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Medicine Refresher > Rheumatology/Immunology > Flashcards

Rheumatology/Immunology Flashcards

1
Q

Vasculitis Gen Info

A

Inflammation within blood vessel walls
Classified based on size of vessel involved
- large vessel: aorta and major branches > takayasu arteritis, temporal arteritis
- medium-vessel: medium arteries > PAN, kawasaki disease
- small-vessel: small arteries and capillaries; further subdivided into ANCA-associated vasculitides and immune complex vasculitides
ANCA > GPA, EGPA, microscopic polyangiitis
Immune complex > cryoglobulinemic, IgA vasculitis
Variable-vessel > behcet syndrome
In all, blood vessels are inflamed and vascular necrosis can result
Clinical findings depend on size of vessel involved + location of involvement (target organ ischemia)
Systemic illness not explained by another process (or ischemia involving 1+ systems) > think vasculitis

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2
Q

Temporal/GCA gen info

A

Large-vessel Vasculitis
Unknown cause; > 50y, 2xfemale > male
temporal arteries mc affected but can involve others > aorta, carotids > carotid bruits, decr pulses in arms, aortic regurgitation
Increased risk of aortic aneurysm/dissection

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3
Q

Temporal/GCA SXS

A

Constitutional: malaise, fatigue, weight loss, low-grade fever
Headaches
Visual impairment (25-50%) d/t ophthalmic artery involvement; optic neuritis; amaurosis fugax > blindness
Jaw pain with chewing > intermittent claudication of jaw/tongue
Tenderness over temporal artery; absent temporal pulse
Palpable nodules
40% have PMR

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4
Q

Temporal/GCA DX

A 
ESR elevated (normal level does not exclude)
Biopsy of temporal artery (sensitivity 90; single negative biopsy doesn't exclude).
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5
Q

Temporal/GCA TX

A

high-dose steroids (prednisone) early to prevent blindness
start treatment immediately even if temporal arteritis only suspected, don’t wait for biopsy results
if + visual loss > admit for IV steroids + start oral prednisone
+ diagnosis > tx 4 weeks then taper gradually but maintain steroid tx for 2-3 yrs (relapse likely if stopped prematurely)
F/u ESR for tx effectiveness
visual loss in one eye temp or permanent (steroid given to prevent loss in other eye)
untreated > disease usually eventually self-limiting (excluding vision loss)

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6
Q

Takayasu Arteritis gen info

A

MC young Asian women
Granulomatous vasculitis of aortic arch and its major branches– leading to fibrosis, stenosis, or narrowing of vessels
Diagnosed via arteriogram
Suspect in young woman + decr/absent peripheral pulses, discrepancies of BP (arm vs leg) + arterial bruits

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7
Q

Takayasu Arteritis sxs

A
  1. constitutional symptoms - fever, night sweats, malaise, arthralgias, fatigue
  2. Pain and tenderness over involved vessels
  3. Absent pulses in carotid, radial, or ulnar arteries; +- aortic regurgitation
    4 sxs of ischemia eventually develop in areas supplied by involved vessels
  4. Severe complications include limb ischemia, aortic aneurysms, aortic regurgitation, stroke, and secondary HTN due to renal artery stenosis.
    Main prognostic predictor = presence or absence of these complications.
  5. Causes visual disturbances due to ocular involvement and hemorrhage of retinal arteries
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8
Q

Takayasu Arteritis tx

A

Steroids like prednisone
Tx HTN
Cytotoxic drugs - MTX, azathioprine, revascularization in some
Surgery or angioplasty to recannulate stenosed vessels. Bypass grafting is sometimes necessary.

