Rheumatology

Question 1

11 diagnostic criteria for SLE (need 4 out of 11)

Answer 1

1) Malar rash
2) Discoid lupus rash
3) Photosensitivity
4) oral/nasal mucocutaneous ulcers
5) NON-erosive arthritis (RA is erosive)
6) Nephritis
7) Encephalopathy
8) Pleuritis or pericarditis
9) cytopenia
10) Positive immunoserology: LE cells, Anti native DNA Ab, Anti-Sm Ab
11) Positive ANA titer

Question 2

Juvenile dermatomyositis

Type IV polymyositis/dermatomyositis is associated with childhood.

80-90% respond to steroids

Answer 2

clinical features include presence of vasculitis, calcinosis (Dystrophic calcinosis is the abnormal collection of calcium salts in or under the skin and in muscles or tendons, even when levels of calcium in the blood are normal. It occurs in some patients with dermatomyositis.)
, lack of association with childhood malignancy

Question 3

CREST syndrome

Answer 3

calcinoosis, raynaud’s, esophageal dysfunction, sclerodactyly. (thickening of the skin of the digits of the hands and feet. Three phases of skin changes are seen in scleroderma: the edematous phase, indurative phase, and atrophic phase), telangiectasia

Question 4

Collagen type affected in OI

Answer 4

Osteogenesis imperfecta, or brittle-bone disease, is an autosomal dominant disorder caused by a gene mutation producing type 1 collagen. Type 1 collagen is the most abundant collagen in the body found in scar tissue during healing. The bone mass in individuals with this disorder is diminished. Patients classically have blue sclera because of the lack of type 1 collagen formation in the eye allowing the choroidal veins to be seen.

Question 5

Which type of arthritis: include erosions, bony decalcification in and next to the involved joints, and symmetric joint-space narrowing (due to loss of articular cartilage)

Answer 5

RA

Question 6

asymmetric joint-space narrowing, osteophyte formation, subchondral bony sclerosis, osseous cysts, and loose bodies. Sclerosis of facet joints, anterior vertebral body osteophytes, and loss of intervertebral space in the spine….what type of arthritis

Answer 6

OA

Question 7

Erosion and periosteal changes at the insertion of the plantar fascia and Achilles tendon (“lover’s heel”)

Answer 7

reactive arthritis

Question 8

Bony erosions, syndesmophytes, and “pencil-in-cup” deformity of the distal interphalanx (DIP)

Answer 8

psoriatic arthritis

Question 9

small to medium artery vasculitis that commonly affects middle-aged males, associated with “saddle-nose” deformity, necrotizing granulomatous vasculitis affecting the respiratory tract, and focal segmental glomerulonephritis.

Answer 9

Wegener’s granulomatosis

Question 10

Medium vessel systemic vasculitis , 2:1 male: F, glomerulonephritis #1 cause of death, lungs SPARED, palpable purpura, mononeuritis multiplex, arthritis

with fever, night sweats, weight loss, skin ulcerations, tender nodules, and severe muscle and joint pains developing over months. THE LUNGS ARE NORMAL. ESR is ELEVATED and Hep B is positive. RF is negative. What is the next best step to reach a diagnosis?

Answer 10

Polyarteritis Nodosa (also seen in RA, SLE, Sjograns)

Question 11

small vessel, oral and genital ulcers, 20% have VTE

Answer 11

Behcets syndrome

Question 12

Large arteries, more common in females, tendenress in scalp and muscles of mastication, headache, associated with PMR, elev ESR, temporal bx

Answer 12

temporal arteritis (high dose steroids asap to avoid vision loss, asa 325 mg improves prognosis).

