Rheumatology Flashcards

Question

Which features are more common in limited compared to diffuse cutaenous SSc

Answer 1

Critical ischemia in digits Calcinosis cutis

Answer 2

Limited cutaenous SSc- anticentromere diffuse cutaneous Ssc - Anti topoisomerase I (Scl 70) and anti RNA pol III yung may numbers sa diffuse

Answer 3

ACE inhibitors Avoid steroids. Use only in high risk SSC px only when absolutely required and at low doses <10mg/d

Answer 4

Hematogenous

Answer 5

Inclusion body myositis

Answer 6

acute gout

Answer 7

Calcium Apatite

Answer 8

macrophage

Answer 9

(HLA-DRB1)

Answer 10

cardiovascular dse

Answer 11

Calcinosis Raynaud's Esophageal Dysmotility Sclerodactyly Telangiectasia

Answer 12

pred > 40 mg

Answer 13

procainamide disopyramide propafenone

Answer 14

Enteric causes: Shigella, Salmonella, Yersinia, Campylobacter Urogenital: Chlamydia

Answer 15

fracture most commonly involved --> lower cervical spine

Answer 16

blurring of the cortical margins of the subchondral bone

Answer 17

Chorea and indolent carditis can last up to 6 mos

Answer 18

Carbamazepine, Sodium Valproate

Answer 19

Without carditis - 5 yrs after last attack or until 21 yrs old which ever is longer With carditis,no valvular dse- 10 yrs after last attack or until 21 yrs old which ever is longer With residual valvular dse- 10 yrs after last attack or until 40 yrs old which ever is longer

Answer 20

Hip and Hand (50%)

Answer 21

Fibrillation of cartilage surface

Answer 22

Pai and swelling of 1st MTP

Answer 23

5-6 mg/dL or < 300-360 umol/L

Answer 24

wrist , PIP, MCP

Answer 25

Discoid Circular lesions with slightly raised, scaly, hyperpigmented erythematous rims and depigmented, atrophic centers

Answer 26

normocytic normochromic anemia

Answer 27

Cardiac catheterization

Answer 28

ILD monitor with PFTs every 6 months NOT recommended for monitoring: HRCT

Answer 29

Anti-Jo1 Antisynthetase syndrome- presence of myositis, non erosive arthritis, Raynauds, mechanic hands, fever

Answer 30

Eosinophilia > 1000cells/uL

Answer 31

3rd gen IV + Vanco

Answer 32

Periarticular osteopenia

Answer 33

polyrthritis (60-75%) next is carditis (50-60%)

Answer 34

reactive arthritis and psoriatic arthritis

Answer 35

peripheral

Answer 36

supraspinatus

Answer 37

cervical Same with RA

Answer 38

P. jiroveci

Answer 39

Anti TNF a Also the drugs that are most useful for psoriatic arthritis

Answer 40

3-16 weeks Cyclophosphamide + GC are given early to px with renal biopsy of ISN III and IV --> reduce progression to ESRD and death

Answer 41

arthritis, urethritis and conjunctivitis

Answer 42

Acute anterior uveitis

Answer 43

Pulmonary tissue

Answer 44

Cyclophosphamide + Glucocorticoids same with MPA GPA is AKA Wegeners

Answer 45

HLA B*5801

Answer 46

Losartan, Amlodipine, Fenofibrate, SGLT2i

Answer 47

Raynaud's phenomenon

Answer 48

cognitive dysfunction

Answer 49

hyaline articular cartilage loss

Answer 50

Flexion, ABduction, External Rotation Pathologies at the hip, lumbar and sacroiliac region

Answer 51

The entry criterion for the new classification criteria requires the occurrence of at least one episode of peripheral joint or bursal swelling, pain, or tenderness.

Answer 52

>Hyperuricemia cannot be corrected by nonpharmacological interventions >Consider in those with CKD Stage >3, serum urate levels >9mg/dL, urate nephrolithiasis >More than 2 gout attacks yearly >Those who already has subcutaneous tophi or chronic gouty arthritis >Radiographic evidence of gout arthritis

Answer 53

Chondrocalcinosis

Answer 54

anti dsdna antismith--> does not correlated with dse activity

Answer 55

Anti Ro Also assoc in neonatal lupus heart block

Answer 56

Anti histone

Answer 57

Myocarditis, Libman Sacks endocarditis however both not included in SLICC

Answer 58

Repeat ANA, add dsDNA and anti Ro if all negative- not SLE

Answer 59

MMF/ Cyclophosphamide in SLE nephritis, may also add calcineurin inhibitors like tacrolimus

Answer 60

cyclophosphamide

Answer 61

Quinacrine

Answer 62

DVT, Stroke

Answer 63

Valvular damage affecting mitral valves and aortic valves eg. MR and AR

Answer 64

Positive throat swab/ rapid antigen Anti ASO, anti DNAse

Answer 65

Antibiotics: Amoxicillin, 50 mg/kg [maximum, 1 g] daily, for 10 days OR Benzathine Pen G 1.2 million units IM Salicylates and NSAIDS- Aspirin 50-60mg/kg per day Max 80-100mg/kg/day divided into 4-5 doses; Decrease dose after 2 weeks to 50-60mg/kg/day for 2-4 weeks Others: Naproxen 10-20mg/kg/day Note: Salicylates and NSAIDs - no value for carditis and chorea

