Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Clin Med semester 2 > Rheumatology-Part 3 > Flashcards

Rheumatology-Part 3 Flashcards

1
Q

JIA: What does JIA stand for?

A

Juvenile Idiopathic Arthritis

another name is Juvenile Rheumatoid Arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

JIA: Describe.

A

A spectrum of chronic arthritides in patients who are < 16 years old that involve AT LEAST 1 joint and have LASTED FOR AT LEAST 6 WEEKS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

JIA: What are the multiple subtypes?

A
  • Oligoarticular
  • Polyarticular
  • – Seropositive
  • – Seronegative
  • Systemic onset
  • Psoriatic
  • Enthesitis-related
  • Undifferentiated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

JIA: Peak ages?

-MC gender?

A
  • About 1/1000 children in US
  • Female > male 2:1-3:1
  • Peaks noted between 1-3 years (mostly girls) and 8-10 years
  • Less common in African-American and Asian, more common in whites
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

JIA - Pathophysiology: Genetic component?

A

Multiple HLA loci are associated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

JIA - Pathophysiology: Main mechanism of disease and S/Sx?

A
  • *** Persistent joint swelling that comes from synovial fluid increase (with inflammatory cells) + expansion of pannus
  • -> joint damage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Oligoarticular JIA: AKA?

A

Pauciarticular JRA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Oligoarticular JIA: Symmetric or asymmetric joint involvement?

A

** asymmetric

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Oligoarticular JIA: How many joints are typically involved?

A

** ≤4 joints involved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Oligoarticular JIA: What joint(s) are most commonly involved?

A
  • *knees MC involved

- Fingers, wrists, elbows, ankles, toes, also common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Oligoarticular JIA: Serology findings?

A

60% ANA positive. ESR/CRP might be mildly↑

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

“a toddler girl who’s parents noticed she started walking funny”

A

think Oligoarticular JIA !!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Oligoarticular JIA: What extra-articular condition(s) is/are also associated with patients ?

A
    • increased risk of uveitis
  • Esp. those with positive ANA
  • **All must have regular slit-lamp exams until the age of 18
  • **Can have serious complications, including blindness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Polyarticular JIA: How many joints are affected at the onset?

A

5 or more joints affected at onset

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Polyarticular JIA: Labs?

A
  • ESR (usually very elevated)
  • CRP (normal or elevated
  • Mild anemia
  • RF (positive or negative
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Polyarticular JIA: Seropositive cases resembles what other disease process?

A
    • resembles the adult version of RA
  • More likely to be aggressive, erosive disease

-Extraarticular: rheumatoid nodules, Felty syndrome, vasculitis, lung disease can occur.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Polarticular JIA: demographic and MC involved joints

A
  • Usually teenage girls

- Symmetric arthritis, usually the small joints of hands and feet

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Polyarticular JIA: What joints are typically involved in seronegative cases?
-demographic?

A

Usually larger joints: knees, ankles, wrists

-younger children, girls»boys

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Polyarticular JIA: articular involvement?

A

-**NO extraarticular features

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

SOJIA: What does this stand for?

A

Systemic Onset JIA aka Still’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

SOJIA: demographic

A

any age, boys=girls

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

SOJIA: sx

A
  • **Daily quotidian fever (child spikes a fever and it spontaneously goes back to normal)
  • Salmon-colored evanescent macular rash occurs with the fever–> Usually trunk, extremities
  • they look sick
  • Can have hepatomegaly, splenomegaly, LAD, serositis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

SOJIA: labs

A

High leukocytosis, very high ESR, elevated ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

SOJIA: complications

A

Pericarditis or MAS (macrophage activation syndrome) can be fatal complications

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Psoriatic Arthritis=

A
  • Arthritis in at least 1 joint

- -Often small or medium joints, asymmetric

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Psoriatic Arthritis: Sx

A
  • *DIP synovitis
  • *Dactylitis
  • *Nail pitting
  • *Psoriatic rash or FH of psoriasis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Psoriatic Arthritis is associated with acute ______ _____

A

**acute anterior uveitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Psoriatic Arthritis: labs

-what increases a Pt’s risk for uveitis?

