Random

Question 1

Behçet’s disease

Answer 1

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

Question 2

Marfan syndrome

Answer 2

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

Question 3

Drug induced lupus

Answer 3

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

Question 4

Enteropathic Spondylarthritis

Answer 4

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

Question 5

Limited cutaneous scleroderma

Answer 5

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

Question 6

Granulomatosis with polyangiitis

Answer 6

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

Question 7

Erythema nodosum

Answer 7

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

Question 8

Giant cell arteritis

Answer 8

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

Question 9

Leukocytoclastic vasculitis

Answer 9

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

Question 10

Takayasu arteritis

Answer 10

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

Question 11

Polymyositis

Answer 11

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

Question 12

Dermatomyositis

Answer 12

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

Question 13

Inclusion body myositis

Answer 13

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

Question 14

Mixed connective tissue disease

Answer 14

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

Question 15

Psoriatic arthritis

Answer 15

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

Question 16

Rheumatic fever

Answer 16

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

Question 17

Antiphospholipid Ab syndrome

Answer 17

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

Question 18

Calcium pyrophosphate deposition disease aka pseudogout

Answer 18

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

Question 19

Dermatomyositis

Answer 19

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

Question 20

Adult onset Still’s disease

Answer 20

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

Question 21

Systemic Juvenile Idiopathic Arthritis (Still disease)

Answer 21

Fever, rash, arthritis, hepatosplenomegaly, leukocytosis

Txt: NSAIDS, IL1, IL6, corticosteroids, methotrexate

Question 22

Oligoarticular juvenile arthritis

Answer 22

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

Question 23

Polyarticular juvenile arthritis

Answer 23

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

Question 24

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Answer 24

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

Question 25

Paget's disease of bone

Answer 25

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

Question 26

Gout

Answer 26

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

Question 27

Charcot-Marie Tooth disease

Answer 27

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

Question 28

Osteomalacia

Answer 28

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

Question 29

Sjogrens syndrome

Answer 29

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

Question 30

Ehler's Danlos Syndrome

Answer 30

``` 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 ```

Question 31

Hemachromatosis arthropathy

Answer 31

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

Question 32

Felty syndrome

Answer 32

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)

Question 33

Adhesive capsulitis

Answer 33

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

Question 34

Alpha 1 antitrypsiin deficiency

Answer 34

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

Question 35

Vasculitis in RA

Answer 35

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

Question 36

Serum sickness

Answer 36

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