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Flashcards in Connective Tissue Diseases Deck (116)
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1

+ ANA is loosely associated with underlying autoimmune diseases. ANA patterns are not specific for individual autoimmune disorders, and a positive test by indirect immunofluorescence often leads to additional testing to detect specific, disease-associated autoantibodies. What defines a normal vs. positive ANA titer?

Normal/negative = titer of <1:40

Positive/clinically significant = 1:160 [higher the ratio, more clinically significant]

2

Another name for c-ANCA

PR3-ANCA

3

Another name for p-ANCA

MPO-ANCA

4

A homogenous staining pattern on immunofluorescence means that the entire nucleus is diffusely stained. What antibodies typically stain this way?

Antibodies include those directed against chromatin, histone proteins, and DNA

Typically corresponds with:
*Drug-induced SLE
Sjogrens syndrome
SLE

5

A speckled staining pattern on immunofluorescence means that fine or coarse speckles are seen throughout the nucleus. What antibodies typically stain this way?

This is a nonspecific pattern — many different Abs stain this way, including those against UI RNP, Sm, and La

Typically corresponds with:
Mixed CT disease
Diffuse systemic sclerosis
Sjogren’s syndrome
SLE

6

________ pattern of immunofluorescence staining that refers to the presence of 30-60 uniform speckles distributed through the nucleus of resting cells.

In mitotic cells, the speckles localize to the chromosomes at the metaphase plate. This pattern is seen with ________

Centromere

Limited systemic sclerosis (CREST)

7

_______ pattern of immunofluorescence refers to homogenous or speckled staining of the nucleolus

Typically corresponds to Abs directed against fibrillarin, ___________, Th, PM-Scl, and RNA helicase.

Corresponds with conditions such as ______ and _______

Nucleolar

RNA polymerase I and III

Diffuse systemic sclerosis; SLE

8

SLE is a multisystem inflammatory autoimmune disease of unknown etiology, but is related to T and B cell hyperactivity with autoantibodies to nuclear antigens, making it a ____________ HSR

It is characterized by spontaneous remissions and relapses with varying severity and presentation. It is more common in females, and in terms of race it is more common in _____ and _____. Socioeconomic factors are correlated with increased incidence as well. Genetic and environmental factors are also suspected, including sex hormones, UV light, and viruses such as ______

Type III HSR

African Americans; Hispanics; EBV

9

Constitutional, cutaneous, and joint findings associated with SLE

Constitutional: fatigue, fever, malaise, weight loss

Cutaneous: rashes (malar), photosensitivity, vasculitis, alopecia, oral ulcers

Joints: inflammatory, symmetric, non-erosive arthritis

10

Hematologic, cardiopulmonary, and renal features of SLE

Hematologic: anemia (may be hemolytic), neutropenia, thrombocytopenia, LAD, splenomegaly, venous or arterial thrombosis

Cardiopulm: pleuritis, pericarditis, myocarditis, endocarditis; increased risk of MI d/t accelerated atherosclerosis

Renal: lupus nephritis

11

GI and neurologic features of SLE

GI: peritonitis, vasculitis (may present with intestinal angina)

Neurologic: organic brain syndromes, seizures, psychosis, cerebritis

12

Labs to order when SLE is suspected

CBC
ESR
ANA + ANA subtypes

Antibodies to: dsDNA, Sm, Ro/SS-A, La/SS-B, histone

Complement levels (C3, C4, CH50)

PT, PTT

Anti-cardiolipin antibody

Lupus anticoagulant

Urinalysis

13

SLE serology includes positive ANA, positive __________, which correlates with disease activity, and _________ - which does NOT correlate with disease activity

_______ or _____ may also be decreased indicating increased consumption during an inflammatory process, suggesting disease activity. These will return to normal when in a period of remission

Anti-dsDNA; Anti-Sm

C3; C4

14

1/3 of SLE pts have a secondary ______ ______ ______ which may be categorized as type 1, type 2, or type 3; Note that antibodies should be measured on 2 occasions, 12 weeks apart

Antiphospholipid antibody syndrome

15

Type of antiphospholipid antibody syndrome associated with SLE that is a risk factor for venous and arterial thrombosis and miscarriage and causes prolongation of the aPTT

Type 2: Lupus anticoagulant

16

How is presence of lupus anticoagulant confirmed in type 2 APA with SLE?

