·
MCTD
was first described as a separate entity in 1972 and is a more specific
designation than UCTD. It is not the mixture or overlap of any rheumatic
diseases; rather, MCTD is used specifically
when features of SLE and systemic sclerosis are present with high titers of
antibody to U1RNP.
Those with no definable serology and a nondescript
clinical picture are defined as having UCTD. Other patients have inflammatory myositis, Raynaud phenomenon, and
sclerodactyly together with very high titer antibodies to the ribonucleoprotein
antigen (U1 RNP) and no anti-DNA or anti-Sm antibody. This set of findings is
defined as MCTD.
·
Osteocartilaginous bodies within a joint are
often termed joint mice or loose bodies
and occur commonly in osteoarthritis.
Rice Bodies: Aggregates of fibrin frequently found in
the synovial fluid of patients with RA.
·
A bunion
(hallux valgus) is a deviation of the proximal phalanx of the great toe toward
the fibular side of the foot. It can be caused by biomechanical factors (tight
and pointy-toed shoes that push the proximal phalanx across the other toes),
inflammatory disease (gout or rheumatoid arthritis), or abnormal alignment
(usually congenital) at the first metatarsal-cuneiform joint. If the cuneiform
is abnormal, the first metatarsal may deviate excessively toward the midline (a
primary varus deformity), which leads to a valgus deformity (lateral deviation)
of the great toe when the abnormal foot is placed into standard shoes.
·
Caplan's
syndrome: rheumatoid arthritis (RA) with pneumoconiosis.
Still's disease is a subset of juvenile RA
that has systemic features (fever, lymphadenopathy, pleuropericarditis,
hepatosplenomegaly, and leukocytosis) as major manifestations.
Felty’s Syndrome: SAUL
(Splenomegaly, Arthritis, Ulcers (leg), Leukopenia)
Cogan's syndrome: an unusual vasculopathy
associated with interstitial keratitis, sensorineural hearing loss, tinnitus,
and vertigo. Systemic features such as fever, weight loss, and fatigue are
present in about one-half of patients.
·
SAPHO syndrome
(synovitis, acne, pustulosism hyperostosis) is a representative example of a
group of syndromes that produce seronegative asymmetric arthritis usually with
chest wall pain (sternoclavicular hyperostosis), chronic aseptic osteomyelitis,
and severe acne. Sacroiliac joint involvement can occur and is usually
unilateral. There is no HLA B27 association.
·
POEMS syndrome
is a plasma cell dyscrasia characterized by polyneuropathy, organomegaly,
endocrinopathy, monoclonal protein, and skin changes, which may resemble
scleroderma.
·
"string"
and "mucin clot" tests: The primary component of joint
fluid is hyaluronic acid. It is quite viscous and makes a "string"
when expressed from a syringe as a single drop. Dilute acetic acid causes
hyaluronate and protein to clump and fall to the bottom of a test tube
(producing the famous "mucin clot"). Inflammatory
mediators cause fragmentation of the hyaluronate-protein complex, rendering it
unable to form a good mucin clot.
·
Serum
transaminases (ALT, AST) are found in muscle. Therefore, elevated
LFTs, especially when no concomitant gamma-glutamyl transferase is available,
may reflect muscle inflammation in a patient with rheumatic disease. Check the
creatine phosphokinase level.
·
Gelling
describes the achiness and stiffness that occurs in
patients with RA after a period of inactivity (such as getting up from
the dinner table or rising from a seat after a movie). The stiffness that
occurs on rising from bed in the morning is also a form of gelling.
·
The synovial
lining is a thin, delicate structure (only one or two cells thick)
and contains two types of synoviocytes: type A
(macrophage-like cells probably derived from bone marrow) and type B (fibroblast-like cells that are
probably of mesenchymal origin, secrete synovial
fluid). The subsynovium
constitutes the second layer of normal synovium.
·
Pannus
is the term used to describe the area of proliferating
synovium that meets the articular cartilage. It is believed to be the
source of erosive damage in RA.
·
Antibodies directed against the citrullinated
portion of certain molecules (filaggrin and fibrin among others) have been
found in the sera of patients with RA and are called anti-cyclic citrullinated peptide antibodies. They are present
in about 66% of RA patients and in fewer than 5% of control subjects making
them quite specific for RA. Their
clinical usefulness seems to be in helping to distinguish true RA in patients
who are otherwise seronegative, or if the exact diagnosis of an inflammatory
arthropathy is otherwise unclear.
·
Signs and symptoms of RA subside in approximately 70% of women during pregnancy.
No data suggest that RA has a detrimental effect on the fetus.
·
Pregnancy and
SLE:
1.
Fertility is unaffected by the disease.
2.