A
  • Usually ↑ESR/CRP
  • Pts with positive ANA (30-60%) also at risk for uveitis
  • -Need regular slit lamp exams like those with oligoarticular JIA
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Enthesitis-related JIA: sx

A
  • ***Sacroiliac tenderness
  • Males 8-12. Boys>girls
  • Includes those with juvenile-onset spondylitis, reactive arthritis, and arthritis associated with inflammatory bowel disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Enthesitis-related JIA: labs

A
  • **Positive HLA-B27

- ESR and CRP frequently ↑

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Enthesitis-related JIA can be associated with ______ ______

A

anterior uveitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

JIA: DDX:

A 
Septic joint
Lyme disease
Viral infection
Reactive arthritis
Malignancy (leukemia, bone tumor)
SLE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

JIA: how to make a diagnosis

A
  • **Clinically diagnosed
  • No specific lab or imaging test to definitively confirm diagnosis, though they can guide you in the right direction.
  • Exclude other possibilities
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

JIA: primary goal of tx?

A

early tx is the goal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

JIA: tx

-who should be involved?

A

Have a pediatric rheumatologist involved

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

JIA: Tx

A

-**NSAIDs and intraarticular joint injections are mainstays of therapy–> Ibuprofen, naproxen, meloxicam, Intraarticular triamcinolone acetate

  • Those with SOJIA might need a steroid initially for symptom control
  • **Some may need a DMARD
  • -Methotrexate drug of choice

-Biologics: Infliximab, etanercept, adalimumab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

JIA tx:

-which Pts should be aggressively treated?

A

Aggressively treat those who with seropositive polyarticular JIA ASAP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

JIA: Prognosis/referral

A
  • Up to 50% can have continued symptoms into adulthood
  • Early detection and treatment has improved outcomes, deformity, and disability
  • Possible complications: growth retardation/short stature, leg length discrepancy, blindness from uveitis
  • **Out of all categories, those with SOJIA have the highest mortality
  • Referral to pediatric rheumatologist, social worker
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q 
Systemic Sclerosis (Scleroderma)
-describe this condtion
A

=Chronic, systemic autoimmune disorder with fibrosis of the skin and other organs
-there are 2 forms

(scleroderma= literally means “hard skin”– this is exactly what’s happening to the Pt)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Systemic Sclerosis (Scleroderma): 2 forms

A

–Limited (includes CREST syndrome) (face, distal to knees/elbows) What does CREST stand for? (Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, sclerodactyly, Telangiectasia)

–Diffuse

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Systemic Sclerosis: epidemiology/risk factors

A
  • 20 cases/1,000,000 yearly
  • Women > men
  • Peak age 30-50
  • Higher incidence in African Americans–>Earlier disease onset & More likely to have diffuse disease
  • 100x more common in Choctaw Native Americans in OK
  • Higher risk for those with 1st degree relatives with the disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Systemic Sclerosis: Pathophys

A
  • Cause or exact genetic factors not known: Infectious agents, drugs, environmental exposures?
  • **“Widespread proliferative/obliterative vasculopathy of small & medium arteries and capillary rarefaction” is the hallmark
    1. Vascular injury + activation
    1. Hypertrophy of the intima
    1. Excessive deposition of fibrin can occlude the lumen–> lose vascular supply–>tissue hypoxia

Fibroblasts overproduce extracellular matrix –> increased collagen deposition in the skin. Collagen cross-linking–>skin tightening

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Systemic Sclerosis: clinical manifestations

A
  • Raynaud phenomenon usually is the first sign

- May have constitutional Sx early in diffuse disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Systemic Sclerosis: skin manifestations?