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9
Q

Polyarteritis Nodosa (PAN) gen info

A
  1. Vasculitis of medium-sized vessels involving nervous system and Gl tract
    NO pulmonary involvement in PAN (distinguishes it from GPA).
  2. Can be associated with hepatitis B, HIV, and drug reactions
  3. Patho: PMN invasion of all layers and fibrinoid necrosis plus resulting intimal proliferation > reduced luminal area, > ischemia, infarction, aneurysms
  4. Necrosis is segmented, leading to “rosary sign” as a result of aneurysms
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10
Q

PAN SXS

A
  1. Early symptoms: fever, weakness, weight loss, myalgias, arthralgias, abdominal pain (bowel angina).
  2. Other findings: HTN, mononeuritis multiplex, livedo reticularis
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11
Q

PAN DX

A
  1. Biopsy of involved tissue or mesenteric angiography (no granulomas)
  2. ESR usually elevated +- p-ANCA
  3. Test for fecal occult blood
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12
Q

PAN TX

A

Poor prognosis, but improved with treatment
Corticosteroids first
Severe > add cyclophosphamide

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13
Q

Granulomatosis with Polyangiitis (GPA aka Wegener Granulomatosis) gen info

A

ANCA-associated small vessel vasculitides
Predominantly involving kidneys + respiratory tract (sometimes other organs as well)
Most have sinus disease, pulmonary disease, and GN (necrosis of nose, lung, & kidney)
Renal disease accounts for the majority of deaths

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14
Q

GPA sxs

A
  1. Upper respiratory sx (e.g, sinusitis); purulent or bloody nasal discharge
  2. Oral ulcers (may be painful)
  3. Pulmonary symptoms (cough, hemoptysis, dyspnea)
  4. Renal involvement (glomerulonephritis- may have rapidly progressive renal failure)
  5. Eye disease (conjunctivitis, scleritis)
  6. Musculoskeletal (arthralgias, myalgias)
  7. Tracheal stenosis
  8. Constitutional findings (fever, wt loss, etc)
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15
Q

GPA dx

A
  1. CXR abnormal (nodules or infiltrates).
  2. Lab findings: +CANCA best initial lab test. Biopsy definitive (lung > sinus or kidney) → large necrotizing granulomas. Markedly elevated ESR, anemia (normochromic normocytic), hematuria, thrombocytopenia
  3. Open lung biopsy confirms diagnosis.
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16
Q

GPA tx

A
  1. Prognosis is poor–most die within 1 year after the diagnosis
  2. A combination of cyclophosphamide + corticosteroids can induce remissions but a relapse may occur at any time.
  3. Consider renal transplantation if have ESRD
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17
Q

Eosinophilic Granulomatosis with Polyangiitis (EGPA aka Churg-Strauss Syndrome)

A

• Vasculitis involving many organ systems (respiratory MC, cardiac, Gl, skin, renal, neurologic)
• SXS: triad - asthma + eosinophilia + chronic rhinosinusitis; prodromal phase atopic dz, allergic rhinitis, asthma, eosinophilic phase peripheral blood eosinophilia & infiltration of organs (skin, lung, GI tract), vasculitic phase constitutional symptoms
• DX: eosinophilia & + PANCA, incr ESR/CRP, IgE, RF, biopsy best (granulomatous necrotizing vasculitis)
• Prognosis poor with a 5-year survival of 25% (death is usually due to cardiac or pulmonary complications).
TX: with steroids > 5-year prognosis improves
to 50%.

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18
Q

Microscopic Polyangiitis

A

Affects capillaries
Like GPA > commonly involves the skin (palpable purpura), lungs (cough, dyspnea, hemoptysis, pulmonary fibrosis, pulmonary hypertension), and kidneys (glomerulonephritis)
Unlike GPA > does not typically have nasopharyngeal involvement
Distinguished from GPA by biopsy > shows necrotizing vasculitis without granulomas

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19
Q

Cryoglobulinemic Vasculitis

A

Immune Complex Small-Vessel Vasculitides
Patho: deposition of cryoglobulins (immunoglobulins that precipitate in cold temps)
Classified into type 1, 2, 3 based on type and clonality of immunoglobulins
MC cause is chronic HCV infection
Other causes: chronic HBV, HIV, other causes of chronic liver disease (AI hepatitis, PBC)
Proposed mechanism: deposition of antigen-antibody complexes (such as HCV antibodies) in small vessels > complement activation and inflammation.
Liver disease may contribute to decreased immune complex clearance > greater tissue deposition and disease activity
Common clinical manifestations include palpable purpura, renal disease, arthralgias/arthritis, peripheral neuropathy, and hypocomplementemia. Pulmonary and CNS involvement are rare.
• Clinical dx: presence of circulating cryoglobulins
+ biopsy showing leukocytoclastic vasculitis (inflammation with polymorphonuclear leukocyte nuclear debris).