Abnormal dilute Russel viper venom time (DRVVT) — corrects with addition of phospholipid but not normal plasma

17

Type of antiphospholipid antibody syndrome associated with SLE that is directed at serum cofactor beta-2glycoprotein I (Beta2GPI)

Type 3: Anti-cardiolipin antibodies

18

Type of antiphospholipid antibody syndrome associated with SLE that causes biologic false-positive tests for syphilis (VDRL) due to measurement of IgG and IgM antibodies to a cardiolipin-lecithin-cholesterol antigen complex

Type 1

19

Describe treatment of SLE with secondary APS — note how this changes during pregnancy

Anticoagulation, continued indefinitely

Start LMWH first, then bridge to vitamin K antagonists (like warfarin, which remain the standard of care) to achieve INR of 2-3 (goal 2.5)

Pregnancy — LMWH (enoxaparin) + aspirin [warfarin contraindicated]

20

Cotton wool spots in the setting of suspected immune-mediated disease are indicative of _______ retinopathy

SLE/APS

21

Drug-induced lupus occurs when there is promotion of ________ of DNA. Contrary to SLE, there are no associated ______ or _______ symptoms.

These pts will be positive for ANA as well as _______ antibodies (95%)

Demethylation; renal; neurologic

Anti-histone

22

Drugs associated with drug-induced lupus

Hydralazine
Isoniazid
Minocycline
TNF inhibitors
Quinidine
Chlorpromazine
Methyldopa
Procainamide

23

_____ antibiotics are associated with risk of SLE flare

Sulfa

24

Lupus in pregnancy is associated with what complications?

Gestational HTN (preeclampsia)

Fetal growth restriction

Fetal distress —> fetal loss or premature delivery

25

Neonatal lupus affects children born of mothers with what antibodies?

Anti-Ro (SSA) or Anti-La (SSB)

26

Clinical features associated with neonatal lupus

Transient symptoms include: rashes, thrombocytopenia, hemolytic anemia, arthritis

Complications: permanent complete heart block

27

Non-pharmacologic/preventative treatment measures in SLE pts

Avoid sun exposure and wear sunscreen

Avoid smoking and minimize other risk factors for atherosclerosis

Annual influenza vaccine and pneumococcal vaccine every 5 years

Preventative cancer screening recommendations should be followed closely since these pts have increased risk of malignancy

Be wary of degree of corticosteroid use — long-term increases risk of avascular necrosis of bone

28

Pharmacologic treatment options for SLE

NSAIDs
Corticosteroids (topical or systemic)
Hydroxychloroquine

Azathioprine
Methotrexate
Mycophenolate mofetil
Cyclophosphamide
IVIG
Plasmapheresis
Monoclonal Abs

29

Describe the bimodal pattern of mortality associated with SLE

In the early years after diagnosis:
Opportunistic infections; active SLE issues with kidney or CNS disease

In later years:
Accelerated atherosclerosis (linked to chronic inflammation) — increased rate of MI and thromboembolic events

30

Libman sacks endocarditis is associated with _____

SLE

31

Type of lupus that resembles ringworm or psoriasis

Discoid lupus

32

Demographics of scleroderma (systemic sclerosis)

Age 30-60

W>M

Increased severity seen in African Americans

33

Hallmark clinical findings associated with scleroderma

Thickening and hardening of skin

Microangiopathy and fibrosis of skin and visceral organs; affects skin, lungs, GI, kidney, MSK, and heart

34

Virtually all scleroderma (systemic sclerosis) pts experience secondary ______ phenomenon

Raynaud

35

What causes the dry, itchy skin associated with scleroderma?

