Clinical Approach to Malaise and Generalized Pain Flashcards

1
Q

What is considered a positive ANA titer?

What is considered normal?

A

(+) ANA - 1:160.

Normal ANA - <1:40.

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2
Q

cANCA =

pANCA =

A

cANCA = PR3-ANCA

pANCA = MPO-ANCA

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3
Q

What ANA pattern is demonstrated in CREST (limited systemic sclerosis)?

A

Centromere pattern

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4
Q

SLE is what type of hypersensitivity?

A

Type 3

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5
Q

What antibodies are positive in SLE? (3)

What tends to be decreased in SLE?

A

+ ANA
+ anti-dsDNA: correlates best with disease activity.
+ Smith: does NOT correlated with disease activity.

Decreased C3 or C4.

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6
Q

What is the difference between primary APS and secondary APS?

A

Primary APS: patients that have it without SLE.

Secondary APS: patients with SLE (1/3 of SLE patients).

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7
Q

What antibodies exist in each of the 3 types of APS?

A

Type 1: false-positive test for syphilis.

Type 2: + lupus anticoagulant (increased risk for thrombosis and miscarriage).

Type 3: anti-cardiolipin antibodies (beta-2 GPI).

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8
Q

When should antibodies be measured in APS?

A

On 2 occasions 12 weeks apart.

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9
Q

What is the treatment for APS?

A

Anticoagulation, which is continued indefinitely.

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10
Q

What is a characteristic on fundoscopic exam in a patient with SLE/APS?

A

Retinaopathy - “cotton wool spots”.

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11
Q

What is the characteristic positive antibody in drug-induced lupus?

A

+ anti-histone antibodies

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12
Q

Which antibiotics cause an SLE flare?

A

Sulfa drugs.

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13
Q

Neonatal lupus tends to affect children with which positive antibodies?

What are the symptoms of nenatal lupus? (4)

What is the major permanent finding in these babies?

A

+ anti-Ro or anti-La antibodies (only 1-2% of lupus patients).

Rashes, thrombocytopenia, hemolytic anemia and arthritis.

Permanent complete heart block.

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14
Q

What are the 4 most common areas of treatment for SLE?

A

Avoidance of sun exposure

NSAIDs

Steroids

Hydroxychloroquine

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15
Q

What are some findings in the early years of SLE?

What are some findings in the later years?

A

Early years: infections, and kidney or CNS disease.

Later years: accelerated atherosclerosis (increased risk for MI) and thrombotic events.

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16
Q

What are some management/preventative measures to employ in a patient with SLE? (6)

A

*Minimize other risks for atherosclerosis.

Avoid smoking.

Pneumococcal vaccine every 5 years.

Check for malignancy (increased risk for CA in patients with SLE).

Evaluate for side-effects of corticosteroid use (OP, hyperglycemia, etc.).

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17
Q

Discoid lupus erythematosus (DLE) is considered a variant of what?

How may it arise?

What is the major characterization of it?

What is the treatment?

A

Cutaneous lupus.

It may arise independent of SLE or be a manifestation of it.

Well-defined inflammatory plaques that evolve into atrophic, disfiguring scars. This makes people desire treatment.

Photoprotection and topical anti-inflammatory drugs or anti-malarials. Early detection is important to minimize scarring.

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18
Q

What are considered hallmarks of scleroderma?

A

Thickening and hardening of the skin, microangiopathy and fibrosis of the skin and visceral organs.

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19
Q

What causes the dry and itchy skin seen in scleroderma?

A

Obliteration of eccrine and sebaceous glands.

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20
Q

What are findings in diffuse (dcSSc) scleroderma? (2)

What is the prognosis?

A
  • Systemic, diffuse involvement of the proximal extremities and trunk.
  • Early and progressive internal organ involvement - kidney, cardiac and interstitial lung disease.

Worse prognosis.

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21
Q

What are findings in limited (lcSSc) scleroderma? (3)

What is the prognosis?

A
  • Affects the fingers, toes, face/neck and distal extremities.
  • Raynaud’s phenomenon.
  • Pulmonary HTN.