Question 13

autosomal dominant condition caused by FBN1 gene mutation located on chromosome 5. There is a defect in the fibrillin protein. Fibrillin is normally found in connective tissue and is necessary for formation of elastic fibers. Excess linear growth of long bones, arachnodactyly (long fingers, toes), and joint laxity are common skeletal findings. Cardiac conditions such as aortic root dilation and mitral valve pathology may be noted, dislocation of the lens in either or both eyes ectopia lentis seen in 50%-80%, and skeletal deformities such as pectus excavatum and scoliosis. The most potentially fatal effect is cardiac involvement, especially with aortic root dilatation and aortic dissection.

Answer 13

Marfan syndrome

Question 14

earliest radiographic sign of rheumatoid arthritis.

Answer 14

Diffuse periarticular osteopenia

Question 15

weakness or rupture of the terminal portion of the extensor hood, which holds the lateral bands in place at the PIP joint. There is initially PIP synovitis then a downward slippage of the lateral bands, causing flexion at the PIP joint.

Answer 15

boutinneres deformity……test with elson test where from a 90 degree flexed position over the edge of table patient tries to extend PIP against resistance, absence of extension at PIP and fixed extension at the distal joint are immediate signs of complete rupture of central slip

Question 16

Scleroderma is RF +/-

Answer 16

Negative

RFs are antibodies against the Fc part of the IgG and are not commonly seen in patients with scleroderma. Although the sensitivity of rheumatoid factor in rheumatoid arthritis is close to 90%, it is nonspecific because elevated levels of RF are found in Sjögren’s syndrome, mixed connective tissue disease, systemic lupus erythematosus, and myositis.

Question 17

Chondrocalcinosis is seen in

Answer 17

is seen in pseudogout (not rheumatoid arthritis) when calcium pyrophosphate dihydrate (CPPD) crystals are deposited in articular cartilage, synovial lining, ligaments, and tendons. Chondrocalcinosis commonly occurs in the menisci of the knee causing narrowing of the tibial-femoral joint.

Question 18

According to the 2010 ACR classification criteria, the patient must meet three of the following conditions: (a) widespread pain index (WPI) of 7 or more and symptom severity (SS) scale score of 5 or more, or WPI 3 to 6 and SS scale score of 9 or more; (b) symptoms have been present for at least 3 months; (c) the patient does not have a disorder that would otherwise explain the pain. Tenderness in at least 11 out of the 18 specific sites was used to diagnose patients with fibromyalgia according to the 1990 ACR classification criteria. The 2010 guidelines do not require a tender point examination.

Answer 18

fibromyalgia

Question 19

fibroblast growth factor receptor mutation

Answer 19

Achondroplasia is an autosomal dominant genetic disorder caused by a mutation in FGFR3. It is the most common form of dwarfism. This mutation leads to impaired cartilage maturation in the growth plate, resulting in shortened bone, shortened stature, and a prominent forehead.

Question 20

common cause of inflammatory arthritis

Answer 20

parvo virus b-19

Question 21

chronic inflammation at the PIP, which causes a stretch of the volar plate. The PIP joint then moves into hyperextension. At the DIP, there is elongation or rupture of the extensor hood at the base of the phalanx.

MCP subluxation, PIP hyperextension with DIP flexion

Answer 21

swan neck deformity

Question 22

scurvy from deficiency in what vitamen

Answer 22

Vitamin C deficiency leads to impaired collagen synthesis, resulting in pathological manifestations in tissues and organs containing collagen.

Question 23

SLE antibodies

Answer 23

antinuclear antibodies (ANA)
antiphospholipid
anti-double stranded DNA
anti-Smith antibodies.

Question 24

Duputryen’s contracture

Answer 24

flexion contracture of the MCP and PIP joints. It is more common in White men age 50 to 70 years. It is associated with alcoholism, pulmonary tuberculosis, epilepsy, and diabetes mellitus

Question 25

xanthine oxidase inhibitors,

Answer 25

such as allopurinol, for chronic management

Question 26

Anti-Scl-70 or antitopoisomerase antibody is specific for
*
Anticentromere antibody is specific for**.