Obliteration of eccrine sweat glands and sebaceous glands

36

T/F: There is no approved disease-modifying therapy for scleroderma; treatment involves symptomatic control to improve quality of life and prolong survival

True

37

Name the 3 types of scleroderma and which one has the worst px

Diffuse (dcSSc) = worst px

Limited (lcSSc)

Localized

38

Clinical features of diffuse scleroderma

Systemic, diffuse involvement including proximal extremities and trunk

Early and progressive internal organ involvement — especially kidney, cardiac, and INTERSTITIAL LUNG DISEASE

Worst prognosis

39

Clinical features of limited scleroderma

Fingers, toes, face/neck, distal extremities

Raynaud’s commonly precedes other symptoms (susceptible to digital ischemia)

CREST syndrome

Indolent course

Good prognosis

40

Clinical features of localized scleroderma

Benign skin conditions; affects CHILDREN

Discrete areas of discolored skin induration (patches = morphea, coalesced patches = generalized morphea)

NO Raynaud’s

NOT systemic

Histologically indistinguishable from SSc

41

What are the components of CREST syndrome and what autoimmune condition is this associated with?

Calcinosis cutis

Raynauds

Esophageal dysmotility

Sclerodactyly

Telangiectasia

[associated with limited scleroderma]

42

Scleroderma serology includes positive ANA titers.

What other antibody titers are present in diffuse cutaneous scleroderma?

Anti-Scl 70 (aka anti-DNA topoisomerase I)

Anti-RNA polymerase III

43

Scleroderma serology includes positive ANA titers.

What other antibody titers are present in limited cutaneous scleroderma?

+ Anti-centromere

44

T/F: no therapy to date significantly alters disease course of scleroderma, and tx goal is to manage organ system involvement

True

However tx strategies do exist, including education, CCBs, ACEIs, PPIs, glucocorticoids, cyclophosphamide, PDE5-I, etc

45

What component of H and P is important to consider in work up of a scleroderma pt?

Blood pressure — heralding feature of renal disease

46

What labs, radiographs, and additional studies are important to consider in a scleroderma pt?

Labs: ESR, ANA

Radiographs: CXR, barium swallow if indicated, hand X-ray may show distal tuft resorption and calcinosis

Additional studies: ECG, echo, PFT, consider skin biopsy

47

Educating pts with systemic sclerosis includes warm clothing and smoking cessation.

In terms of pharmacologic therapy, ________ are useful for Raynaud’s phenomenon. ________ are good for controlling HTN and limiting progression of renal disease. Anti-reflux meds are given for GERD sxs. ________ improves lung function and survival in some pts. _________ may improve cardiopulmonary hemodynamics in pts with pulmonary HTN

CCBs
ACE inhibitors
Cyclophosphamide
PDE-5 inhibitors

48

When are glucocorticoids indicated for scleroderma/systemic sclerosis?

Indicated for inflammatory myositis or pericarditis

Glucocorticoids show no efficacy in slowing progression of SSc, and high doses may be associated with development of RENAL CRISIS

49

Type of SSc associated with long-standing hx of Raynauds, indolent course, pulmonary HTN, pronounced vascular manifestations, GERD, telangiectasia, and cutaneous calcinosis

Limited cutaneous systemic sclerosis

[note that with this type renal crisis is uncommon]

50

Type of SSc with soft tissue swelling, erythema, pruritis, fatigue, stiffness, malaise, arthralgias, muscle weakness, carpal tunnel, raynaud’s, and possible hemolytic anemia seen during renal crisis

Diffuse cutaneous systemic sclerosis

51

Describe the 2 phases of diffuse cutaneous systemic sclerosis

Inflammatory edematous phase —> fibrotic phase

Skin induration, hyperpigmentation (dark skin may develop hypopigmentation), loss of body hair and impaired sweating; fibrotic joints —> stiffness

52

Skin findings associated with diffuse scleroderma

Hyper/hypopigmented

Dry/itchy (glands obliterated by fibrosis — contrast to sjogren)

Masklike facies/wrinkles

Microstomia

Telangiectasias

Atrophic skin —> ulcerations

Calcium deposits

Raynaud

53

Primary biliary cirrhosis in the setting of systemic sclerosis may be associated with what antibody?