A
  • Initially, edema + pruritis
  • Later, thick, hide-like skin
  • Hyperpigmentation or depigmentation
  • Digital ulcers
  • Pitting of fingertips
  • Loss of appendicular hair
  • Telangiectasias
  • Calcinosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Systemic Sclerosis: GI Sx (think fruity stomach)

A
  • Dysphagia, reflux symptoms, hypomotility

- Vascular ectasia in stomach antrum “watermelon stomach”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Systemic Sclerosis: Pulmonary Sx

A
  • Pulmonary fibrosis

- Pulmonary vascular disease –>pHTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Systemic Sclerosis: Cardiac Sx

A
  • Pericarditis
  • Heart block
  • Myocardial fibrosis
  • Heart failure (esp. diastolic)
  • Arrhythmia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Systemic Sclerosis: other clinical manifestations?

A

sicca syndrome (An autoimmune disease, also known as Sjogren syndrome, that classically combines dry eyes, dry mouth, and another disease of connective tissue such as rheumatoid arthritis (most common),)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Systemic Sclerosis: Renal Sx

A

-***Scleroderma renal crisis (SRC)–> Sudden onset of malignant HTN that can lead to renal failure + death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Systemic Sclerosis:

-Pt’s have a 3x risk for ________ disease

A

thromboembolic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Systemic sclerosis: MSK sx

A
  • Joint pain, contractures, immobility

- Tendon friction rubs

52
Q

Systemic Sclerosis: neuromuscular sx

A
  • Neuropathies

- Myopathy/myositis

53
Q

Systemic Sclerosis: GU

A
  • Erectile dysfunction

- Female sexual dysfunction (decreased vaginal lubrication, constriction of introitus)

54
Q

Describe CREST syndrome–Systemic Sclerosis (Scleroderma)

A
  • Calcinosis cutis (Ca 2+ deposits in the skin)
  • Raynaud phenomenon (white/or red discoloration and pain to fingertips)
  • Esophageal dysmotility
  • Sclerodactyly (thickening & tightness of the skin on fingertips and toes
  • Telangiectasia (spidery red spots on skin)
55
Q

Systemic Sclerosis: Labs?

which lab is usually positive?**

A
  • **ANA usually positive (>95%)
  • **Anti-SCL-70 positive in 1/3 with diffuse disease and 20% with CREST–> Poor prognosis

-Anti-centromere antibodies–>
Associated specifically with limited skin involvement, pHTN, & digital ischemia

  • Anti-RNA polymerase III antibody–> In those with diffuse disease, associated with ↑ risk SRC
  • May have mild anemia
  • In SRC, peripheral smear shows microangiopathic hemolytic anemia.
  • Proteinuria with renal involvement
56
Q

Systemic Sclerosis: imaging tests

A
  • High resolution CT best to detect lung changes, especially early on
  • Screen at baseline with PFTs, then repeat Q4-12 months, depending on symptoms
  • Could check echo to monitor for pHTN
  • If longstanding reflux, EGD to evaluate for Barrett’s
57
Q

Systemic Sclerosis: DDx

A 
SLE
Diabetic cheiropathy
Scleredema
Eosinophilic fasciitis
RA
Primary Raynaud phenomenon
Amyloidosis
Chronic graft vs host
Nephrogenic systemic fibrosis
58
Q

Systemic Sclerosis: general tx for skin involvement?

-tx for raynaud phenomenon?

A
  • No proven disease-modifying drug to treat scleroderma
  • **tx is targeted to organs affected
  • Diffuse skin involvement and/or systemic involvement
  • -**Immune process targeted: glucocorticoids, methotrexate, mycophenolate mofetil (MMF), cyclophosphamide, IVIG
  • Tx of Raynaud phenomenon:
  • -Calcium channel blockers: Long-acting nifedipine 30-180 mg daily
  • Sildenafil 50 mg PO BID
  • Exposure precautions
59
Q

Systemic sclerosis:

-Tx of GI sx?

