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Rheumatology > Random > Flashcards

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1
Q

Behçet’s disease

A 
Oral and genital ulcers
Uveal and retinal disease 
Erythema nodosum
Mono/polyarthritis 
Intestinal ulcers 
CNS involvement-meningitis, FND, psychiatric sxs
HLA-B51, inc IgD

Painful ulcers with necrotic center and red rim
Txt: steroids

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2
Q

Marfan syndrome

A 
Autosomal dominant 
Aortic insufficiency 
Risk for aortic dissections
MVP
Pectus carinatum/excavatum (restrictive lung disease pattern)
Arm span to height ratio>1.05 
Pes planus 
Joint hyper mobility 
Ectopia lentis 
Spontaneous pneumothorax 
Striae without weight gain

Txt: no contact sports, yearly echo

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3
Q

Drug induced lupus

A

TNF alpha inhibitors, diltiazem
SHIPP-sulfonamides, hydralazine, isoniazid, procainamide, phenytoin
red and scaly rash on sun exposed areas, photosensitivity, serositis, fever, oral ulcers, arthritis

+anti-histogram Ab
- anti ds-DNA Ab (+ if caused by TNF alpha inhibitors)

Txt: DC Med

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4
Q

Enteropathic Spondylarthritis

A

Associated with IBD
Can have peripheral oligo arthritis prior to diagnosis of UC
Can present as:
Type 1: one joint <10 w
2: months or years
3: peripheral and axial arthritis
Large joints in LE
Sacroiliitis (more common in Crohn’s disease)
Enthesopathy, dactylitis, tendinitis, uveitis, erythema nodosum, pyoderma gangrenosum
No evidence of erosion or joint space narrowing on imaging

Txt: cortical steroids, DMARD’s, TNF alpha inhibitor‘s, NSAIDs

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5
Q

Limited cutaneous scleroderma

A

Fibrosis limited to hands, arms and face
Reynards phenomenon
Pulmonary hypertension
Fatigue, stiff joints, dec strength, pain, sleep issues, skin discoloration
Calcinosis, esophageal motility dysfunction, sclerodactyly and telangiectasia (CREST)
Anticentromere Ab

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6
Q

Granulomatosis with polyangiitis

A

A.k.a. Wegner granulomatosis
Fever, fatigue, rhinosinusitis, cough, dyspnea
Asymptomatic hematuria, proteinuria, RPGN
Arthralgia, purpura, neurologic dysfunction
CXR: nodules/patchy or diffuse opacities, pulmonary infiltrates, hilar adenopathy
Granulomas on biopsy showing necrotizing inflammation and small vessel vasculitis and respiratory involvement required
+PR3-ANCA

Txt: Systemic glucocorticoids and methotrexate in limited disease
Cyclophosphamide for more severe disease
Rituximab

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7
Q

Erythema nodosum

A

Caused by infection such as strep pharyngitis, IBD, sarcoidosis, TB, leukemia/lymphoma, medications such as OCP/penicillin/sulfa, systemic rheumatological conditions
Painful, red 2–5 cm nodules on anterior shin’s

Txt: Self-limiting, and said, potassium iodide, corticosteroids
Self results in about eight weeks

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8
Q

Giant cell arteritis

A

Vasculitis of large and medium size vessels
Fever, fatigue, anorexia, weight loss, jaw claudication
Visual loss
Inc ESR and CRP
Associated with polymygalia rheumatica
Temporal artery bx

Txt: glucocorticoid

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9
Q

Leukocytoclastic vasculitis

A

Painful burning rash LE with palpable non-blanching purpura
Caused by medications Phenytoin, penicillin, sulfonamide, diuretic after 7-10d
Negative ANCA and cryoglobulin
Skin bx: Neutrophil predominant inflammatory infiltration of small vessel walls with fibrinoid degeneration