20
Q

Behcet Syndrome

A

An autoimmune, multi-system vasculitic disease; cause is unknown; mc in Middle Easterners.
SXS: painful, sterile oral and genital ulcerations (pathergy), arthritis (knees and ankles MC), eye involvement (uveitis, optic neuritis, iritis, conjunctivitis), CNS involvement (meningoencephalitis, intracranial HTN), fever, and weight loss.
DX: biopsy of involved tissue (lab tests not helpful)
TX: steroids

21
Q

Takayasu Arteritis dx

A

angiography (MRA or CTA) necessary to confirm diagnosis. Nonspecific - incr ESR/CRP, normochromic, normocytic anemia, leukocytosis

22
Q

Kawasaki syndrome gen info

A

medium & small vessel necrotizing vasculitis including coronary arteries. Thought to be an unidentified respiratory agent or viral pathogen w propensity towards vascular tissue. Rfx children <5y, boys, asians

23
Q

Kawasaki syndrome sxs

A

warm + CREAM = fever > 5 days + 4 of these 5; Conjunctivitis, Rash, Extremity changes (edema, erythema, desquamation of palms/soles, beau’s lines, arthritis, Adenopathy (cervical), Mucositis (strawberry tongue, lip swelling, fissures, pharyngeal erythema).

24
Q

Kawasaki syndrome dx

A

labs - nonspecific, elevated WBC & platelet count, anemia, incr ESR/CRP, sterile pyuria. Echo/ECG - to look for complications (coronary vessel arteritis, coronary artery aneurysm, MI, pericarditis, myocarditis)

25
Q

Kawasaki syndrome tx

A

IV immunoglobulin + aspirin for fever, joint pain & prevention of coronary complications

26
Q

Rheumatoid Arthritis gen info

A

AI disease involving the synovium of joints > inflamed synovium > damage to cartilage + bone
chronic systemic autoimmune inflammatory dz with symmetric polyarthritis, bone erosion, cartilage destruction & joint structure loss.
Hyperplastic synovial tissue (pannus) → joint destruction (T-cell mediated)
Rfx: women, 30-50y, smoking, HLA-DRB 1 & 4
RA unlikely if > joint distribution not symmetric, DIPs involved, morning stiffness absent
Much of debilitating joint damage happens in early disease > early tx with DMARDs critical
Poor prognosis if > high RG titers, subQ nodules, erosive arthritis
Etiology uncertain; may be caused by infection but genetic predisposition necessary
RA joint changes more extensive than OA bc entire synovium involved (osteophytes from OA not present in RA)