A
  • Prokinetic agents: metoclopramide, erythromycin
  • Malabsorption: treat small bowel bacterial overgrowth with abx

Reflux: lifestyle modification, PPIs, H2 blockers

60
Q

Systemic sclerosis:

  • Tx of pHTN
  • Tx of ILD?
A
  • pHTN: Don’t smoke. O2, diuretic, digoxin, as indicated. Sildenafil or prostaglandins may be used
  • ILD: Immunosuppresants often used: cyclophosphamide, MMF.
61
Q

Systemic sclerosis:

tx of Renal sx?

A

SRC (scleroderma renal crisis): ACEI (captopril) to return to baseline BP. May require dialysis or transplant.

62
Q

Systemic Sclerosis:

  • tx of cardiac Sx?
  • tx of Erectile dysfunction?
A

cardiac: Treat as you would in a patient without scleroderma

- ED: On-demand pills usually not effective, use sildenafil (Viagra), tadalafil (Cialis) better

63
Q

ACR (american college of rheum.) diagnostic criteria for Systemic sclerosis (scleroderma): a score of ___ or above is considered definitive scleroderma

A

9 or above

64
Q

Scleroderma Prognosis:

  • what does it depend on?
  • _____ disease is the leading cause of mortality
  • worse prognosis?
A
  • depends on organ involvement
  • **Lung disease= leading cause of mortality
  • -Advanced CHF and CKD also common causes

Worse prognosis: diffuse disease, males, pulmonary involvement, SRC, cardiac involvement

-5 year survival
Limited: 90%
Diffuse: 70-80%

-May be linked with breast and lung cancer

65
Q

Scleroderma Pts should be referred to?

A

rheumatology, and other specialists depending on organ involvement

66
Q

Systemic Lupus Erythematosus (SLE)=

MC known for which 3 Sx?**

A

=Chronic inflammatory disease with autoantibodies and varying presentations that can affect every organ
–>Most commonly known for **fatigue, joint pain, and a rash

67
Q

SLE: risk factors/ epidemiology

A

-Highest prevalence in **childbearing females. W:M 10-15:1 –>Use of estrogen-containing OCPs, early menarche, or giving estrogen to postmenopausal women ↑ risk

  • Black, Hispanic, Asian>Caucasian
  • MC in urban areas
  • Genetic component: MHC and genes coding for the complement pathway
  • Smoking is a risk factor
68
Q

SLE: pathophys

A
  • Exact etiology unclear
  • Lupus autoantibodies often target intracellular particles that have both nucleic acid and nucleic-acid binding proteins
  • Antibodies bind to targets –>activate inflammatory pathway –> immune complexes and complement are deposited in organs, esp. skin, kidneys, and heart valves
69
Q

SLE: constitutional Sx

A

Fever, fatigue anorexia, malaise, weight loss

70
Q

Describe the Sx associated with Acute Cutaneous Erythemeatous (ACLE)

A
  • -**Localized: malar “butterfly” rash
  • -**Spares nasolabial folds
  • -**Often triggered by sun exposure
  • -May be scaly, indurated
  • -Generalized: maculopapular lesions in a photosensitive distribution (any part of body)
71
Q

Subacute cutaneous lupus erythematosus (SCLE)

A
  • -**MOST photosensitive (sensitive to sun) of all SLE rashes
  • -Scaly, erythematous papules
72
Q

Describe Chronic cutaneous lupus erythematosus (CCLE) (

A
  • *Discoid lupus most common subtype:
  • -Raised, erythematous plaques with a scale. Raised erythematous ring around the lesion–> Leads to scarring, skin atrophy
  • Lupus panniculitis (=persistent, tender and hard nodules localized on the face, arms, shoulders, breast and buttocks)
  • Alopecia
  • Plaques or psoriasiform lesions
  • Nail fold infarcts
  • Periungual erythema
  • Livedo reticularis

Mucosal ulcerations:

  • Esp. during exacerbations
  • Usually not painful
  • Most often on hard palate
73
Q

SLE: MSK manifestations

A
  • Arthralgia and nonerosive arthritis extremely common
  • Early manifestation of disease
  • PIP, MCP, wrists, knees
74
Q