Answer 66

neutropenia, splenomegaly, and nodular RA

Answer 67

P. gingivalis

Answer 68

Synovial inflammation and proliferation, focal bone erosions, thinning of articular cartilage

Answer 69

anti CCP However, its diagnostic specificity approaches 95%, so a positive test for these antibodies in the setting of an early inflammatory arthritis is useful for distinguishing RA from other forms of arthritis

Answer 70

Infliximab

Answer 71

(1) arthritis of the DIP joints (2) asymmetric oligoarthritis (3) symmetric polyarthritis similar to RA (4) axial involvement (spine and sacroiliac joints) (5) arthritis mutilans (3)- most common, occurs in 40%

Answer 72

DIP involvement, classic “pencil-in-cup” deformity; marginal erosions with adjacent bony proliferation (“whiskering”); small-joint ankylosis; osteolysis of phalangeal and metacarpal bone, with telescoping of digits; periostitis, “ray” distribution of lesions.

Answer 73

Tofacitinib

Answer 74

Secukinumab and ixekizumab, monoclonal antibodies to IL-17A; SEventeen ustekinumab, a monoclonal antibody to the shared IL-23/IL-12p40 subunit.

Answer 75

Upper respiratory tract involvement Lower respiratory tract involvement Glomerulonephritis

Answer 76

Glomerulonephritis does NOT have granulomas and pulmonary involvement

Answer 77

Necrotizing **small**-vessel vasculitis + Granulomas (intravascular or extravascular)

Answer 78

ENT particularly sinusitis

Answer 79

Glomerulonephritis

Answer 80

Pulmonary infiltrates

Answer 81

Heart disease (myocardial involvement) 2DECHO should be done in ALL newly diagnosed patients

Answer 82

Leukocytosis with neutrophilic predominance

Answer 83

Nodular skin lesions Painful testes Nerve/muscle

Answer 84

Angiographic studies

Answer 85

GI complaints (bowel infarction, perforation) Cardiovascular causes

Answer 86

Temporal artery Other name for giant cell arteritis is Temporal arteritis

Answer 87

subclavian artery

Answer 88

Prednisone (40-60 mg OD x 1 month then taper) (first-line treatment for general presentation) Methylprednisolone (1 gram OD x 3 days) (first-line treatment for ocular presentation)

Answer 89

Prednisone 40-60 mg OD for symptomatic treatment

Answer 90

Postcapillary venules

Answer 91

Palpable purpura

Answer 92

Lower extremities

Answer 93

Prednisone

Answer 94

Cyclophosphamide

Answer 95

Cogan syndrome

Answer 96

Coronary arteries – beadlike aneurysms and thromboses

Answer 97

Hydralazine Propylthiouracil

Answer 98

Palpable purpura of the lower extremities

Answer 99

Gottron papules NOT sign Gottron signs also seen in other areas like elbows , knees, ankles

Answer 100

Anti-TIF1 (p155) Anti-NXP2

Answer 101

Anti-HMGCR

Answer 102

Anti-cytosolic 5’nucleotidase 1A (anti-cN-1A)

Answer 103

flexor digitorum profundus, vastus medialis, vastus lateralis

Answer 104

High-dose glucocorticoids (0.75 – 1 gram mg/kg/day- First-line treatment to steroid-responsive inflammatory myopathies Methotrexate – second-line treatment Exception: IBM --> OT and PT

Answer 105

c. A 38/M with serum uric acid level of 14 mg/dL Since > 9 Why other options are wrong a. A 55/M with documented radio-opaque kidney stone--> radiolucent b. A 48/M 2 weeks after resolution of his first acute gouty attack --> should be at least 2 attacks d. A 50/M with concomitant osteoarthritis- not indicated

Answer 106

a. Her pattern of joint involvement makes heritability more likely Heritability of OA o Hip and hand (50%) o Knee (up to 30%) o Generalized OA (rarely inherited) * Growth differentiation factor 5 (GDF5) polymorphism is an associated mutation

Answer 107

d. Hip, knee and hand Obesity and OA * Risk factor for knee, hip, and hand OA * Obese persons have more severe OA symptoms * Not just a consequence of inactivity due to OA * Stronger risk factor among women * In women, risk is correlated to weight in a linear fashion * Weight loss reduces risk for symptomatic OA * Adipokines may also play a role (note risk for hand OA) Repetitive joint use and exercise * Exercise is not associated with OA risk in most people * Joint injury – involved joints are at greater risk with certain types of exercise * Recreational runners – modestly increased risk for hip OA (but not knee OA) * Professional runners – high risk for hip and knee OA * Workers performing repetitive tasks for many years – high risk for involved joints

Answer 108

b. Decrease in proteoglycan, increase in water content Pathology * Cartilage surface fibrillation → non-uniform erosions → extension to bone * Injury → chondrocyte mitosis and clustering → catabolic > synthetic activity → negative charges of proteoglycans are exposed → cartilage swelling → vulnerability to injury * Activation of osteoclasts and osteoblasts in subchondral bone * Osteophyte formation at the joint margin – radiologic hallmark of OA * Synovial inflammation and proliferation accelerate matrix destruction * Capsular edema and fibrosis *Basic calcium phosphate and calcium pyrophosphate dihydrate crystals in end-stage OA → synovitis

Answer 109

c. Neuropathy Neuropathic joint disease (Charcot joint) is a progressive destructive arthritis associated with loss of pain sensation, proprioception, or both. Diabetes mellitus is the most frequent cause of neuropathic joint disease. Radiographs and physical exam demonstrate loss of the arch due to bony fragmentation and dislocation in the midfoot.