27
Q

Rheumatoid Arthritis sxs

A
  1. Inflammatory polyarthritis (joint swelling mc); can involve every joint in the body except DIP joints
    a. Morning stiffness lasting > 1 hour- improves as day progresses.
    b. Joints commonly involved; joints of hands (PIP, MCP), wrists, knees, ankles, elbows, hips, and shoulders.
    c. Characteristic hand deformities.
    • Ulnar deviation of MCP joints
    • Boutonnière deformities of the PIP joints (PIP flexed, DIP hyperextended)
    • Swan-neck contractures of the fingers (MCP flexed, PIP hyperextended, DIP flexed)
  2. Constitutional symptoms can be prominent
    a. Low-grade fever, weight loss, fatigue
  3. Cervical spine involvement common at C1-C2 (subluxation and instability), but less common in the lower cervical spine.
    a. C-spine instability potentially life-threatening. Most patients don’t have neuro involvement; can be progressive/fatal if not treated surgically.
    b. Seen in 30-40%. All patients should have cervical spine xrays before undergoing any surgery (due to risk of neurologic injury during intubation). DMARDs have dramatically reduced need for c spine surgery in RA.
  4. Cardiac involvement; pericarditis, pericardial effusions, conduction abnormalities, valvular incompetence.
  5. Pulmonary involvement; usually pleural effusions; interstitial fibrosis
  6. Ocular involvement; episcleritis or scleritis.
  7. Soft tissue swelling (rather than bony enlargement).
  8. Drying of mucous membranes: sjögren xerostomia.
  9. Subcutaneous rheumatoid nodules over extensor surfaces; may also occur in visceral
    structures- lungs, pleura, pericardium (Figure 6-5).
    a. Pathognomonic for RA.
    b. Nearly always occurs in seropositive patients (i.e., those with RF).
28
Q

Rheumatoid Arthritis dx

A
  • Inflammatory arthritis of multiple joints (higher likelihood if 3 or more joints involved); MCP. PIP, wrist, elbow, knee, ankle, MTP joints
  • Symptoms lasting at least 6 weeks
  • Positive serum RF or ACPA
    1. Lab findings:
    a. High titers of RF (nonspecific, a/w more severe disease)
  • RF titers rarely change with disease activity (not useful for following)
  • helpful for prognosis
    b. anticitrullinated peptide/protein antibodies (ACPA)
    c. Elevated ESR, CRP
    d. Anemia of chronic disease.
    2. Xrays, not required but may have:
    a. Loss of juxta-articular bone mass (periarticular osteoporosis) near the finger joints
    b. Narrowing of the joint space (due to thinning of the articular cartilage) > late disease.
    c. Bony erosions at the margins of joint.
    3. Synovial fluid analysis is nonspecific.
29
Q

RA tx

A
  1. Goal; minimize pain/swelling + prevent disease progression + help pt remain as functional as possible.
  2. Exercise helps to maintain ROM and muscle strength.
  3. Symptomatic treatment.
    a. NSAIDs drugs of choice for pain control.
    b. Corticosteroids (low dose) use these if NSAIDs do not provide adequate relief. Short-term treatment may be appropriate but avoid long-term use.
  4. DMARDS
  5. Surgery (in severe cases)
    a. Synovectomy (arthroscopic) decreases joint pain/swelling but does not prevent x-ray progression + doesn’t improve joint ROM
    b. Joint replacement surgery for severe pain unresponsive to conservative measures
30
Q

RA - DMARDS

A

a. General principles
• Can reduce morbidity and mortality (~30%) by limiting complications, slowing progression of disease, preserving joint function
• Should be initiated early (at time of diagnosis)
• They have a slow onset of action (6 weeks+ for effect to be seen), so begin treating RA while waiting for therapy to take effect. Gradually taper and discontinue NSAIDs + corticosteroids once effects are evident
b. Methotrexate > best initial DMARD
• Initial improvement seen in 4-6 weeks.
• SE: Gl upset, oral ulcers (stomatitis), mild alopecia, bone marrow suppression (co-administer with folinic acid), hepatocellular injury, and idiosyncratic interstitial pneumonitis > pulmonary fibrosis +- elevated LFTs
• Closely monitor liver + renal function
• Supplement with folate.
c. Alternatives: leflunomide, sulfasalazine, hydroxychloroquine
d. Antitumor necrosis factor (anti-TNF) inhibiting agents (etanercept, infliximab, etc.) used if methotrexate does not fully control disease. Requires PPD
screening because of risk of reactivation of TB.
e. Non-TNF biologics (abatacept, rituximab, tofacitinib) also options if disease activity remains high despite methotrexate.