Anti-mitochondrial

54

GI manifestations of scleroderma

Malnutrition — fat, protein, B12, Vit D deficiencies

Xerostomia

GERD, dysphagia [strictures, barrett esophagus —> increased risk of esophageal adenocarcinoma]

Gastroparesis

Gastric antral vascular ectasia [GAVE]

Chronic diarrhea from bacterial overgrowth

PBC

55

Primary cause of morbidity and mortality in scleroderma involves the pulmonary system. Aspiration pneumonia d/t GERD is one potential cause. What is the primary cause of morbidity/mortality in diffuse scleroderma and how is it diagnosed?

Interstitial lung disease

Manifests as dry cough, dyspnea, “velcro” crackles; dx by PFTs

56

Primary cause of morbidity and mortality in scleroderma involves the pulmonary system. Aspiration pneumonia d/t GERD is one potential cause. What is the primary cause of morbidity/mortality in limited scleroderma and how is it diagnosed?

Pulmonary artery hypertension (PAH)

Mean pulmonary arterial pressure of 25 mmHg or greater (>40 mmHg by echo)

Exertional dyspnea, syncope, angina, right heart failure

Right heart cath to confirm dx

57

Primary cause of morbidity and mortality in scleroderma involves the pulmonary system. Aspiration pneumonia d/t GERD is one potential cause. There is also an increased incidence of ________ carcinoma

Bronchoalveolar carcinoma

58

Primary effect of scleroderma on renal system and how this presents clinically

CKD; renal crisis uncommon but life threatening - typically occurs w/i 4 years of disease onset and is a medical emergency

Characterized by abrupt onset of malignant hypertension, hemolytic anemia, and progressive renal insufficiency

[more commonly occurs in diffuse SSc, avoid high dose corticosteroids]

59

Cardiac manifestations in scleroderma

Myocardial fibrosis
Cardiomyopathy
Pericarditis
Myocarditis
Pericardial effusion
Arrhythmia

[cardiac manifestations occur in 50% of SSc pts]

60

The MSK findings of carpal tunnel syndrome, tendon friction rubs, fibrosis and adhesion of tendon sheaths, and hypothyroid d/t thyroid fibrosis occur in what CT disease?

Scleroderma (systemic sclerosis)

61

Sjogren’s syndrome is more common in females, particularly in their mid-50’s (postmenopausal). They often complain of sicca symptoms — what are sicca symptoms and what causes them?

Dry eyes, dry mouth, vaginal dryness, tracheobronchial dryness

Caused by immune-mediated dysfunction of lacrimal and salivary glands

62

Complications of sicca symptoms in sjogrens syndrome

Increased incidence of oral infection (candida)

Dental caries

Parotid or other major salivary gland enlargement

63

What is keratoconjunctivitis sicca? (Seen in sjogrens)

Foreign body sensation-inadequate tear production

Decreased lacrimal flow and impaired lacrimal composition (lymphocyte and plasma cell infiltration of lacrimal glands) damage the cornea and conjunctiva epithelia

64

The ______ test can be used to check for keratoconjunctivities sicca, as it measures the quantity of tears secreted

Schirmer

65

What malignancy is sjogren’s syndrome associated with having increased risk of developing?

B cell non-hodgkin lymphoma

66

What step is essential in the diagnosis of sjogrens?

Lip biopsy — reveals characteristic lymphoid foci in accessory salivary glands

[biopsy of parotid gland is reserved for pts with atypical presentation like unilateral gland enlargement]

67

Serology findings in sjogrens syndrome

+ANA
+RF

Hypergammaglobulinemia
+Anti-SSA/Ro
+Anti-SSB/La (never present w/o Ro)

Low C4 complement

High ESR

Anemia of chronic disease

68

Presence of ______ in sjogren syndrome pt serology is associated with newborn complete heart block

Anti-SSA/Ro

69

Treatment for Sjogrens is symptomatic, with regular follow up at the dentist and ophthalmologist. For dry eyes, artificial tears, ophthalmic lubricating ointments, or local stimulation with cAMP or _________ drops may be used.