Answer 110

c. Quadriceps exercise

Answer 111

b. Osteoarthritis Hand OA affects the distal interphalangeal joints (Heberden’s nodes) and the proximal interphalangeal joints (Bouchard’s nodes). Another common site of bony enlargement in the hands is the thumb base. Sources of pain in osteoarthritis * Cartilage loss is NOT accompanied by pain, unless if with neurovascular invasion in advanced OA * Innervated structures: o Synovium, ligaments, joint capsule, muscles, subchondral bone * Severity of X-ray changes does not correlate well with pain severity Likely sources of pain * Synovitis * Joint effusion * Bone marrow edema (from loading-related bone injury) * Periarticular sources including bursae (ie. anserine bursitis, iliotibial band syndrome) * Peripheral and central sensitization

Answer 112

d. Morning stiffness for 30 minutes, pain worsens with prolonged activity Clinical features * Pain is initially episodic, activity-related, triggered by overactive use of involved joint * Can become continuous pain at progression * Morning stiffness <30mins

Answer 113

a. Anserine bursitis Bursitis occurs commonly around knees and hips. A physical examination should focus on whether tenderness is over the joint line (at the junction of the two bones around which the joint is articulating) or outside of it. Anserine bursitis, medial and distal to the knee, is an extremely common cause of chronic knee pain that may respond to a glucocorticoid injection.

Answer 114

c. Osteoarthritis For hip pain, OA can be detected by loss of internal rotation on passive movement, and pain isolated to an area lateral to the hip joint usually reflects the presence of trochanteric bursitis. Ankylosing spondylitis: (+) Schober test, (+) sacroiliitis

Answer 115

c. Prescribe an oral analgesic in OA, radiographic findings correlate poorly with the presence and severity of pain. Further, in both knees and hips, radiographs may be normal in early disease as they are insensitive to cartilage loss and other early findings.

Answer 116

b. <1,000 /uL Workup for osteoarthritis * No routine blood test indicated, unless inflammatory arthritis is suspected * Synovial fluid WBC <1000/uL Indications for radiographs * Symptoms or PE findings are atypical for OA * Pain persists after initial treatment * Imaging correlates poorly with presence/severity of symptoms * May be normal in early knee and hip OA MRI * Not part of diagnostic workup for OA * Meniscal tears, cartilage and bone lesions are common in knee OA * These findings are also common in older persons who are asymptomatic * MRI findings almost never warrant a change in therapy ** additional note Prominent nocturnal pain in the absence of end-stage OA merits a distinct workup

Answer 117

d. Shift to another sport that does not trigger pain Treatment * Reassurance and non-pharmacologic tx for mild and intermittent symptoms * Add pharmacotherapy for ongoing, disabling pain o Adjunct for symptomatic relief only o Does not alter disease process * The simplest treatment for many patients is to avoid activities that precipitate pain.

Answer 118

a. Use the hand contralateral to the affected hip, and the elbow bent by around 15 degrees Ways to decrease focal load on joint * Avoid painful activities * Improve strength and conditioning of bridging muscles * Redistribute load using a brace or splint * Unload the joint during weight bearing by using a crane in the opposite hand * Weight loss – each pound lost has a 3 to 6-fold multiplier effect in unloading knees and hips

Answer 119

c. Water aerobics Low-impact exercises (water aerobics, water resistance training) are better tolerated Impact loading exercised (running, treadmill) are less tolerable Tai chi may be effective for knee OA

Answer 120

b. Splinting In hand joints affected by OA, splinting, by limiting motion, often minimizes pain for patients with involvement especially in the base of the thumb. There is no strong evidence that patients with hand OA benefit from therapeutic exercise.

Answer 121

c. Medial unloader knee brace Correction of malalignment can lessen pain in OA * Varus-valgus malalignment - surgical correction or fitted knee brace (must not slip) * Patellar malalignment - patellar brace or tape Other non-pharmacologic approaches to lessen pain (adjuncts) * Neoprene sleeves – questionable effect on malalignment * Acupuncture

Answer 122

d. Intraarticular steroid Topical NSAIDs are slightly less efficacious than oral agents, but have far fewer GI and systemic side effects. Glucocorticoid injections are useful to get patients over acute flares of pain, but their effects usually last less than 3 months. Hyaluronic acid injections can be given but there is controversy as to whether they have efficacy versus placebo

Answer 123

a. Topical NSAID Topical NSAIDs are slightly less efficacious than oral agents, but have far fewer GI and systemic side effects. * Guidelines recommend against the use of glucosamine or chondroitin for OA. Large trials have failed to show that these compounds relieve pain in persons with disease. * Opiates - Common side effects include dizziness, sedation, nausea or vomiting, dry mouth, constipation, urinary retention, and pruritus.