SLE: Renal Sx

A
  • **Commonly involved
  • ->Poor prognostic indicator
  • ->There are several types of **lupus glomerulonephritis
75
Q

SLE: Vascular Sx

A
  • Raynaud phenomenon
  • Vasculitis (vessels of all size, but small most common)
  • Thromboembolism, esp. with those with antiphospholipid antibodies
76
Q

SLE: cardiac sx

A
  • Pericarditis most common
  • Increased risk for CAD
  • Arrhythmias, MR from atypical verrucous endocarditis (Libman-Sacks)
77
Q

SLE: lung sx

A
  • Pleuritis most common

- Pleural effusion, ILD, pneumonitis, pHTN, alveolar hemorrhage

78
Q

SLE: GI sx

A
  • Dysphagia most common
  • PUD, intestinal pseudo-obstruction, protein-losing enteropathy, mesenteric vasculitis, acute pancreatitis, peritonitis, perforation
79
Q

SLE: eye sx

A
  • **Keratoconjunctivitis sicca most common (secondary Sjӧgren’s syndrome)
  • Retinal vasculopathy (cotton wool spots) 2nd most common
80
Q

SLE: neuro sx

A

Cognitive dysfunction, psychosis, mood disorder, seizures, cerebrovascular disease, neuropathy, headache

81
Q

SLE: LAD how common?

A

common! usually affects the cervical, axillary, inguinal nodes.

82
Q

SLE: Hematologic

A
  • Anemia of chronic disease most common type.

- Leukopenia and mild thrombocytopenia also common

83
Q

SLE: Labs (which ones are always positive?)

A

-**ANA almost always positive–> But not specific for SLE

-Anti-dsDNA
Specific but not sensitive
Levels often correspond to disease activity
-Anti-Sm (anti-smith)–> 99% specific, but not very sensitive

  • Antiphospholipid antibodies:
  • -False positive for syphilis
  • -Lupus anticoagulant. Can prolong aPTT.

-May have Anti-Ro (SSA) and anti-La (SSB) antibodies

84
Q

SLE: other labs

A 
Anemia
Leukopenia
Thrombocytopenia
Elevated Cr
False-positive for syphilis
Proteinuria, abnormal urine sediment
Hematuria
Hypocomplementemia
Direct Coombs positive
85
Q

SLE: ddx

A 
RA
Fibromyalgia
Viral infections: Parvovirus B19, HIV, Hep B/C, EBV, CMV
Malignancy, esp. non-Hodgkin lymphoma
Mixed connective tissue disease
Drug-induced lupus
Sjӧgren’s Syndrome with extraglandular manifestations
Systemic vasculitis
Serum sickness
Scleroderma
86
Q

Drug-induced Lupus:

  • Ex’s of drugs?
  • tx?
A

-Procainamide, hydralazine, isoniazid, chlorpromazine, methyldopa, minocycline, quinidine

  • Differentiating factors:
  • Equal sex ratio
  • Doesn’t normally have nephritis and CNS involvement
  • No anti-dsDNA antibodies or hypocomplementemia
  • Lab abnormalities and clinical manifestations usually normalize when drug is discontinued
87
Q

Dx- ACR Criteria for SLE:

how many criteria need to be met for a dx?

A

4/11 needed

is there a malar rash, photosensitivity, oral ulcers, renal impairment, ect

88
Q

SLICC (systemic lupus internation colab. criteria) Classification Criteria__/17 needed

DONT MEMORIZE the criteria

A

4 out of 17 needed

Acute cutaneous lupus
Chronic cutaneous lupus
Nonscarring alopecia
Oral or nasal ulcers
Joint disease
Serositis
Renal
Neurologic
Hemolytic anemia
Leukopenia or lymphopenia
Thrombocytopenia
ANA
Anti-dsDNA
Anti-Sm
Antiphospholipid
Low complement
Direct Coomb’s test
89
Q

SLE dx: what if the pt doesn’t meet criteria?