Xerostomia is treated by frequent sips of water and/or sugar-free candy. Other sicca manifestations may improve with ______ or ________.

________ may help associated arthralgias. ______ are not effective for sicca symptoms but may have role in tx of extraglandular manifestations

Cyclosporine

Pilocarpine; cevimeline

Hydroxychloroquine; glucocorticoids

70

T/F: atropinic drugs and decongestants are standards of care in sjogrens syndrome, and there are several options for immunomodulatory drugs that may benefit pts

False - atropinic drugs and decongestants should be avoided in sjogrens syndrome, and no immunomodulatory drug has proved efficacious

71

Inflammatory myopathies are more common in African American females and clinical features include myalgias, symmetric b/l proximal muscle weakness (i.e., difficulty rising from a chair or climbing stairs) and may be associated with skin manifestations in certain conditions. What are general lab findings associated with inflammatory myopathies? (Serum muscle enzymes, ESR, CRP, EMG, bx findings?)

Elevation of serum muscle enzymes — creatine kinase (CK) and aldolase

ESR and CRP often normal

Characteristic EMG findings

Characteristic muscle biopsy

72

Characteristic skin lesions of DM

Gottron’s patches/papules — raised violaceous lesions overlying the dorsa of DIP, PIP, and MCP joints

Heliotrope rash — periorbital edema, purplish suffusion over eyelids

“Shawl-sign” — erythema over neck/shoulders, upper chest and back

Periungual erythema

73

CT disease associated with increased risk of occult malignancy, necessitating frequent cancer screening, most commonly ovarian, lung, pancreatc, stomach, colorectal, and NHL

Dermatomyositis

[when dermatomyositis is diagnosed, start looking for occult malignancy — CBC, CMP, SPEP, UA and age appropriate cancer screenings. Some malignancies may not be evident for months after initial presentation of dermatomyositis and initial screenings]

74

Ovarian cancer is the most common occult malignancy associated with dermatomyositis, what screening measures should be performed to dx this?

Check transvaginal US

CT abd/pelvis

CA-125

75

Demographics and initial presenting sign/symptom of dermatomyositis

Age 7-15 and 30-60

Presents as weakness WITHOUT sensory symptoms; affects proximal muscles early and progresses to distal muscles late in disease course

76

Biopsy and lab findings that are diagnostic of dermatomyositis

Biopsy — perimysial and perivascular inflammation; PERIFASCICULAR ATROPHY

Elevated CK, aldolase

Anti Jo-1
Anti-Mi2
Anti-MDA5
Anti-P155/P140

77

Polymyositis is typically dx at age 30-50 and manifests as subacute (over weeks to months) proximal muscle weakness WITHOUT skin changes. What are biopsy and lab findings associated with polymyositis?

Muscle biopsy shows ENDOMYSIAL inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx (IBM or muscular dystrophy)

Labs: elevated serum CK, Anti-Jo-1

78

Primary pharmacologic tx strategy for dermatomyositis and polymyositis

Corticosteroids

[other tx options include methotrexate, azathioprine, cyclophosphamide, cyclosporine, IVIG, mycophenolate mofetil, rituximab, and hydroxychloroquine]

79

4 treatment steps often effective for polymyositis and dermatomyositis, but not for IBM

1. Glucocorticoids x3-4 weeks then tapered

2. 75% of pts require additional immunosuppressive drugs

3. IVIG

4. Trial of one of the following: rituximab, cyclosporine, cyclophosphamide, or tacrolimus

80

Demographics of inclusion body myositis (IBM)

>40-50

M>F

Caucasian more common

81

Clinical features of IBM

Finger flexion or quadriceps weakness

82

Biopsy and lab findings associated with IBM

Muscle biopsy shows ENDOMYSIAL inflammation and RIMMED VACUOLES; invasion of non-necrotic muscle fibers, anti-cN1A autoantibodies