Good prognosis with an indolent course.

22
Q

What is CREST syndrome?

What does CREST mean?

A

A sub-type of limited scleroderma.

C - calcinosis cutis.
R - Raynaud's (usually first symptom).
E - esophageal dysmotility.
S - sclerodactyly.
T - telangiectasia.
23
Q

What is localized scleroderma?

Who does it affect?

What is a characteristic finding?

What findings are absent in this form of scleroderma?

A

A form of scleroderma defined by benign skin lesions.

Children.

Morphea patches.

No Raynaud’s phenomenon and it is NOT systemic.

24
Q

Which forms of scleroderma would you expect to find ILD vs. PAH?

A

ILD - diffuse scleroderma (dx by PFT).

PAH - limited scleroderma.

25
Q

What is used to confirm diagnosis of PAH due to scleroderma?

A

Right heart catheterization.

26
Q

What malignancy are patients with limited scleroderma at a higher risk for?

A

Bronchoalveolar carcinoma.

27
Q

CKD and renal crisis is more common in which form of scleroderma?

What symptoms are seen in the onset?

What can induce the crisis?

A

Diffuse SSc.

Abrupt onset of malignant HTN, hemolytic anemia and progressive renal insufficiency.

High dose corticosteroids.

28
Q

What organ system is the major (primary) cause of morbidity and mortality in patients with SSc?

A

Pulmonary - ILD, aspiration pneumonia, PAH, CA, etc.

29
Q

What are the major GI conditions are associated with SSc? (3)

A

Malnutrition - fat, protein, B12, vit. D deficiency, etc.

GERD - increased risk for esophageal CA.

Gastric antral vascular ectasia (GAVE) - AKA “watermelon stomach”.

30
Q

What is the major MSK finding in SSc?

A

Carpal tunnel syndrome

31
Q

What is the major endocrine dysfunction in SSc?

A

Hypothyroidism due to thyroid fibrosis.

32
Q

What are the + ANA findings in diffuse SSc?

What are the + ANA findings in limited SSc?

A

Diffuse SSc: + anti-Scl 70 (AKA anti-topoisomerase I) and + anti-RNA polymerase III.

Limited SSc: + anti-centromere.

33
Q

What is the name of the symptoms associated with Sjogren syndrome?

What is the associated malignancy?

A

Sicca symptoms: mucosal dryness (mouth, eyes, vagina, etc.).

B-cell NHL (MALToma).

34
Q

What antibodies are positive in Sjogren syndrome?

What is necessary for diagnosis?

What drugs must be avoided in patients with Sjogren’s?
Which drugs prove efficacous?

A

+ anti-SSA/Ro
+ anti-SSB/La

Lip biopsy.

Atropinic drugs and decongestants.
NO drugs have been proved efficacous.

35
Q

What symptoms are typical of inflammatory myopathies?

What lab findings are typical?

A

Symmetrical (BL) involvement of proximal muscles.

Elevated CK and aldolase; ESR/CRP is normal usually.

36
Q

What findings are common in dermatomyositis? (4)

There is an increased risk of what?

What is the major histological finding?
What are the typical lab findings?
What antibodies are positive?

A

Gottron’s patches - dorsum of DIP, PIP and MCP jts.
Heliotrope rash - eyes/eyelids.
Periungual erythema.
V-neck erythema (Shawl sign).

*Occult malignancy.

Histology: perimysial and perivascular inflammation with perifascicular atrophy.
Labs: elevated CK and aldolase.
Antibodies: + anti-Jo1/Mi2, + anti-MDA5, +anti-P155/P140.

37
Q

What is the most common malignancy associated with dermatomysositis?

A

Ovarian CA: check transvaginal US, CT abd./pelvis and CA-125.

**maligancies should be evaluated starting from the beginning of a diagnosis of dermatomyositis.

38
Q

What finding is seen in dermatomyositis and NOT in polymyositis?

A

No skin changes in in polymyositis.

39
Q

What is seen on biopsy in polymyositis?

What labs are abnormal?
What antibodies are positive?

A

Endomysial inflammation.