Although recent data suggest that pregnant
patients with lupus do not have disease flares more frequently than nonpregnant
patients, disease exacerbations during pregnancy do occur. Because such
flares can be severe, patients with SLE should be considered at high risk.
Active disease during the antecedent 3-6 months may increase the risk of a
flare.
3.
Preeclampsia occurs more frequently in
pregnant patients with lupus. There is also increased risk of miscarriage,
abortion, intrauterine growth delay, and prematurity in patients with SLE
compared with controls.
4.
The Ro antibody crosses the placenta and
is responsible for most of the neonatal lupus syndromes, including skin
manifestations and congenital heart block.
·
Jaccoud's
deformity: Deformities of the hands secondary to chronic
inflammation of the joint capsule, ligaments, and tendons. The changes may mimic those of RA (ulnar deviation of the fingers,
MCP joint subluxation). Erosions are not
present, although after several recurrences, notches may be seen in x-rays on
the ulnar side of the metacarpal heads. Early in the course, patients can
correct these changes voluntarily. Although originally described in rheumatic
fever, this disorder has been extended to include the arthropathy in other
conditions, most commonly SLE.
·
Pyoderma
gangrenosum consists of skin lesions that begin as pustules or
erythematous nodules and break down to form spreading ulcers with necrotic,
undermined edges. It is associated with IBD but also occurs in chronic
active hepatitis, seropositive RA (without evidence of
vasculopathy), leukemia, and polycythemia vera. Differential diagnosis of the lesions includes
necrotizing vasculitis, bacterial infection, and spider bites.
·
The role of genetic factors in rheumatic disease
is an area of vigorous research. OA clearly has
a hereditary component. Perhaps the most recognized feature is the
presence of Heberden's nodes in mothers and sisters. Recent studies have
uncovered a mutation in a type II collagen gene (Arg519 to Cys) that
predisposes to early OA.
·
Vacuum sign:
A radiographic sign of intervertebral osteochondrosis. Radiolucencies represent
gas (nitrogen) that appears at the site of negative pressure produced by
abnormal spaces or clefts. Clefts are produced by degeneration of
intervertebral disc, especially the nucleus pulposus.
·
DILS
(diffuse infiltrative lymphocytosis syndrome) is a condition occurring in
between 3% and 8% of HIV-infected
patients. Although it produces profound salivary gland enlargement and symptoms
of dryness (sicca), it is a distinct entity with
different immunogenetics and pathophysiology from Sjögren's syndrome.
African American DILS sufferers show a high incidence of HLA-DR8, while DR6 and
DR7 are more prevalent in Caucasians. By contrast, in patients with Sjögren's
syndrome, HLA DR2 and DR3 predominate.
·
When patients with established rheumatoid
arthritis have fever and an apparent flare, joint
infection should be excluded by joint aspiration because septic
arthritis occurs more frequently in such patients.
·
IgM rheumatoid factor is most commonly detected;
IgG and, less frequently, IgA rheumatoid factors are also sometimes found. The
presence of IgG rheumatoid factor is associated
with a higher rate of systemic complications (e.g., necrotizing
vasculitis).
·
Unlike acute inflammatory arthritides (e.g.,
gout or septic arthritis), RA tends not to cause
marked erythema, and swelling usually does not extend far beyond the
articulation. Classically, RA is
symmetrical.
·
RA Rx: Advancement
from NSAIDs to second-line agents is recommended
if (1) symptoms have not improved sufficiently after a short trial of NSAIDs,
(2) the patient has aggressive seropositive disease, or (3) there is
radiographic evidence of erosions or joint destruction.
The trend today is
for more aggressive treatment, and the majority of patients require additional
pharmacotherapy. Most patients require rapid advancement from NSAIDs to a
second-line agent, most often methotrexate.
Infliximab is used
in combination with methotrexate; this appears to permit long-term use of
infliximab with less formation of neutralizing antibodies. Infliximab is administered by intravenous infusion; the
recommended dose is 3 to 10 mg/kg every 8 weeks. As with etanercept and
all TNF inhibitors, the drug must be used with care in the presence of
infections. The conventional wisdom is that
glucocorticoids neither alter the course of the disease nor affect the ultimate
degree of damage to joints or other structures.
To prevent the
development of erosive joint changes, the standard of treatment for rheumatoid
arthritis has become much more aggressive aka Inverted
Pyramid Approach.
A favorable course and long remissions are
associated with age less than 40 years, acute onset restricted to a few large
joints, disease duration less than 1 year, and negative test results for
rheumatoid factors. An unfavorable prognosis
is associated with insidious onset, constitutional symptoms, the rapid
appearance of rheumatoid nodules, the appearance of bone erosions early in the
course of disease, and high titers of rheumatoid factors.