31
Q

Lupus gen info

A
  1. Autoimmune disorder > inflammation/tissue damage in multiple organ systems.
  2. SLE is an idiopathic chronic inflammatory disease
    with genetic, environmental, + hormonal factors.
  3. Patho: autoantibody production, deposition of immune complexes, complement activation, and accompanying tissue destruction/vasculitis.
  4. Types:
    a. Spontaneous SLE
    b. Cutaneous lupus erythematosus (skin lesions without systemic disease)
    c. Drug-induced lupus
    d. ANA-negative lupus- associated findings:
    • Arthritis, Raynaud phenomenon, subacute cutaneous lupus
    • Serology: Ro (anti-SS-A) or antiphospholipid antibody-positive, ANA negative
    • Risk of neonatal lupus in infants of affected women
    • ANA negativity can be influenced by lab testing methods, disease duration, treatment
    RFx: women (childbearing age), African Americans
    Chronic disease > exacerbations and remissions
    Malar rash, joint pain, fatigue mc
    More advanced disease > renal, pulm, CV, and nervous systems are affected.
32
Q

Lupus sxs

A
  1. Constitutional: fatigue, malaise, fever, weight loss
  2. Cutaneous: Butterly rash (found 1/3 cases), photosensitivity, discoid lesions (erythematous raised patches with keratotic scaling), oral or nasopharyngeal ulcers, alopecia, Raynaud phenomenon (vasospasm of small vessels when exposed to cold; found in ~20%)
  3. Musculoskeletal: Arthralgias (may be first sx; found ~90%), arthritis (inflammatory and symmetric, rarely deforming like in RA), myalgia w/w/o myositis
  4. Cardiac: Pericarditis, endocarditis (Libman-Sacks endocarditis > serious complication), myocarditis
  5. Pulmonary: Pleuritis mc, pleural effusion, pneumonitis (may lead to fibrosis), pulmonary HTN (rare)
  6. Hematologic: Hemolytic anemia + anemia or reticulocytosis of chronic disease, leukopenia, lymphopenia, thrombocytopenia
  7. Renal: Proteinuria >0.5 g/day (may have nephrotic syndrome), cellular casts, glomerulonephritis (may have hematuria), azotemia, pyuria, uremia, HTN
  8. Immunologic: Impaired immune response due to many factors, including autoantibodies to lymphocytes, abnormal T-cell function, and immunosuppressive medications; often a/w antiphospholipid syndrome
  9. Gl: Nausea/vomiting, dyspepsia, dysphagia, PUD
  10. CNS: Seizures, psychosis (may be subtle), depression, headaches, TIA/CVA
  11. Other findings: conjunctivitis, increased incidence of Raynaud phenomenon and Sjögren syndrome
33
Q

Lupus dx

A
  1. Made based on 2012 SLICC criteria; must have at least 4 criteria (at least 1 clinical criteria + 1 immunologic criteria) OR biopsy-proven lupus nephritis + positive ANA or anti-dsDNA.
  2. Autoantibodies in lupus:
    a. ANA: sensitive but not specific; almost all have elevated serum ANA levels
    b. Anti-ds DNA (in 70%): very specific (but not sensitive)
    c. Anti-Smith (in 30%): very specific (but not sensitive)
    d. Antiphospholipid antibody positivity, as determined by positive lupus anticoagulant, false-positive RPR, medium-high titer anticardiolipin antibody level, or
    positive anti-beta-2 glycoprotein
    e. Antihistone Abs (in 70%) are present in >95% of cases of drug-induced lupus. If negative, drug-induced lupus can be excluded.
    f. Ro (SS-A) and La (SS-B) are found in 15% to 35% Associated with:
    • Sjögren syndrome
    • Subacute cutaneous SLE
    • Neonatal lupus (with congenital heart block)
    • Complement deficiency (C2 and C4)
    • ANA-negative lupus
34
Q

Criteria for Diagnosing SLE (2012 SLICC Criteria)