Anti-Jo-1 antibody is associated with **

Answer 26

diffuse scleroderma
limited scleroderma

polymyositis and or/dermatomyositis
polymyositis can present with dysphagia from weak pharyngeal muscles

Question 27

joint commonly affected by pseudogout

what diseases are associated with pseudo gout?

Answer 27

knee and wrist

Amyloidosis, hypo- (thyroidism, magnesemia, phosphatemia), hyperparathyroidism and hemochromatosis may be associated with pseudogout.

Question 28

contractures after forearm injury

Answer 28

Volkmann’s contracture is contracture of the flexor muscles owing to ischemia in the volar aspect of the forearm. It usually occurs after a forearm injury.

Question 29

types of hypersensitivity reactions

Answer 29

Type I hypersensitivity reaction is immediate with IgE as a mediator. Examples include asthma and anaphylaxis. Type II is also known as cytotoxic, or antibody dependent, with IgM or IgG as mediators. Examples of type II hypersensitivity reaction include thrombocytopenia and erythroblastosis fetalis. Type III is also known as immune complex with IgG as a mediator. Examples include rheumatoid arthritis and systemic lupus erythematosus. SLE is an autoimmune connective tissue disease. The immune system attacks the patient’s own cells and tissues causing inflammation and damage to the tissue. Type IV is also known as delayed type or cell mediated, with T-cells as a mediator. Examples include multiple sclerosis and chronic transplant rejection.

Question 30

if family history of fibromyalgia how much higher risk to devlop

Answer 30

8x higher

Question 31

Pseudogout associated with

Answer 31

hyperparathydoirism, hypothyroidism, amyloidosis, hemachromatosis, hypophosphatemia and hypomagnesemia

Question 32

organisms in septic arthritis

Answer 32

Neonates : Staph A, GBS
6 mos-2 years: H influenza
2 years-adult: Staph, GBS
Sexually active: N gonorrhea
Non sexually adults: Staph A
RA: Staph A
Prosthetic joint: Early (0-3 months): Staph aureus, Delayed 3-24 months: Coag neg staph

Question 33

The most serious complication associated with Sjogren’s Syndrome is:

Answer 33

Lymphoproliferative disease: The risk of lymphoma in patients with primary Sjögren syndrome is 40 times that of the general population.

Question 34

Glucocerebroside accumulates in the reticuloendothelial cells of the spleen, liver and bone marrow in

Answer 34

Gaucher’s disease.

Question 35

Deficiency of the enzyme homogentisic acid oxidase causes

Answer 35

alkaptonuria, which results in bluish discoloration of urine, cartilage, skin and sclera due to homogentisic acid build up.

Question 36

osteochondritis dissecans

Answer 36

Medial femoral condyle most commonly….also found in Capitellum of the humerus (wilsons test, medial rotation of tibia with knee in 90 deg of flexion…then there is pain at 30 deg of flexion…and pain is relieved with external rotation of tibia)

Clanton Classification of Osteochondritis (Clanton and DeLee) –

Type I — Depressed osteochondral fracture
Type II — Fragment attached by osseous bridge
Type III — Detached non-displaced fragment
Type IV — Displaced fragment

Question 37

components of diarthrodial joint

Answer 37

type II collagen
synovial membrane
synovial fluid
joint capsule

Question 38

HLA-DR4 is associated with

Answer 38

rheumatoid arthritis.

Question 39

Intrapelvic displacement of the medial wall of the acetabulum

Answer 39

Protrusio acetabuli

Question 40

RF+ is a more unfavorable thing for RA

Llsters tubercle common site of rupture

Answer 40

.

Question 41

triad of RA, splenomegaly, leukopenia is known as:

Answer 41

Felty’s syndrome

Question 42

Still’s disease

Answer 42

myalgia, onset peak 1-6 yo m=f, rash on trunk + fever

Question 43

charcot arthropathy

Answer 43

Immobilization is indicated in the acute phase of Charcot Arthropathy with casting that should be evaluated every 1-2 weeks to ensure a proper fit. Total non-weight bearing is preferred.