·
Differentiation
of the Scleritis & Episcleritis is based on the more violaceous
hue of the sclera in scleritis—caused by inflammation around the sclera vessels—and
pain on pressure over the closed lid onto the globe, which is not seen in
episcleritis. Confirmation can be done by slit-lamp examination.
·
Patients with
hepatitis C infection may have polyarthralgias or polyarthritis that can
resemble rheumatoid arthritis. To make matters even more
problematic, rheumatoid factor is present in many patients with hepatitis C,
especially in the setting of mixed cryoglobulinemia. The rheumatoid factor, as
part of the cryoglobulin, may not be present in the serum if it is collected
and allowed to clot at room temperature. Cryoglobulins will aggregate and
clot if subjected to temperatures generally lower than 100.4° F (38° C). If
rheumatoid arthritis is suspected, the specimen should be allowed to clot in a
38° C water bath and then checked for rheumatoid factor. Patients with hepatitis C should in general avoid
potentially hepatotoxic drugs such as methotrexate.
·
Only those patients
whose clinical presentation and examination are consistent with ankylosing
spondylitis but whose radiographic testing is negative should undergo HLA-B27
testing in c/o AS.
·
In ReA,
Early treatment of genitourinary
infections with appropriate antibiotics (tetracycline or erythromycin) has been
shown to reduce the likelihood of subsequent reactive arthritis; however, even
early antibiotic use in patients with gastroenteritis
does not appear to prevent reactive arthritis.
·
An inflammatory
arthropathy attributable to psoriasis appears in 5% to 7% of
patients with the skin disease, especially in those whose nails are affected. In general, there is little relation between joint disease and the severity of
skin involvement. In fact, psoriatic skin lesions may be found only
after careful scrutiny of scalp, umbilicus, or gluteal regions, and nail
pitting or other changes may be the only clues supporting a diagnosis of
psoriatic arthritis. Asymmetrical oligoarthritis of both small and large
joints is the most common form of psoriatic arthritis. Involvement of the
distal interphalangeal joints and sausage-shaped toes or fingers are highly
suggestive signs. A disparity is often noted between clinical appearance and
subjective symptoms; overtly involved joints may be largely asymptomatic,
unlike the concordance usually found in rheumatoid arthritis. A characteristic
change is the whittling of the distal ends of phalanges, giving the joints a
so-called pencil-in-cup appearance, which
is radiographically distinctive for psoriatic arthritis. Periostitis, bony
erosions, and joint effusions are also common and so are diagnostically useful.
·
The differential diagnosis of unilateral uveitis includes ankylosing spondylitis, Reiter syndrome, and inflammatory
bowel disease. Many cases, however, are idiopathic. Sarcoidosis typically causes bilateral uveitis.
·
Scleroderma renal
crisis is a dreaded complication of diffuse scleroderma. It can
occur rapidly and is more likely to be seen in patients with rapidly
progressive skin disease. Hypertension of new onset, in conjunction with
proteinuria and microscopic hematuria, is highly characteristic.
·
Inflammatory
myopathy: Autoantibodies that are in large part directed against
cytoplasmic ribonucleoproteins have been designated as myositis-specific
autoantibodies (MSA) and are present in 30% of the patients. These
antibodies tend to correlate with some specific clinical presentations,
responses to therapy, and prognoses. Three groups of patients can be
defined by the MSA specificities. The first group is defined by the presence of
antibodies directed against aminoacyl-tRNA
synthetases. These patients are generally characterized by an acute
onset of muscle disease, with a high incidence of associated interstitial lung
disease. They may also have arthritis and a hyperkeratotic rash on the hands,
known as mechanic’s hands. This description fits the patient presented in this
case. The second group includes patients with anti-SRP
antibodies; these patients tend to have an abrupt onset of weakness,
and they may have cardiac disease. The third group is identified by the
presence of antibodies against Mi-2;
these patients have a dermatomyositis with the so-called shawl sign.
·
Patients with polymyositis
frequently have extramuscular manifestations. One of the most common is pulmonary fibrosis. Almost 70% of
patients with pulmonary fibrosis will have the autoantibody
Jo-1 in their serum. Anti–Jo-1 is one of the antisynthetase antibodies currently found only in patients with
myositis. Besides pulmonary fibrosis, the antisynthetase syndrome includes Raynaud phenomenon, polyarthritis, and, in some cases,
so-called mechanic’ s hands.
·
Biopsy of the muscle
showing type 2 fiber atrophy is typical of steroid myopathy.
·
Cancers of the
ovary, lung, lymphatic system, and hematopoietic system are overrepresented in
patients with dermatomyositis. In women older than 40 years with
dermatomyositis, the risk of ovarian cancer is 20 times that of the general
population. Ovarian tumors are notoriously hard
to find early. The most recent recommendations for detecting them in
patients with dermatomyositis include a careful gynecologic examination,
measurement of CA-125, and transvaginal ultrasound at 3- to 6-month intervals.