A 
Clinical criteria, in the absence of other causes:
1. Acute cutaneous lupus
2. Chronic cutaneous lupus
3. Oral or nasal ulcers
4. Nonscarring alopecia
5. Synovitis involving two or more joints
6. Serositis
7. Renal: urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500-mg protein over
24 hours OR red blood cell casts
8. Neurologic
9. Hemolytic anemia
10. Leukopenia or lymphopenia
11. Thrombocytopenia
Immunologic criteria:
1. ANA
2. Anti-ds DNA
3. Anti-Smith
4. Antiphospholipid antibody positivity
5. Low complement (C3, C4, CH50)
6. Coombs positivity (in absence of hemolytic anemia)
35
Q

Lupus tx

A
  1. Avoid sun exposure because it can exacerbate cutaneous rashes
  2. NSAIDs– for less severe symptoms
  3. Either local or systemic corticosteroids– for acute exacerbations
  4. Systemic steroids for severe manifestations
  5. Best long-term therapy is antimalarial agents such as hydroxychloroquine– for constitutional, cutaneous, and articular manifestations.
    Hydroxychloroquine is continued as preventative measure even after resolution of symptoms.
    Baseline and subsequent annual eye exams are needed because of retinal toxicity.
  6. Cytotoxic agents such as cyclophosphamide– for active glomerulonephritis
  7. Monitor the following + tx:
    a. renal disease (most significant morbidity)
    b. HTN
36
Q

ANA positive conditions

A 
SLE (almost all patients) *highly sensitive
RA
Scleroderma
Sjorgren syndrome
Mixed connective tissue disease
Polymyositis and dermato-myositis
Drug-induced lupus
37
Q

RF

A 
RA (70%) - not sensitive or specific
healthy people (3%)
38
Q

C-ANCA

A

GPA - sensitive + specific

varies with disease activity

39
Q

P-ANCA

A

PAN (70-80% sensitive for microscopic PAN; not specific)

40
Q

Lupus Anticoagulant

A

APLS

41
Q

ESR

A 
Infection (acute or chronic)
Malignancy
Rheum disease
Misc (tissue necrosis, pregnancy)
Major uses: diagnose/rule out inflammatory process + monitor course
42
Q

CRP

A

Inflammatory states and infection
Misc conditions (MI, vasculitis, trauma, malignancy, pancreatitis)
Uses: primarily infection (more sensitive + specific than ESR)
If markedly elevated > 15 – bacterial infection likely

43
Q

Common variable immunodeficiency (CVID) gen info

A

Group of heterogeneous disorders characterized by hypogammaglobulinemia of ≥ 2 immunoglobulin (Ig) isotypes, IgG plus IgA and/or IgM
MC clinically-apparent primary immunodeficiency

Most have sporadic disease with unknown cause
5%-25% have heritable disease > pathogenic variants in genes related to B-cell antibody production, T-cell differentiation, DNA metabolism.

44
Q

CVID sxs

A
  • Recurrent infections (respiratory and GI infections mc)
  • Autoimmune diseases
  • Gastrointestinal diseases.
  • Chronic lung disease and bronchiectasis.
  • Malignant and nonmalignant neoplasms.
  • Granulomatous inflammation
45
Q

CVID dx

A

Hypogammaglobulinemia with both:
1- low serum IgG (≥ 2 standard deviations below mean) in ≥ 2 measurements at least 3 weeks apart based on age-adjusted local reference (repeat measurement not necessary in patients requiring immediate treatment)
2- low serum IgA or IgM
Testing: CBW w diff, urine protein analysis (r/o nephrotic syndrome etiology), serum Igs + subclasses
Absent isohemagglutinins and/or poor response to vaccines
Exclusion of other causes of hypogammaglobulinemia
Genetic testing not required for diagnosis or management of CVID. Generally not necessary outside a research setting

46
Q

CVID tx

A

Treatment/prevention of infections and specific therapy for noninfectious manifestations
immunoglobulin replacement (significant impaired ab production)
Start antimicrobial regimens early and for prolonged periods in patients with signs of infection
Consider prophylactic antibiotics to prevent recurrent infections
Stem cell transplantation > CVID plus malignancy or severe organ damage
F/U: assess patients every 6-12 months to monitor for development of cancer or autoimmunity
Immunizations

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