Labs: + CK.
+ anti-Jo1.

40
Q

What is first-line treatment of DM/PM?

A

Steroids.

41
Q

What parts of the body are characteristically weakened in inclusion body myositis?

What is seen on biopsy?

What labs are abnormal?
What antibodies are positive?

What makes treatment difficult?

A

Flexors of the fingers and quadriceps.

Rimmed vacules.

Elevated CK.
+ anti-cN1A antibodies.

IBM is refractory to treatment, so treatment is supportive.

42
Q

Henoch-Schonlein purpura affects what?

Which patients are most often affected?

What is the tetrad of symptoms?

What is seen on biopsy?

What is the treatment?

A

Small vessels.

It can occur at any age, but it is most common in children.

*Palpable prupura
No thrombocytopenia
Arthritis/arthralgia
Abdominal pain
Renal disease (GN)

IgA deposits.

Steroids/supportive care.

43
Q

What vessels are affected in Takayasu arteritis?

What ages are most common?

What is seen on biopsy?

What symptoms are prominent? (6)

What are the most concerning complications?

How is it diagnosed?

A

Large vessels (aorta and its branches).

<40 y/o; F>M.

Bx: Long smooth tapered stenosis and granulomas with giant cells.

"Pulseless disease" due to obliteration of UE pulses.
Pulmonary involvement.
Renal artery stenosis.
HTN
Retinopathy
Cardiac involvement.

Aortic dilations, regurgitation, aneurysm and rupture.

MRI or CT angiography.

44
Q

What sized vessels are affected in Behcet syndrome?

What is the triad of symptoms?

What marker is positive?

A

Variable; if in large vessels it may cause aneurysms. If in veins, may cause DVT.

Recurrent mouth ulcers.
Genital ulcers.
Uveitis.

+ HLA-B51.

45
Q

What vessel size is implicated in PAN?

What is a unique association(s)?

What site tends to be spared?

What is the typical finding on biopsy?

A

Medium-sized vessels.

HBV and foot drop.

Lungs.

Fibrinoid necrosis.

46
Q

What sized vessels are implicated in Kawasaki disease?

What are typical symptoms?

What is the major cause of death?

What is the treatment?

A

Medium-sized vessels.

Fever, LAD, rash and “strawberry tongue”.

Coronary disease: aneurysm or MI.

IVIG and high-dose ASA (the one unique scenario to use ASA in a pediatric patient).

47
Q

What sized vessels are implicated in granulomatosis with polyangiitis?

What is the hallmark histological finding?

What antibody is positive?

What symptoms are most classic?

What is the treatment?

A

Small vessel.

Granulomatous inflammation.

+ cANCA.

Nasal involvement and kidney involvement.

Cyclophosphamide, high dose steroids or Rituximab.

48
Q

What symptoms suggest Churg-Strauss syndrome?

What antibody is positive?

What PE finding is classic?

A

Asthma and eosinophilia.

+ pANCA.

Palpable purpura.

49
Q

What vasculitis only occurs in smokers? What age are they?

What is needed for diagnosis?

A

Thromboangiitis obliterans (AKA Buerger disease).

Angiography with “corkscrew” appearance.

50
Q

What arteries are often affected in giant cell arteritis?

What other disease is it associated with?

What are the common symptoms?

What is needed for diagnosis?

If untreated, what is the worst outcome?

A

Cranial arteries (temporal/facial/ophthalmic) and aortic arch.

Polymyalgia rheumatica.

HA, jaw pain, visual changes, etc.

HLA-DR4, ESR > 50 mm/h, and temporal artery biopsy.

Blindness.

51
Q

What is primary Raynaud’s phenomenon vs. secondary Raynaud’s?

A

Primary: benign and symmetric. It is exaggerated by cold or emotion. Ages 15-30 y/o.

Secondary: occurs in adjunct with other disease. It is more severe and ofte iscemic. >30 y/o typically.

52
Q

What finding is used to differentiate between in primary and secondary Raynaud’s?

A

Nailfold capillaroscopy: NL in primary, but distorted with irregular loops in secondary.