Rheumatology Flashcards
11 diagnostic criteria for SLE (need 4 out of 11)
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
Juvenile dermatomyositis
Type IV polymyositis/dermatomyositis is associated with childhood.
80-90% respond to steroids
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
CREST syndrome
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
Collagen type affected in OI
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.
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)
RA
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
OA
Erosion and periosteal changes at the insertion of the plantar fascia and Achilles tendon (“lover’s heel”)
reactive arthritis
Bony erosions, syndesmophytes, and “pencil-in-cup” deformity of the distal interphalanx (DIP)
psoriatic arthritis
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.
Wegener’s granulomatosis
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?
Polyarteritis Nodosa (also seen in RA, SLE, Sjograns)
small vessel, oral and genital ulcers, 20% have VTE
Behcets syndrome
Large arteries, more common in females, tendenress in scalp and muscles of mastication, headache, associated with PMR, elev ESR, temporal bx
temporal arteritis (high dose steroids asap to avoid vision loss, asa 325 mg improves prognosis).
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.
Marfan syndrome
earliest radiographic sign of rheumatoid arthritis.
Diffuse periarticular osteopenia
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.
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
Scleroderma is RF +/-
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.
Chondrocalcinosis is seen in
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.
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.
fibromyalgia
fibroblast growth factor receptor mutation
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.
common cause of inflammatory arthritis
parvo virus b-19
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
swan neck deformity
scurvy from deficiency in what vitamen
Vitamin C deficiency leads to impaired collagen synthesis, resulting in pathological manifestations in tissues and organs containing collagen.
SLE antibodies
antinuclear antibodies (ANA)
antiphospholipid
anti-double stranded DNA
anti-Smith antibodies.
Duputryen’s contracture
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