A 
-Can still diagnose SLE if the patient has 2-3 criteria plus at least 1 other feature associated with SLE:
Optic neuritis
Aseptic meningitis
Pneumonitis, pulmonary hemorrhage, pHTN, ILD
Myocarditis, Libman-Sacks endocarditis
Abdominal vasculitis
Raynaud phenomenon
Elevated ESR/CRP
90
Q

SLE tx: general stuff to tell Pts and consider

A
  • Regular exercise
  • Smoking cessation
  • Smokers have more active disease
  • Sun protection–>**DAILY sunscreen of at least 55 SPF
  • Healthy diet
  • Prevention of glucocorticoid-induced osteoporosis
  • Stay up to date on vaccines, esp. before starting immunosuppressants
  • Many patients will need to be treated with antimalarials (hydroxychloroquine)
91
Q

Cutaneous SLE: tx

A

Initial: topical glucocorticoids. Start low potency 1st–> medium –>high
-Can do intralesional injections of triamcinolone for refractory lesions
-May need systemic meds:
Hydroxychloroquine is treatment of choice

92
Q

SLE- MSK Sx tx:

A

NSAIDs or Tylenol are first-line

-May add hydroxychloroquine

93
Q

SLE: tx of serositis

A

serositis= inflammation of serous tissues of the body

  • NSAIDs and/or low-mod glucocorticoids
  • Hydroxychloroquine or PO colchicine if persistent or recurrent
  • If severe or refractory: mod-high dose steroids, then taper and switch to MTX, azathioprine, or MMF
94
Q

SLE: Tx of renal sx

A
  • must get a Renal biopsy–> Exact pathology will guide treatment choice
  • ACEI or ARB for HTN/proteinuria
  • Likely to be given an immunosuppressant (table on following slides)
95
Q

SLE: tx of neuropsychiatric sx

A
  • Depends on manifestation
  • Seizures often treated with regular anticonvulsants used in non-SLE patients
  • CVA associated with antiphospholipid antibodies: long-term anticoagulation
  • Glucorticoids often used, but controversial
96
Q

SLE: tx of hematologic sx

A

-If leukopenic with recurrent infections might use prednisone to ↑ WBC.

  • Thrombocytopenia <20k or <50k + hx bleeding and severe autoimmune hemolytic anemia:
  • -High-dose glucocorticoids, then taper once improvement seen.
  • -IVIG often used
  • -Immunosuppressants
  • Splenectomy last resort
97
Q

Ex’s of immunosuppresants used in SLE Pts

A
  • glucocorticoids
  • **Hydroxychloroquine
  • Methotrexate
  • Azathiopine
98
Q

Important S/E associated with hydroxychloroquine in SLE pts

A
  • **Ocular effects including inability to focus, corneal deposits, and retinopathy; rash, hyperpigmentation, myopathy, headache, nausea
  • these Pts will need yearly eye exams!!
99
Q

SLE: prognosis

A

-Improved drastically in recent times to a 90% 10 year survival

-Bimodal mortality:
–Early after diagnosis due to
Infections (esp. opportunistic)
or Active SLE: renal or CNS

  • -Later years:
  • Atherosclerosis from chronic inflammation
  • 5x higher incidence of MI than general population
  • Higher malignancy risk: (esp. an aggressive non-Hodgkin lymphoma)
  • Avascular necrosis of bone and other long-term effects of steroids
    note: -Those with renal disease have the highest mortality risk. and SLE pts have increased cancer risk
100
Q

Polymyositis= autoimmune myopathy characterized by..