Txt: Remove offending agent, glucocorticoids

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10
Q

Takayasu arteritis

A

large vessel arteritis affecting aorta and branches
thickening, stenosis, and aneurysmal dilatation of the branches of the aorta
women 10-40, weight loss, fatigue, fever, arm/leg claudication
Absent or diminished peripheral pulses
Inc ESR and CRP
MRA or CTA head, neck, chest and abd-luminal narrowing or occlusion with thickening of vessel wall
6 criteria to diagnose: age <=40, extremity claudication, dec brachial artery pulse, diff in SBP between arms of >10, a bruit over subclavian arteries or the aorta, and imaging showing narrowing of aorta/branches
Txt: high dose prednisone

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11
Q

Polymyositis

A

multiple myalgias, proximal>distal
Inc CK, aldolase, Muscle bx and EMG
Anti-Jo-1 antibodies, Mechanic’s Hands, ILD, Raynaud, Inflammatory arthritis
Txt: corticosteroids, screen for malignancies

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12
Q

Dermatomyositis

A

Heliotrope rash with myalgias, Grotton papules, shawl sign
Proximal >distal, Inc CK/aldolase, Muscle bx and EMG
Anti-Jo-1 antibodies, Mechanic’s Hands, ILD, Raynaud, Inflammatory arthritis
Txt: corticosteroids, DMARDS and IVIG if refractory, screen for malignancies

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13
Q

Inclusion body myositis

A

autoimmune degeneration of muscle cells
pharyngeal muscle weakness
distal > proximal, inc CK/aldolase
Muscle bx (vacuoles and inclusion bodies) and EMG
Txt: corticosteroids, DMARDS and IVIG if refractory

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14
Q

Mixed connective tissue disease

A

Combo of SScl, SLE, polymyositis
hand and finger swelling, myalgias, arthralgias, fatigue, fever, Raynaud phenomenon, esophageal dysmotility, ILD, pHTN
high + speckled ANA titer, high + anti-U1-ribonucleoprotein Ab titer

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15
Q

Psoriatic arthritis

A

oligo/polyarticular disease, psoriasis, enthesitis, dactylitis, IBD, chronic uveitis, nail pitting
Arthritis mutilans, HLA B-27
Txt: mild-NSAIDs, severe-DMARD, anti-TNF alpha inh, anti IL 17 agents

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16
Q

Rheumatic fever

A

About 20 days after group A strep infection
Jones criteria: 2 maj or 1 maj +2 min
Major-carditis, arthritis, chorea, subcutaneous nodules, erythema marginatum
Minor- fever, arthralgia, Inc ESR/CRP, prolonged PR with evidence of strep infxn
Txt: Salicylates, corticosteroids, IVIG, daily amoxicillin for prevention in future

17
Q

Antiphospholipid Ab syndrome

A

Primary or with SLE
Venous/arterial thrombi, pregnancy complications (fetal loss, severe preeclampsia, placental sufficiency), livedo reticularis, thrombocytopenia, TIA
Dx: hx of pregnancy complications/thrombotic event + positive antiphospholipid Ab (anticardiolipin Ab, anti-beta2-glycoprotein-I Ab, lupus anticoagulant)
Tx: Heparin bridge to warfarin

18
Q

Calcium pyrophosphate deposition disease aka pseudogout

A

calcium deposition in the synovium causing acute attacks of arthritis (erythema, pain, edema of knee or wrist)
risk factors: older age, previous trauma to joint, underlying OA, hyperparathyroidism, hemochromatosis, low Mg
Aspiration of fluid: positively birefringent, rhomboid-shaped crystals
XR: chondrocalcinosis (highly specific)
Txt: NSAIDS, intra-articular glucocorticoid injections, and oral colchicine

19
Q

Dermatomyositis

A

Inflammatory myopathy with skin findings and muscle weakness
Periorbital heliotrope rash with edema; erythematous rash on the face, knees, elbows, malleoli, neck, anterior chest (V-sign), back, and shoulders (shawl sign); and violaceous eruptions (Gottron’s rash) on the finger joints, ILD
Photosensitive lesions
Anti-MDA-5 Ab, anti-Mi-2 Ab, anti-TIF-1 Ab, and anti-NXP-2 Ab
MRI muscle: active inflammation
CK >50 x ULN
Txt: prednisone for new-onset disease, IV glucocorticoids for weakness at onset or rapid worsening. Azathioprine, methotrexate, mycophenolate mofetil, or cyclosporine if avoiding steroids, IVIG if no response to steroids

20
Q

Adult onset Still’s disease

A

Yamaguchi criteria: T> 102 x 1 w, arthralgia/arthritis >2w, salmon colored rash on trunk and proximal extremities, leukocytosis (minor: sore throat, LAD, hepato/splenomegaly, abnormal LFTs)
bone marrow bx and BCx to rule out infection/malignancy
Txt: corticosteroids>TNF alpha/IL1/IL6> refractory-anakinra

21
Q

Systemic Juvenile Idiopathic Arthritis (Still disease)

A

Fever, rash, arthritis, hepatosplenomegaly, leukocytosis

Txt: NSAIDS, IL1, IL6, corticosteroids, methotrexate

22
Q

Oligoarticular juvenile arthritis

A

chronic arthritis in 1-4 weight bearing joints, chronic uveitis, young female, ANA positive, ESR normal
Txt: NSAID, corticosteriods, methotrexate, TNF inh

23
Q

Polyarticular juvenile arthritis

A

symmetric chronic arthriits in >5 joints, mild systemic sxs
ANA pos, RF pos, Inc ESR
Txt: NSAIDs, methotrexate, corticosteroids, TNF inh

24
Q

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

A

ANCA to myeloperoxidase
hx of asthma, symmetric pulmonary infilatrates, pulmonary nodules, allergic rhinitis (nasal polyps, nasal obstruction, recurrent sinusitis), peripheral neuropathy, HTN, pericarditis, renal dz, peripheral eosinophil count <10%
Tissue bx: elevation of tissue eosinophilia
Txt: corticosteroids> add cyclophosphamide

25
Q

Paget’s disease of bone

A

increased bone resorption by osteoclasts and subsequent proliferation of new, weaker bone
arthritis, pain, bone deformity, fx, hearing loss
Inc alk phos, Ca and P WNL
XR: cortical thickening and sclerotic lesions
Txt: bisphosphonates

26
Q

Gout

A

monosodium urate crystal deposition in joint
triggered by thiazide diuretic intake, alcohol intake, hospitalization, surgery, rapid inc/dec uric acid level
rapid development (within 24 hours) of painful, erythematous, and swollen joints, 1st MCP
hyperuricemia
synovial fluid: negatively birefringent monosodium urate crystals
Txt: NSAIDS, colchicine, corticosteroids, or corticotropin
prevention: Allopurinol, febuxostat, uricosuric medications (probenecid) only 2-4w after flare

27
Q

Charcot-Marie Tooth disease

A

hereditary neuromuscular disorder causing motor and sensory neuropathy (distal weakness and atrophy)
<20yo, pes cavus, pes planus, hammer toe, twisted ankle, foot drop, weakness and sensory loss in distal UE/LE, dec/absent DTR
Nerve conduction studies to show slow velocity vs mildly reduced velocity
molecular testing to show demyelinating disease vs axonal degeneration disease
Txt: rehab, orthotics, surgery for skeletal abnormalities

28
Q

Osteomalacia

A

decreased mineralization of newly formed osteoid during bone turnover
caused by insufficient dietary Ca and vit D, renal phosphate wasting syndromes, CKD, limited sun exposure, and malabsorption such as inflammatory bowel disease, celiac disease, and gasatric bypass
bone pain, muscle weakness, fx, difficulty walking, muscle spasms, cramps, tingling, numbness, positive Chvostek sign
inc alk phos, inc PTH, dec Ca/P/urinary Ca and 25-hydroxyvitamin D levels
XR: thinning of the cortex, reduced bone mineral density, changes in vertebral bodies, and bilateral looser pseudofractures, fissures, and radiolucent lines
Txt: Vit D

29
Q

Sjogrens syndrome

A

destruction of cells in lacrimal, parotid, and minor salivary glands
keratoconjunctivitis sicca, dry mouth, palpable and nonpalpable purpura, symmetrical arthralgia and arthritis, myalgia, symptoms of polymyositis, interstitial pneumonitis, pericarditis, and pHTN
bx of the salivary gland which may reveal anti-SSA/Ro and anti-SSB/La, Schirmer test
Txt: NSAIDS, hydroxychloroquine, glucocorticoids, azathioprine, leflunomide, and methotrexate

30
Q

Ehler’s Danlos Syndrome

A 
genetic connective tissue disorder, aut dom
joint hypermobility, skin hyperextensibility, and tissue fragility
frequent joint dislocations/subluxations, Thoracolumbar scoliosis, velvety skin, impaired wound healing, easy bruising, atrophic scarring, aortic root dilation, MVP
Molluscoid pseudotumors (spongy, violaceous nodules of subq fat herniating through atrophic dermal scars) on shins and forearms
31
Q

Hemachromatosis arthropathy

A

intestinal dietary iron absorption with deposition in multiple organs and areas of the body
into 2/3 MCP joints
XR: hook-like osteophytes, squared-off bone ends, chondrocalcinosis
Dx: transferrin saturation, ferritin levels, if elevated>test for HFE genotype, liver bx to assess increased iron deposits
Tx: phlebotomy, iron chelation, erythrocytapharesis

32
Q

Felty syndrome

A

uncommon extra-articular manifestation of longstanding and severe RA
seropositive RA, neutropenia, and splenomegaly
Resp and skin infxn
Labs: + RF, + anti CCP, + ANA, anemia, thrombocytopenia, elevated APRs
Dx: exclude large granular lymphocyte syndrome and SLE
Txt: MTX and rituximab after current infxn treated, steroids (when refractory to first 2), G-CSF (ANC<1000 and refractory prior txt or life threatening infxn and just started DMARD)

33
Q

Adhesive capsulitis

A

frozen shoulder syndrome, progressive and painful loss of multidirectional shoulder motion, higher incidence in DM
Txt: self resolves 1-3 years, steroid injection, PT

34
Q

Alpha 1 antitrypsiin deficiency

A

allows proteolytic enzymes to degrade normal tissue
Hx: COPD, particularly panacinar emphysema. cough, progressive exertional dyspnea, exercise intolerance, and wheezing. portal hypertension, chronic hepatitis, cirrhosis, and hepatoma. persistent jaundice, hepatomegaly, hematemesis, ascites, granulomatosis with polyangiitis or panniculitis
Labs: elevated LFTs
CXR: flattened diaphragm, hyperinflated lung fields, and hyperlucent lung bases
Dx: initial screening for serum alpha-1 antitrypsin levels and confirmed by alpha-1 antitrypsin genotyping or protein phenotyping

35
Q

Vasculitis in RA

A

fever, weight loss, skin, CNS involvement, kidney disease, GI involvement
in long standing RA, erosive, seropositive disease, + anti CCP, small/med vessels, poor prognosis
PE: digital ischemia, cutaneous ulcers, and nail fold infarcts. Mononeuritis multiplex. alveolar hemorrhage, necrotizing glomerulonephritis, and ischemic colitis.
Labs: Inc ESR, CRP, WBC and platelets
Txt: corticosteroids, azathioprine, and tocilizumab

36
Q

Serum sickness

A

after immunization with nonhuman serum proteins (vaccines, antivenoms, and medications such as rituximab) leading to the formation of immune complexes
Sxs: malaise, fever, polyarthralgias, and polyarthritis. pruritic rash that does not involve the mucous membranes 1-2 w after initiating offending agent, if previously exposed then earlier onset and more severe sxs
Txt: self limiting, resolves 2 w after removing agent

Rheumatology (11 decks)

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