Answer 124

d. Total knee replacement Arthroscopic debri¬dement and lavage – no better than sham surgery for relief of pain or disability Partial meniscectomy in persons with OA and a symptomatic meniscal tear does not relieve knee pain or improve function or even lead to resolution of catching or locking of the knee Chondrocyte transplantation has not been found to be efficacious in OA, perhaps because OA includes pathology of joint mechanics, which is not corrected by chondrocyte transplants.

Answer 125

a. Optimal non-surgical therapy Although MRI may reveal the extent of pathology in an osteoarthritic joint, it is not indicated as part of the diagnostic workup. Findings such as meniscal tears and cartilage and bone lesions occur not only in most patients with OA in the knee, but also in most older persons without joint pain. MRI findings almost never warrant a change in therapy.

Answer 126

b. ANA, CBC with platelet, urinalysis Discoid lupus erythematosus (DLE) is the most common chronic dermatitis in lupus; lesions are roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented, atrophic centers in which all dermal appendages are permanently destroyed

Answer 127

Advise to monitor for symptoms The presence in an individual of multiple autoantibodies without clinical symptoms should not be considered diagnostic for SLE, although such persons are at increased risk.

Answer 128

c. Decrease prednisone dose In SLE, myositis with clinical muscle weakness, elevated creatine kinase levels, positive magnetic resonance imaging (MRI) scan, and muscle necrosis and inflammation on biopsy can occur, although most patients have myalgias without frank myositis. Glucocorticoid therapies (commonly) and antimalarial therapies (rarely) can cause muscle weakness; these adverse effects must be distinguished from active inflammatory disease.

Answer 129

b. Anti-dsDNA Titers of anti-dsDNA vary over time. In some patients, increases in quantities of anti-dsDNA herald a flare, particularly of nephritis or vasculitis, especially when associated with declining levels of C3 or C4 complement. Antibodies to Sm are also specific for SLE and assist in diagnosis; anti-Sm antibodies do not usually correlate with disease activity or clinical manifesta¬tions.

Answer 130

b. ANA and Anti-histone Drug-induced lupus: This is a syndrome of positive ANA associated with symptoms such as fever, malaise, arthritis or intense arthralgias/myalgias, serositis, and/ or rash. It is predominant in whites, has less female predilection than SLE, rarely involves kidneys or brain, is rarely associated with anti-dsDNA, is commonly associated with antibodies to histones, and usually resolves over several weeks after discontinuation of the offending medication.

Answer 131

c. Lipid profile Control of hypertension and appropriate prevention strategies for atherosclerosis, including monitoring and treatment of dyslipidemias, management of hyperglycemia, and management of obesity, are recommended. Statin therapies reduce all-cause deaths in SLE patients and should be considered in patients with elevated LDL or total cholesterol levels.

Answer 132

Belimumab human monoclonal antibody that inhibits B-cell activating factor, also known as B-lymphocyte stimulator

Answer 133

Latent TB via PPD Hepatitis B Anti TNF also inc risk for fungal infections

Answer 134

2-3 if unprovoked or if with arterial thrombosis on VKA and low dose aspirin 3-4 if with arterial thrombosis and only on VKA

Answer 135

Aspirin + LMWH

Answer 136

Diffuse microangiopathy Inflammation and autoimmunity Visceral and vascular fibrosis in multiple organs

Answer 137

Widespread capillar loss Obliterative microangiopathy Fibrosis in the skin

Answer 138

Non specific interstitial pneumonia

Answer 139

loss of spinal mobility with limitation of anterior and lateral flexion and extension of the lumbar spine and of chest expansion measured by Schober test (decreased if less than 5)

Answer 140

First line: NSAIDs May also use >anti-TNF-a --> induce rapid and sustained reduction in dse activity > Secukinumab --> IL-17a antibody > Sulfasalazine--> modest benefit for PERIPHERAL arthritis No role: MTX, oral glucocorticoids, gold compound MTX is for RA not AS

Answer 141

ReA: lower extremity symptoms predominate Gonococcal : LE = UE

Answer 142

ReA: lower extremity symptoms predominate PsA: gradual, primarily uppe extremities

Answer 143

a. Chronic inflammation Calcinosis cutis: Dystrophic calcifications in the skin, subcutaneous, and soft tissues (calcinosis cutis) in the presence of normal serum calcium and phos-phate levels occur in up to 40% of patients, most commonly in those with long-standing anti-centromere antibody-positive lcSSc. These deposits occur when calcium precipitates in tissue damaged by inflammation, hypoxia, or local trauma. Common locations include the finger pads, palms, extensor surfaces of the forearms, and the olecranon and prepatellar bursae

Answer 144

b. Perform nail microscopy Primary Raynaud’s disease is a benign condition that must be differentiated from early or limited SSc. Nailfold microscopy is particularly helpful in this situation, because in contrast to SSc, nail¬fold capillaries are normal.

Answer 145

c. Chest Because pigment loss spares the perifollicular areas, the skin may have a “salt-and-pepper” appearance, most prominently on the scalp, upper back, and chest.