A

proximal muscle weakness

101
Q

Polymyositis: epidemiology/risk factors

A
  • Rare, about 1/100,000/year
  • Women > men 2:1
  • Black>Caucasian
  • Peak ages 45-60
  • Genetic risk factors: HLA polymorphisms
102
Q

Polymyositis: pathophys

A
  • Many patients have myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs)
  • Inflammatory cell infiltrates in muscle tissue
  • Nonimmune processes also thought to have a role in muscle dysfunction/damage
  • Pathophysiology poorly understood
103
Q

Polymyositis: clinical sx

A
  • **Proximal muscle weakness & muscle fatigue
  • Usually gradual and progressive
  • **Symmetric
  • **Leg weakness usually before arm weakness
  • Neck flexor weakness also common
  • Patients may complain of trouble rising out of a chair, climbing stairs, washing hair
  • May have dysphagia, difficulty breathing, fecal incontinence
  • May have muscle pain & tenderness in some, but usually not CC
  • May have ILD, myocarditis, overlap syndrome with connective tissue diseases
104
Q

Polymyositis: Labs

most Pts will have elevated ___?

A
  • **Most will have ↑ CK
  • Troponin I most helpful for detecting cardiac muscle involvement
  • LDH, aldolase, AST, and ALT can also be elevated
  • ESR/CRP not particularly useful
  • ANA can be positive in many (~70%)

-Autoantibodies:
–Anti-Jo-1 antibody
In those with associated ILD
–Anti-SRP: Rapidly progressive, severe polymyositis & dysphagia
–Anti-Mi-2 & anti-155/140: Associated with dermatomyositis

105
Q

Polymyositis: gold standard dx test

A
  • **muscle biopsy= gold standard
  • Pick a muscle that’s weak and affected, but not atrophied–> Quadriceps or deltoid good choices

-**Inflammatory infiltrate invades the fascicle.

106
Q

Polymyositis: other good dx tests

A
  • **EMG and MRI
  • Good to identify which muscle you want to biopsy
  • Do bx on contralateral side to avoid needle artifact
  • MRI might be even better than EMG at this
  • Helps differentiate myositis from a neuropathic cause for weakness
  • -EMG typically shows early recruitment–> Increased number of motor units firing rapidly to produce a low level of contraction
  • MRI can show areas of muscle inflammation, edema, fibrosis, calcification
107
Q

Polymyositis: DDx

A 
-Drug-induced myopathy
(Statin, glucocorticoids, antimalarials, antipsychotics, colchicine, and more)
-Myasthenia gravis
-Hypothyroidism
-HIV infection
-Inclusion body myositis
-Muscular dystrophy
-Myotonic dystrophy
-Inherited metabolic myopathies
108
Q

Polymyositis: Dx

A

-No validated dx/classification criteria

Can make the dx without a muscle biopsy if the patient has:

  • -Characteristic clinical symptoms: Proximal muscle weakness
  • -Abnormal muscle enzyme labs
  • -No better explanation for a diagnosis

-Some will also get an EMG or MRI

109
Q

Polymyositis: tx

A
  • **steroids: Prednisone 1 mg/kg/day PO daily (max 80 mg) 4-6 weeks to achieve disease control
  • -Taper slowly based upon treatment response/labs
  • Osteoporosis prophylaxis

-Frequently combined with another immunosuppressant:
(May be started initially or later if no response seen to steroids)
–Azathioprine (2 mg/kg/day) or
–Methotrexate (similar dosing to RA, up to 25 mg/day)

-No need to avoid exercise, it actually helps. PT recommended

110
Q

Polymyositis: Prognosis

A

-With immunosuppressant meds, 75% improve

-Worst prognosis in those who had delayed treatment:
Permanent muscle damage/atrophy can develop

  • Most don’t regain completely normal muscle function
  • Those with anti-Jo-1 antibodies usually don’t completely respond to treatment & do worse in general
  • Increased risk of malignancy- need close screening
  • Steroid side effects from long-term use
111
Q 
Psoriatic Arthritis (PsA)=
-also describe the clinical domains
A

=Chronic inflammatory arthritis associated with psoriasis

Clinical domains:

  • Peripheral arthritis
  • Axial disease
  • Enthesitis
  • Dactylitis
  • Skin & Nail disease
112
Q 
Psoriatic Arthritis (PsA)=
etiology
A

-Unknown etiology
-Association with HLA alleles
-Usually develops after (or at the same time) the onset of psoriasis
But not always

