s disease (rare)

Usually occurs after onset of GI disease, but can occur before

59
Q

Describe the genetic haplotypes found in individuals with celiac disease.

A

HLA-DQ2 & HLA-DQ8

60
Q

Describe the common symptoms of pts with celiac disease.

A

Diarrhea, IBS, fatigue, headache, arthralgias. Also seen, less commonly: Type I diabetes, anemia, osteoporosis, neuropathies, and joint symptoms.

61
Q

How do you Dx celiac disease? What is Tx?

A

Small bowel biopsy, presence of serum IgA antitissue transglutaninase, and IgA antiedomysial abs.
Tx: gluten free diet.
Bonus: there is an association (10% of IgA deficients) between celiac disease and selective IgA deficiency.

62
Q

What is rheumatoid factor?

A

An IgM ab against IgG

63
Q

MTX is a primary tx modality for RA. List its major complications.

A

Pulm interstitial fibrosis

macrocytic anema with hypersegmented neutrophils

64
Q

Which Ab has 100% specificity for Lupus?

A

Anti-Sm

65
Q

A pt has anti-dsDNA. They have lupus (more than likely) and what other pathological condition?

A

Nephritis

66
Q

A negative side effect of this class of drugs used in the treatment of RA is a lupus-like syndrome and also can produce a lab result showing dsDNA.

A

Anti-TNF-a mabs

67
Q

Methotrexate is thought to accelerate the formation of this pathological feature of advanced RA.

A

subcutaneous nodules of pallisading histiocytes. Usually present as non-tender, moveable bumps on the elbow or just distal to it along the ulna.

68
Q

Clinicians are reluctant to make a Dx of SLE without this antibody titer being positive:

A

ANA is highly sensitive for SLE but not very specific. Pt doesn’t have (+) ANA titer, prob doesn’t have Lupus.

69
Q

In a pt w/ systemic sclerosis, a prolonged rise in BP from their baseline would suggest:

A

Renal capillary pathology (hypertensive renal crisis) necessitating starting the pt on ACE-Is.

70
Q

Which Ab is found in Systemic Sclerosis (SSc) that is associated with hypertensive renal crisis, a complication of SS?

A

Anti RNA polymerase-3

71
Q

Which Ab is found in Systemic Sclerosis (SSc) that is associated with limited cutaneous disease, a complication of SS?

A

Anti centromere ab

72
Q

Which Ab is found in Systemic Sclerosis (SSc) that is associated with pts with antisynthetase syndrome, a subset of the inflammatory myopathies associated with SS?

A

Anti Jo1

73
Q

This Ab is found in a subset of systemic sclerosis pts suffering from dermatomyositis

A

Anti Mi-2

74
Q

This Ab is associated with diffuse scleroderma and the presence of interstitial lung disease.

A

Anti SCL-70

75
Q

The Dx of Sjogren’s Syndrome lies in these 3 general tests:

A

serologic (anti-SSA, anti-SSB, ANA, RF)
ophthalmologic (schirmer test)
oral signs (labial salivary gland biopsy)

76
Q

What is the most common pulmonary manifestation seen in Sjogren’s Syndrome?

A

Nonspecific interstitial pneumonia (NSIP)

Remember, PAH, is seen in SSc and MCTD, but it’s occurrence in SS is rare.

OS4-MS/Derm/Rheum (10 decks)

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