Answer 146

b. Pulmonary function test, 2D echocardiogram The diagnosis of SSc is made primarily on clinical grounds and is generally straightforward in patients with established disease. Patients with PAH are often asymptomatic in early stages. In light of the poor prognosis of untreated PAH and better therapeutic response in patients with early diagnosis, all SSc patients should be screened for PAH at initial evaluation, followed by annual evaluation. Doppler echocardiography provides a noninvasive screening method for estimating the pulmonary arterial pressure. Pulmonary involvement can remain asymptomatic until it is advanced. Pulmonary function testing (PFT) is relatively sensitive for detecting early pulmonary involvement. Chest radiography is relatively insensitive for the detection of early ILD

Answer 147

a. Blood pressure Risk factors for scleroderma renal crisis include African-American race, male sex, and diffuse or progressive skin involvement. Palpable tendon friction rubs, pericardial effusion, new unexplained anemia, and thrombocytopenia may be harbingers of impending scleroderma renal crisis. High-risk patients with early SSc should monitor their blood pressure daily.

Answer 148

a. 2D echocardiography * Common associated conditions: myocarditis, ILD, malignancy, vasculitis, other CTDs

Answer 149

b. Colonoscopy There is a higher risk for malignancy in adult onset cases, ~15% within the first 2–3 years. Anti-TIF1 (or p155) antibodies and anti-NXP2 antibodies are associated with an increased risk of cancer. EMG findings are non-specific and can be seen in other myopathies

Answer 150

b. Middle aged women Gout is a metabolic disease that most often affects middle-aged to elderly men and postmenopausal women. Women represent only 5–20% of all patients with gout. Most women with gouty arthritis are postmenopausal and elderly, have osteoarthritis and arterial hypertension that causes mild renal insufficiency, and usually are receiving diuretics. Pre-menopausal gout is rare.

Answer 151

b. CPPD commonly occurs in patients with pre-existing joint damage The deposition of CPP crystals in articular tissues is most common in the elderly. > 80% of patients are >60 years old and 70% have pre-existing joint damage from other conditions. A minority of patients with CPPD arthropathy have metabolic abnormalities (such as hyperparathyroidism, hemochromatosis, hypophosphatasia, and hypomagnesemia) or hereditary CPP disease. The presence of CPPD arthritis in individuals aged <50 years should lead to consideration of these metabolic disorders and inherited forms of disease.

Answer 152

d. Ankle The most common sites of apatite deposition include bursae and tendons in and/or around the knees, shoulders, hips, and fingers.

Answer 153

c. Menstruation Several events may precipitate acute gouty arthritis: dietary excess, trauma, surgery, excessive ethanol ingestion, hypouricemic therapy, and serious medical illnesses such as myocardial infarction and stroke

Answer 154

d. Bipyramidal shaped strongly birefringent crystals: Calcium Oxalate deposition Disease Needle- and rod-shaped negative birefringent crystals are consistent with gout. * Rod-shaped, and rhomboid crystals that are weakly positively or nonbirefringent crystals are consistent with CPPD. * Small nonbirefringent globules that stain purplish with Wright’s stain and bright red with alizarin red S are consistent with Calcium appatite deposition disease. * Calcium oxalate deposition disease crystals have variable shape and variable birefringence to polarized light. The most easily recognized forms are bipyramidal, have strong birefringence and stain with alizarin red S.

Answer 155

b. Linear radio dense deposits within articular hyaline carriage: CPPD CPPD shows radiographs or ultrasound findings of punctate and/or linear radiodense deposits within fibrocartilaginous joint menisci or articular hyaline cartilage (chondrocalcinosis). This can also be seen in Calcium oxalate deposition disease in some patients with chronic renal disease. Radiographic findings of gout include cystic changes, well-defined erosions with sclerotic margins (often with overhanging bony edges), and soft tissue masses are characteristic radiographic features of advanced chronic tophaceous gout. Ultrasound may aid earlier diagnosis by showing a double contour sign overlying the articular cartilage. Intra- and/or periarticular calcifications with or without erosive, destructive, or hypertrophic changes may be seen on radiographs of Calcium appatite deposition disease

Answer 156

b. Clarithromycin Colchicine should be given in lower doses to the patients with P glycoprotein or CYP3A4 inhibitors such as clarithromycin that can increase toxicity of colchicine.

Answer 157

d. Extraarticular manifestation are significant and may occur prior to the onset of arthritis. Incidence of RA increases between 25 and 55 years of age, after which it plateaus until the age of 75 and then decreases. Patients often complain of early morning joint stiffness lasting more than 1 h that eases with physical activity. Presenting symptoms typically involve the small joints of the hands and feet, usually in a symmetric distribution. Once the disease process of RA is established, the wrists, metacarpophalangeal (MCP), and proximal interphalangeal (PIP) joints stand out as the most frequently involved joints. Distal interphalangeal joint are usually an manifestation as compared to the ankle and mid tarsal region but is not one of the most common involved joints. Extraarticular manifestations may develop during the clinical course of RA in up to 40% of patients, even prior to the onset of arthritis

Answer 158

a. Anakinra Based on recent evidence on the essential role of the inflammasome and interleukin 1β (IL-1β) in acute gout, daily anakinra has been used when other treatments have failed or were contraindicated

Answer 159

d. Lumbar spine The wrists, metacarpophalangeal (MCP), and proximal interphalangeal (PIP) joints stand out as the most frequently involved joints in rheumatoid arthritis. Elbows are commonly involved, and can have rheumatoid nodules. In the feet, metatarsophalangeal (MTP) involvement are frequent and is an early feature of disease while chronic inflammation of the ankle and midtarsal regions usually comes late. Large joints such as knees and shoulders are also often affected in established disease. Cervical spine involvement was more prevalent before and can cause compressive myelopathy and neurologic dysfunction. Thoracolumbar spine are rarely affected.

Answer 160

c. Bilateral interstitial infiltrates All are potential pulmonary manifestations in RA. Pleuritis is the most common and presents with pleuritic chest pain and dyspnea. Pulmonary nodules maybe solitary or multiple. ILD are found in up to 12% of RA patients and is heralded by symptoms of dry cough and progressive shortness of breath. Diagnosis is readily made by high resolution chest CT scan, which shows infiltrative opacification in the periphery of both lung. Pulmonary nodules associated with rheumatoid arthritis may be solitary or multiple. They often occur in conjunction with cutaneous nodules. Bronchiectasis and respiratory bronchiolitis may also be due to rheumatoid arthritis.

Answer 161

c. Lymphoma Felty syndrome, typically occurring in late-stage poorly controlled disease, is characterized by the triad of neutropenia, splenomegaly, and rheumatoid nodules Large cohort studies have shown a two- to fourfold increased risk of lymphoma in RA patients compared with the general population. The most common histopathologic type of lymphoma is a diffuse large B-cell lymphoma. The risk of developing lymphoma increases if the patient has high levels of disease activity or Felty syndrome

Answer 162

a. Subcutaneous nodules are the most common extraarticular manifestation of rheumatoid arthritis Subcutaneous nodules have been reported to occur in 30–40% of patients and more commonly in those with the highest levels of disease activity. the nodules are generally firm; nontender; and adherent to periosteum, tendons, or bursae; developing in areas of the skeleton subject to repeated trauma or irritation such as the forearm, sacral prominences, and Achilles tendon. They may also occur in the lungs, pleura, pericardium, and peritoneum. Nodules are typically benign, although they can be associated with infection, ulceration, and gangrene.

Answer 163

a. The preclinical stage of rheumatoid arthritis is characterized by a break-down in self-tolerance of immune cells The pathogenic mechanisms of synovial inflammation are likely to result from a complex interplay of genetic, environmental, and immunologic factors that produces dysregulation of the immune system and a breakdown in self-tolerance. Smoking, silicone dust, mineral oil, periodontal pathogens are some of the environmental factors described. Synovial CD4+ T cells differentiate into TH1 and TH17 cells, each with their distinctive cytokine profile. CD4+ TH cells in turn activate B cells, some of which are destined to differentiate into autoantibody-producing plasma cells. Immune complexes, possibly comprised of rheumatoid factors (RFs) and anti–cyclic citrullinated peptides (CCP) antibodies, may form inside the joint, activating the complement pathway and amplifying inflammation. T effector cells stimulate synovial macrophages and fibroblasts to secrete proinflammatory mediators, among which is tumor necrosis factor α (TNF-α). TNF-α upregulates adhesion molecules on endothelial cells, promoting leukocyte influx into the joint and stimulates production of other inflammatory mediator. TNF-α also has a role in regulating other mediators in bone destruction and bone formation.

Answer 164

a. Synovial lining hyperplasia The pathologic hallmarks of RA are synovial inflammation and proliferation, focal bone erosions, and thinning of articular cartilage. Chronic inflammation leads to synovial lining hyperplasia and the formation of pannus, a thickened cellular membrane containing fibroblast like synoviocytes and granulation-reactive fibrovascular tissue that invades the underlying cartilage and bone. the rest of the pathologic findings are consistent with osteoarthritis.

Answer 165

d. Testing for both rheumatoid factor and anti-CCP has added value Serum RF and anti-CCP antibodies is important in differentiating RA from other polyarticular diseases. RF (IgM subtype) is found in 75-80% of RA patients; therefore, a negative result does not exclude the presence of this disease. It is also found in other diseases (Sjogren, SLE, type II mixed cryoglobulinemia, subacute bacterial endocarditis and hepatitis B and C) as well as in 1-5% of normal population. anti-CCP is as sensitive as rheumatoid factor, but more specific. Therefore positive anti-CCP in early inflammatory arthritis distinguishes RA from other arthritis. Both RF and anti-CCP has prognostic significance, with anti-CCP showing the most value for predicting worse outcomes. Lastly, there is some incremental value in testing for the presence of both RF and anti-CCP, as some patients with RA are positive for RF but negative for anti-CCP and vice versa.

Answer 166

d. Periarticular osteopenia Joint imaging is for diagnosing RA and for tracking progression of joint damage. Plain X-ray is the most common imaging modality used, but is limited to visualization of bony structures and inferences on the state father articular cartilages based on the amount of joint space narrowing. Classically in RA, the initial radiographic finding is periarticular osteopenia. Other findings on plain radiographs include soft tissue swelling, symmetric joint space loss, and subchondral erosions, most frequently in the wrists and hands (MCPs and PIPs) and the feet (MTPs). MRI and ultrasound techniques offer the added value of detecting changes in the soft tissues such as synovitis, tenosynovitis, and effusions, as well as providing greater sensitivity for identifying bony abnormalities

Answer 167

d. Ultrasound offers the greatest sensitivity for detecting synovitis and joint effusion MRI and ultrasound offer added value over x-ray in detecting changes in the soft tissues such as synovitis, tenosynovitis, and effusions, as well as providing greater sensitivity for identifying bony abnormalities. MRI offers the greatest sensitivity for detecting synovitis and joint effusions, as well as early bone and bone marrow changes. These soft tissue abnormalities often occur before osseous changes are noted on x-ray. Presence of bone marrow edema has been recognized to be an early sign of inflammatory joint disease and can predict the subsequent development of erosions on plain radiographs as well as MRI scans. Ultrasound can detect more erosions than plain radiography. It can also reliably detect synovitis, including increased joint vascularity indicative of inflammation. It is technician dependent; however, it does offer the advantages of portability, lack of radiation, and low expense relative to MRI, factors that make it attractive as a clinical tool.

Answer 168

d. Continue current DMARD and add on anti-TNF therapy Failure to achieve adequate improvement with DMARD monotherapy calls for a change in DMARD therapy, usually a transition to an effective combination regimen. Effective combinations include methotrexate, sulfasalazine, and hydroxychloroquine (oral triple therapy); methotrexate and leflunomide; and methotrexate plus a biological

Answer 169

a. Hydroxychloroquine Conventional DMARDs include hydroxychloroquine sulfasalazine, methotrexate, and leflunomide and all have ability to slow or prevent structural progression of RA except for hydroxychloroquine has not been shown to delay radiographic progression of disease and thus is not considered to be a true DMARD.

Answer 170

b. Etanercept Anakinra is a recombinant IL-1 receptor antagonist. Abtacept inhibits the co-stimulation of T cells by blocking CD28-CD80/86 interactions. Tocilizumab is a humanized monoclonal antibody directed against IL-6 receptor.

Answer 171

a. Rituximab Reactivation of viral hepatitis has been observed in association with some of the DMARDs, such as rituximab. Antiviral prophylaxis is indicated to prevent hepatitis flares and complications.

Answer 172

b. Thoracic and lumbar spine

Answer 173

d. Anti-CCP antibody

Answer 174

a. Dermatomyositis

Answer 175

d. lumbar spine unlike RA and PsA

Answer 176

a. Antimalarials

Answer 177

b. myocarditis

Answer 178

d. Telangiectasias

Answer 179

a.Endoscopic laser cryotherapy

Answer 180

b. Cyclophosphamide

Answer 181

c.Anti-proteinase ANCA

Answer 182

b.Polymyositis IBM usually >50 and males

Answer 183

Lower respiratory tract involvement Upper respiratory tract involvement Kidney involvement Note GPA was also known as Wegener

Answer 184

MPA does not have upper respiratory tract involvement and does not present with pulmonary nodules MPA does not have granulomas

Answer 185

Mononeuritis multiplex

Answer 186

Dermatomyositis dermatoMYXO

Answer 187

Inclusion Body Myositis M>F CK levels can be normal or only slightly elevated + antibodies against cN-1A ENDOmysial inflammatory infiltrates Most sensitive stain for inclusion: p62

Answer 188

Muscle MRI may show predilection for flexor digitorum profundus, vastus medialis and lateralis with sparing of rectus femoris

Answer 189

PT and OT Since IBM does not typically respond to any known immunotherapy

Answer 190

Methotrexate

Answer 191

Azathioprine

Answer 192

Cyclosporine

Answer 193

Mycophenolate mofetil

Answer 194

Knee and Spine Farmers: Hip

Answer 195

Recreational: hip only Elite: Hip and Knee

Answer 196

A. Duration of symptoms of greater or less than 8 weeks should be 6 weeks

Answer 197

D. Pulmonary Function Test with DLCO

Answer 198

d. ProinIammatory cytokines act on synovial fibroblasts causing synovial membrane infIammation and damage to the soft tissues and cartilage Patient has RA a. Crystal induced mediated release of proinfIammatory cytokines causing soft tissue and joint infIammation --> crystal induced b. Deterioration of hyaline cartilage due to damage of chondrocytes as a result of infIammatory cytokine mediated release of matrix metalloproteinases (MMPs) --> OA c. Enthesitis, which is inIammation at the site where ligaments, tendons, and joint capsules attach to the bone, is the prominent pathologic lesion --> spondyloarthropathies

Answer 199

b. Limited systemic sclerosis CREST --> Limited SCC

Answer 200

a. Adalimumab Adalimumab is a TNF-α inhibitor, and TNF-α plays a critical role in the pathogenesis of PsA. TNF-α inhibitors are first-line biologics for moderate to severe PsA, especially when there is axial involvement, enthesitis, or peripheral arthritis. Adalimumab has proven efficacy for both joint and skin symptoms in PsA.