113
Q

Psoriatic Arthritis (PsA)= clinical sx (of the joints)

A

-Pain & stiffness of affected joints–>**Morning stiffness >30 minutes common. Worse with prolonged immobility

  • **Symmetric Polyarthritis (more common) or asymmetric oligoarthritis (less common)
  • ->Can be hard to distinguish from RA
  • **Distal arthritis–>DIP involvement
  • **Arthritis mutilans: Deforming, destructive arthritis
  • ***Spondyloarthritis: Sacroiliitis & spondylitis–>C spine involvement common
114
Q 
Psoriatic Arthritis (PsA):
-Describe Periarticular Disease (3 things)
A
  • Enthesitis
  • Tenosynovitis (Flexor tendons of hands, extensor carpi ulnaris, & others)
  • Dactylitis
115
Q

Enthesitis=

A

=Inflammation at the site of insertion of tendons

–>Soft tissue swelling, TTP on exam

116
Q

Dactylitis=

A

“Sausage digit”- diffuse swelling of an entire finger or toe

117
Q

Arthritis usually appears after onset of _____ _____

A

skin lesions–>Erythematous plaques with scaling

118
Q 
Psoriatic Arthritis (PsA);
-Nail Sx
A
  • **Pitting
  • **Ridging
  • **Onycholysis
  • Nail bed hyperkeratosis
  • Splinter hemorrhage
119
Q

Psoriatic Arthritis (PsA)=

  • May see ____ _____ in hands/feet
  • Eye Sx?
A
  • pitting edema in hands/feet

- Ocular inflammation: uveitis, conjunctivitis

120
Q

Psoriatic Arthritis (PsA): labs

A
  • Nonspecific, no labs diagnostic for PsA
  • ESR/CRP often elevated
  • Synovial fluid analysis: inflammatory
  • RF usually negative
121
Q

Psoriatic Arthritis (PsA): X-ray will show? (**Key finding)

A
  • Joint space narrowing
  • Erosions involving DIP and PIP joints
  • ***“Pencil-in-cup” deformity
122
Q

Psoriatic Arthritis (PsA): dx

A
  • No consensus on diagnostic criteria
  • Generally, diagnosis made in a patient with both psoriasis & inflammatory arthritis in a typical pattern
  • Can diagnose it in the absence of psoriasis:
  • -Distal joint involvement
  • -Asymmetric distribution
  • -Nail lesions
  • -Dactylitis
  • -FH of psoriasis
  • -Presence of HLA-C*06
123
Q

CASPAR Criteria: A Pt w/ inflammatory MSK disease can be classified as having Psoriatic Arthritis (PsA) if they have at least 3 points from the following:

A
  • Skin psoriasis that is:
  • -Present (2 points)
  • -Previously present by history (1 point)
  • -FH of psoriasis, if patient not affected (1 point)
  • Nail lesions (1 point)
  • Dactylitis (present or past) (1 point)
  • Negative RF (1 point)
  • Juxtaarticular bone formation on radiographs (1 point)
124
Q

Psoriatic Arthritis (PsA): tx general principles (who should be involved?)

A

Get derm & rheum involved
Early treatment
PT/OT/exercise
Weight reduction

125
Q

PsA: tx meds

-mild peripheral or axial arthritis?

A

**NSAIDS 1st choice–> Naproxen, Celebrex

126
Q

PsA: tx meds

-for Pt with severe or unresponsive to NSAIDS

A

-**DMARDs: **Methotrexate, **sulfasalazine, azathioprine, cyclosporine, hydroxychloroquine

-**Biologic TNF inhibitors:
Etanercept, infliximab, adalimumab, golimumab, certolizumab pegol

-Immunosuppression, reactivation of latent TB

  • **DONT use PO steroids
  • ->Can cause a flare of pustular psoriasis
127
Q

PsA: comorbidities and risks

A
  • Increased risk of CV disease
  • Diabetes, atherosclerosis, metabolic syndrome
  • Increased rates of depression & anxiety

Clin Med semester 2 (24 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions