Connective Tissue Diseases Flashcards
+ 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]
Another name for c-ANCA
PR3-ANCA
Another name for p-ANCA
MPO-ANCA
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
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
________ 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)
_______ 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
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
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
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
GI and neurologic features of SLE
GI: peritonitis, vasculitis (may present with intestinal angina)
Neurologic: organic brain syndromes, seizures, psychosis, cerebritis
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
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
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
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
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
Type of antiphospholipid antibody syndrome associated with SLE that is directed at serum cofactor beta-2glycoprotein I (Beta2GPI)
Type 3: Anti-cardiolipin antibodies
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
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]
Cotton wool spots in the setting of suspected immune-mediated disease are indicative of _______ retinopathy
SLE/APS
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
Drugs associated with drug-induced lupus
Hydralazine Isoniazid Minocycline TNF inhibitors Quinidine Chlorpromazine Methyldopa Procainamide
_____ antibiotics are associated with risk of SLE flare
Sulfa
Lupus in pregnancy is associated with what complications?
Gestational HTN (preeclampsia)
Fetal growth restriction
Fetal distress —> fetal loss or premature delivery
Neonatal lupus affects children born of mothers with what antibodies?
Anti-Ro (SSA) or Anti-La (SSB)
Clinical features associated with neonatal lupus
Transient symptoms include: rashes, thrombocytopenia, hemolytic anemia, arthritis
Complications: permanent complete heart block
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
Pharmacologic treatment options for SLE
NSAIDs
Corticosteroids (topical or systemic)
Hydroxychloroquine
Azathioprine Methotrexate Mycophenolate mofetil Cyclophosphamide IVIG Plasmapheresis Monoclonal Abs
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
Libman sacks endocarditis is associated with _____
SLE
Type of lupus that resembles ringworm or psoriasis
Discoid lupus
Demographics of scleroderma (systemic sclerosis)
Age 30-60
W>M
Increased severity seen in African Americans
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
Virtually all scleroderma (systemic sclerosis) pts experience secondary ______ phenomenon
Raynaud
What causes the dry, itchy skin associated with scleroderma?
Obliteration of eccrine sweat glands and sebaceous glands
T/F: There is no approved disease-modifying therapy for scleroderma; treatment involves symptomatic control to improve quality of life and prolong survival
True
Name the 3 types of scleroderma and which one has the worst px
Diffuse (dcSSc) = worst px
Limited (lcSSc)
Localized
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
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
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
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]
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
Scleroderma serology includes positive ANA titers.
What other antibody titers are present in limited cutaneous scleroderma?
+ Anti-centromere
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
What component of H and P is important to consider in work up of a scleroderma pt?
Blood pressure — heralding feature of renal disease
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
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
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
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]
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
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
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
Primary biliary cirrhosis in the setting of systemic sclerosis may be associated with what antibody?
Anti-mitochondrial
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
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
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
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
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]
Cardiac manifestations in scleroderma
Myocardial fibrosis Cardiomyopathy Pericarditis Myocarditis Pericardial effusion Arrhythmia
[cardiac manifestations occur in 50% of SSc pts]
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)
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
Complications of sicca symptoms in sjogrens syndrome
Increased incidence of oral infection (candida)
Dental caries
Parotid or other major salivary gland enlargement
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
The ______ test can be used to check for keratoconjunctivities sicca, as it measures the quantity of tears secreted
Schirmer
What malignancy is sjogren’s syndrome associated with having increased risk of developing?
B cell non-hodgkin lymphoma
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]
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
Presence of ______ in sjogren syndrome pt serology is associated with newborn complete heart block
Anti-SSA/Ro
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
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
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
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
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]
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
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
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
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
Primary pharmacologic tx strategy for dermatomyositis and polymyositis
Corticosteroids
[other tx options include methotrexate, azathioprine, cyclophosphamide, cyclosporine, IVIG, mycophenolate mofetil, rituximab, and hydroxychloroquine]
4 treatment steps often effective for polymyositis and dermatomyositis, but not for IBM
- Glucocorticoids x3-4 weeks then tapered
- 75% of pts require additional immunosuppressive drugs
- IVIG
- Trial of one of the following: rituximab, cyclosporine, cyclophosphamide, or tacrolimus
Demographics of inclusion body myositis (IBM)
> 40-50
M>F
Caucasian more common
Clinical features of IBM
Finger flexion or quadriceps weakness
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
Treatment strategy for IBM
IBM is refractory to tx, so tx is supportive
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
Complications of takayasu arteritis
50% have pulmonary involvement
Retinopathy (copper-wiring)
Renal artery stenosis
Aortic dilations, AAA, aortic rupture
Aortic regurgitation
Diagnosis imaging strategies and histology of takayasu arteritis
Dx = MRI or CT angiography
Histology = granuloma with some giant cells, fibrosis in chronic stages
Tx of takayasu arteritis
Glucocorticoids
Palpable purpura NO thrombocytopenia Arthritis Abdominal pain Glomerulonephritis Any age, more common in kids
IgAV aka Henoch-Schonlein Purpura (HSP)
Dx and tx of HSP
Dx by Bx shows IgA deposits
Tx: supportive/glucocorticoids
Manifestations of anti-GBM
Deposition of anti-basement membrane autoantibodies in basement membrane; affects glomerular capillaries and pulmonary capillaries
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
- Recurrent mouth ulcers
- Genital ulcers
- Eye inflammation (uveitis)
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
Medium vessel/segmental vasculitide that may be ANCA+; associated with HBV and more common in males
Polyarteritis Nodosa
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
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
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
Cause of death in kawasaki disease
Death from coronary involvement (aneurysm or MI — can occur years later)
Tx of kawasaki disease
IVIG within 10 days of sxs and High dose ASA
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)
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
3 hallmarks of granulomatosis with polyangiitis:
_________ inflammation
Necrotizing _________
Segmental __________
Granulomatous
Vasculitis
Glomerulonephritis (hematuria/RBCs/proteinuria)
Tx for granulomatosus with polyangiitis
Cyclophosphamide and high dose glucocorticoids or rituximab
[methotrexate can be considered if renal function is normal]
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
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
Treatment for eosinophilic granulomatosis with polyangiitis
Glucocorticoids
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)
Dx and tx for thromboangiitis obliterans
Dx: angiography - “corkscrew” appearance
Tx: stop smoking (glucocorticoids and anticoagulation doesnt work)
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”
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
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
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)
HLA and demographic associations with giant cell arteritis
HLA-DR4
> 50 y/o
W> M
Northern european ancestry
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!!!!]
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
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
Tx of polymyalgia rheumatica
Corticosteroids