Answer 201

b. Anti-centromere antibody Anti-centromere antibody (ACA) is associated with limited cutaneous systemic sclerosis (lcSSc) and a lower risk of developing Scleroderma renal crisis

Answer 202

c. Prednisone, Hydroxychloroquine and Azathioprine These 3 are safe in pregnancy

Answer 203

b. Urinary excretion of >800mg/24h uric acid suggest purine overproduction

Answer 204

c. Probenecid

Answer 205

d. High dose steroids, hydroxychloroquine and mycophonelate mofetil Class IV

Answer 206

a. Anti-cardiolipin APAS headache due to arterial thrombosis mottled skin = livedo reticularis

Answer 207

b. Granulomatosis with Polyangiitis previously known: Wegeners Triad: URT + LRT +kidney involvement

Answer 208

a. Benzathine Penicillin 1.2M units IM This patient presents with symptoms and signs consistent with acute rheumatic fever (ARF): Migratory polyarthritis: Joint pain moves from one joint to another (ankle → knee → wrist → elbow), which is characteristic of ARF. High-grade fever: Suggests systemic inflammation. Elevated ESR (52 mm/h): Indicates active inflammation.

Answer 209

a. Arteriography Takayasu Arteritis The diagnosis is confirmed by the characteristic pattern on arteriography, which includes irregular vessel walls, stenosis, poststenotic dilation, aneurysm formation, occlusion, and evidence of increased collateral circulation. Complete imaging of the aorta and its major branches by magnetic resonance or computed tomography arteriography should be obtained to fully delineate the distribution and degree of arterial disease. Because of the involvement of the large vessels, tissue is rarely available as a means of diagnosis and obtained only if vascular surgery is necessary

Answer 210

b. Ischemic necrosis of the bone This patient has systemic lupus erythematosus (SLE) and has been self-medicating with high-dose prednisone (30 mg/day) for years without medical supervision. These factors strongly predispose her to avascular necrosis (AVN) (also called ischemic necrosis or osteonecrosis of the bone).

Answer 211

c. Application of ice pack on affected joint Colchicine should be 1 mg then 0.5 mg 1 hr after

Answer 212

d. Pseudo-obstruction Pseudo-obstruction: This gastrointestinal complication, caused by dysmotility due to smooth muscle atrophy and fibrosis, is more frequently seen in lcSSc. Gastrointestinal manifestations, particularly involving the esophagus and small intestine, are hallmark features of lcSSc.

Answer 213

c. Pregnancy

Answer 214

a. Phosphate lowering agents

Answer 215

c. Methotrexate Methotrexate: A disease-modifying antirheumatic drug (DMARD) commonly used as a second-line agent in inflammatory myopathies to reduce inflammation and allow tapering of steroids. It is highly effective for both muscle weakness and systemic symptoms. Other second line: Azathioprine, Mycophenolate, IVIG, Rituximab

Answer 216

c. MRI MRI = more useful for early diagnosis of AS

Answer 217

c. Loss of aggrecan, unfurling of collagen matrix, loss of type 2 collagen The patient's clinical presentation of symmetric hand and knee pain, worsened by movement, morning stiffness lasting less than 30 minutes, and relief with exercise, is consistent with osteoarthritis (OA)

Answer 218

d. Venous thrombosis associated with APAS Livedo reticularis = venous

Answer 219

c. Severe cervical spine involvement The patient's symptoms and physical examination findings are suggestive of psoriatic arthritis (PsA). This condition is associated with HIV and features a combination of arthritis, enthesitis (e.g., tender Achilles tendon), and skin and nail changes typical of psoriasis (e.g., erythematous scaly papules, nail pits, and discoloration). Psoriatic arthritis can have both peripheral and axial joint involvement. Axial Imaging Findings in Psoriatic Arthritis: Severe cervical spine involvement: Psoriatic arthritis commonly affects the axial skeleton, particularly the cervical spine, often leading to spondylitis and severe cervical involvement. Imaging typically shows syndesmophytes, asymmetrical sacroiliitis, and cervical spine changes, such as fusion or erosion.

Answer 220

c. During flares, needle-shaped MSU is usually present both intracellularly and extracellulary

Answer 221

d. No diagnostic testing correlates with pain and disability

Answer 222

C. No additional treatment The patient has Class VI Advanced Sclerotic Lupus Nephritis. In general, class III and IV disease, as well as class V accompanied by III or IV disease, should be treated with aggressive immunosuppression if possible because there is a high risk for ESRD if patients are untreated or undertreated. In contrast, treatment for lupus nephritis is not recommended in patients with class I or II disease or with extensive irreversible changes (class VI).

Answer 223

d. Pulmonary Blastomycosis

Answer 224

B. Aspirin plus LMWH In pregnant women with a history of obstetric APS, combination treatment with low dose aspirin (LDA) and prophylactic dose of low molecular-weight heparin (LMWH) is recommended, whereas in cases of thrombotic APS, LDA plus therapeutic LMWH dose should be administered

Answer 225

A. It is a highly heritable disease

Answer 226

B. Gradual depletion of aggrecan OA cartilage is characterized by gradual depletion of aggrecan, an unfurling of the tightly woven collagen matrix, and loss of type 2 collagen. With these changes comes increasing vulnerability of cartilage, which loses its compressive stiffness.

Answer 227

C. Flexor pollicis longus

Answer 228

C. MS mean gradient ≥4 mmHg