CK is mildly elevated or NORMAL

83

Treatment strategy for IBM

IBM is refractory to tx, so tx is supportive

84

Systemic vasculitide most prevalent in adolescent girls and young women and more common in Asia; affects large vessels, exhibiting long smooth tapered stenosis (most commonly subclavian and innominate) and as relapsing/remitting course. Nicknamed the “pulseless disease” due to obliteration of UE peripheral pulses

Takayasu arteritis

85

Complications of takayasu arteritis

50% have pulmonary involvement

Retinopathy (copper-wiring)

Renal artery stenosis

Aortic dilations, AAA, aortic rupture

Aortic regurgitation

86

Diagnosis imaging strategies and histology of takayasu arteritis

Dx = MRI or CT angiography

Histology = granuloma with some giant cells, fibrosis in chronic stages

87

Tx of takayasu arteritis

Glucocorticoids

88

Palpable purpura
NO thrombocytopenia
Arthritis
Abdominal pain
Glomerulonephritis
Any age, more common in kids

IgAV aka Henoch-Schonlein Purpura (HSP)

89

Dx and tx of HSP

Dx by Bx shows IgA deposits

Tx: supportive/glucocorticoids

90

Manifestations of anti-GBM

Deposition of anti-basement membrane autoantibodies in basement membrane; affects glomerular capillaries and pulmonary capillaries

91

Behcet Syndrome = variable vessel size; associated with silk route (Turkey, Asia, Mid East). What is the HLA associated and the triad of Behcet Syndrome?

HLA-B51

1. Recurrent mouth ulcers
2. Genital ulcers
3. Eye inflammation (uveitis)

92

Involvement of large vessels by Behcet syndrome is associated with ________, while venous involvement may lead to _____.

_____ is the clinical feature of pustules at the site of sterile needle pricks.

Behcet is also associated with large joint arthralgias, neuro involvement that may mimic MS, ulcers in distal ileum or cecum, and treatment includes low dose _______

Aneurysms; DVT

Pathergy

Glucocorticoids

93

Medium vessel/segmental vasculitide that may be ANCA+; associated with HBV and more common in males

Polyarteritis Nodosa

94

Signs/symptoms of polyarteritis nodosa (constitutional, skin, peripheral nerve, GI, renal, cardiac, lungs)

Constitutional: fever, malaise, weight loss

Skin: livedo reticularis, subcutaneous nodules, ulcers, digital gangrene

Peripheral nerve (80% have vasculitis neuropathy) — mononeuritis multiplex manifests as foot drop

GI: “intestinal angina” - post prandial abd pain

Renal: renin-mediated HTN, renal infarct

Cardiac: CHF, MI, new HTN

Lungs typically spared

95

How is polyarteritis nodosa diagnosed and treated?

Dx confirmed by biopsy or angiogram:

Bx — infiltration and destruction of blood vessels by inflammatory cells —> fibrinoid necrosis, NO granulomas

Angiogram — micro-aneurysm

ANCA negative (check HBsAg and HBeAg)

Tx: corticosteroids

96

Medium vessel vasculitide often affecting kids <5 y/o; may be referred to as mucocutaneous lymph node syndrome or strawberry tongue; clinical features include fever, inflamed tongue, LAD, rash, peeling of skin around fingernails and toenails, red eyes

Kawasaki disease

97

Cause of death in kawasaki disease

Death from coronary involvement (aneurysm or MI — can occur years later)

98

Tx of kawasaki disease

IVIG within 10 days of sxs and High dose ASA

99

Small vessel vasculitide more common in males >40 y/o; characterized by +C-ANCA (PR3-ANCA)

Affects respiratory tract, kidneys, can have hearing loss, orbital masses, ulcerative keratitis/scleritis/episcleritis/conjunctivitis, and venous thrombotic events

Granulomatosis with Polyangiitis (aka Wegener’s granulomatosus)

100

Respiratory tract findings with granulomatosis with polyangiitis (including CXR findings)

Involves upper and lower respiratory tract

90% nasal involvement — saddle nose/crusting/bleeding/obstruction

Erosive sinus disease

Lungs: asymptomatic nodule —> alveolar hemorrhage

CXR shows infiltrate, nodules, and/or cavitary lesions

101

3 hallmarks of granulomatosis with polyangiitis:

_________ inflammation

Necrotizing _________

Segmental __________

Granulomatous

Vasculitis

Glomerulonephritis (hematuria/RBCs/proteinuria)

102

Tx for granulomatosus with polyangiitis

Cyclophosphamide and high dose glucocorticoids or rituximab

[methotrexate can be considered if renal function is normal]

103

Eosinophilic granulomatosis with polyangiitis (aka EGPA or Churg-Strauss Syndrome) is ANCA+ in 50% of pts — typically ____-ANCA

This disease is characterized by granulomas, ______ + eosinophilia —> _______

MPO-ANCA

Asthma; vasculitis

104

3 phases of eosinophilic granulomatosis with polyangiitis

Prodromal phase: allergic disease (asthma/allergic rhinitis) — lasts months to years

Eosinophilia-tissue infiltration phase: high peripheral eosinophilia; tissue infiltration in lung/GI/other tissues

Vasulitis phase: systemic necrotizing heart/lungs/nerves/skin; palpable purpura

105

Treatment for eosinophilic granulomatosis with polyangiitis

Glucocorticoids

106

Medium vessel vasculitide affecting young males (<35 y/o) and only occurs in smokers; typically distal vessels affected first and considered a segmental disease that is complicated by thrombosis —> loss of digits and possibly hands/feet

Thromboangiitis obliterans (buerger disease)

107

Dx and tx for thromboangiitis obliterans

Dx: angiography - “corkscrew” appearance

Tx: stop smoking (glucocorticoids and anticoagulation doesnt work)

108

Differentiate primary from secondary raynauds

Primary — benign, symmetric exaggerated physiologic response to cold or emotion, ages 15-30. Nailfold capillaroscopy is normal in primary Raynauds

Secondary — occurs in CTD, hematologic and endocrine conditions, occupational d/o, use of beta blockers or cancer drugs cisplatin and bleomycin; UNILATERAL, more severe — ischemia. Nailfold capillaroscopy is distorted with widened and irregular loops, dilated lumen and areas of vascular “dropout”

109

Treatment options for raynauds

Wear gloves when temp outside is low

Lotions

Stop smoking

Limit use or stop sympathomimetic drugs — decongestants, diet pills, amphetamines

Utilize calcium channel blockers like amlodipine

Surgery (sympathectomy) is an option if medical and supportive tx have failed, and there are frequent severe attacks of digital ischemia

110

Polymyalgia rheumatica (PMR) and ______________ vasculitide frequently co-exist; this is more common in W>M, >40-50, and white>AA. Constitutional symptoms include fever, malaise, weight loss, and a ______ WBC count

Giant cell arteritis; normal

111

Large vessel vasculitide affecting cranial arteries (temporal/facial/ophthalmic) and aortic arch; manifests clinically as HA, jaw claudication, PMR, visual abnormalities (amaurosis fugax or diplopia), and ESR increased (>50 mm/h)

Giant cell arteritis (GCA; aka temporal arteritis)

112

HLA and demographic associations with giant cell arteritis

HLA-DR4

>50 y/o

W> M

Northern european ancestry

113

Dx and tx of giant cell arteritis

Gold standard dx = temporal a. bx (need 1 cm segment) — shows segmental granulomatous vasculitis with multinucleated giant cells

Start corticosteroids (before biopsy!!) —> dramatic improvement

[W/o treatment pt goes blind — so you MUST start tx before biopsy!!!!]

114

Proximal severe symmetrical morning and daylong stiffness, soreness, and pain in shoulder, nck, and pelvic girdles with feelings of weakness as a result of pain (not a true weakness) — manifests as trouble combing hair or putting on a coat

Polymyalgia rheumatica

115

Biopsy, muscle enzyme, EMG findings, ESR, and CRP with polymyalgia rheumatica

No inflammation on muscle bx

Muscle enzymes and EMG normal

Elevated ESR and CRP

116

Tx of polymyalgia rheumatica

Corticosteroids