·
CSS differs most
strikingly from WG in that the former occurs in patients with a history of
atopy, asthma, or allergic rhinitis, which is often ongoing. In the
prevasculitic atopy phase, as well as during the systemic phase of the illness,
eosinophilia is characteristic and often of striking degree (≥ 1,000
eosinophils/mm3). When eosinophilia is present in WG, it is usually more modest
(~500 eosinophils/mm3). Chronic sinusitis can be seen in both CSS and WG,
although it is more characteristic in the latter than the former. Polymyalgia
rheumatica is not associated with either CSS or WG; there is, however, a clear
association between polymyalgia rheumatica and temporal arteritis. A preceding
streptococcal or viral infection has been seen occasionally with both WG and
CSS.
·
In GOUT, At least half of initial attacks
occur in the first metatarsophalangeal joints (a condition known as podagra), but other joints of the foot may be
involved simultaneously or in subsequent attacks.
Agents
available for terminating the acute attack include colchicine, NSAIDs,
adrenocorticotropic hormone (ACTH), and corticosteroids. Corticosteroids and ACTH have been used more often in
recent years in patients with multiple comorbid conditions, because of the
relatively low toxicity profile of these agents.
·
Most of the association of obesity with osteoarthritis of the knee appears
to be related to environmental, rather than
genetic, factors. An association between increased bone density and
osteoarthritis has been noted in several studies. Women
with osteoporosis and hip fractures have a decreased risk of osteoarthritis,
and those affected by osteoarthritis have significantly increased bone density.
This negative association suggests that soft subchondral bone absorbs impact
and protects articular cartilage better than dense bone. Many patients with
osteoarthritis have a family history of the disorder, and multiple genetic
factors may be responsible in various forms of osteoarthritis. In women, osteoarthritis with finger joint involvement is
probably the best-recognized form of arthritis with familial associations,
but hereditary factors are also important in osteoarthritis of the hip.
Erythema and warmth are unusual and should suggest the
presence of coexistent crystal-induced inflammation or other conditions.
Erosive osteoarthritis is characterized by
polyarticular involvement of the small joints of the hand and tends to occur
more often in middle-aged and elderly women.
·
For the treatment
of acute back pain, NSAIDs and mild analgesics may be useful for
symptom control. Muscle relaxants and opiates should be used sparingly. Spinal
manipulation or specific exercise programs may also be effective in acute back
pain. Over 90% of patients will improve within 1 month. Strict bed rest should
be kept to a minimum, and continuation of normal activities should be enforced.
The management of chronic back pain is complex.
Patients should undergo physical therapy, an exercise program, and an education
program that emphasizes proper ergonomics for lifting and other activities.
Light normal activity and a regular walking program should be encouraged.
Encouraging the patient to apply for disability before trying different
therapeutic interventions is not appropriate. Judicious use of NSAIDs and mild
analgesics may improve patient function and outcome.
·
Plantar fasciitis
is one of the most common causes of hindfoot pain. Patients report pain over
the plantar aspect of the heel and midfoot that worsens with walking. Localized
tenderness along the plantar fascia or at the insertion of the calcaneus is
helpful in diagnosis. Plantar fasciitis is associated with obesity, pes planus,
and activities that stress the plantar fascia. It may also be seen in systemic
arthropathies such as ankylosing spondylitis and Reiter syndrome. Although radiographic spurs in the affected area are
common, they may also be seen in asymptomatic persons and are therefore not
diagnostic.
·
Fibromyalgia = tendon insertions and muscles hurt
all the time
o
multiple tender trigger points (11/18 for Dx)
o
Amytriptyline is the treatment → because
of ↑↑↑ incidence of depression
There are four principal
categories of pain: nociceptive, neuropathic, psychogenic, and
chronic pain of complex etiology. Chronic pain of
complex etiology is the type of pain characteristic of fibromyalgia.
Fibromyalgia patients often have fixed beliefs that minor traumatic events,
pathogens, chemicals, or other physical agents caused their illness.
Evidence of synovitis (e.g., joint effusion, warmth over the
joint, pain on joint motion), objective muscle weakness, or other
definite physical or neurologic signs suggest the presence of either comorbid
disease or an alternative diagnosis. Eighteen
specific tender points have been identified in fibromyalgia. A patient with
fibromyalgia will have pain, not just tenderness, on palpation at many of these
tender points. Palpation is performed with the thumb,
using approximately 4 kg of pressure—about the pressure necessary to
blanch the examiner’s thumbnail. Attempting to confirm pain at all 18 tender
points is not necessary for diagnosis and is inconsiderate toward patients,
many of whom find tender-point palpation quite distressing. Useful tests in
fibromyalgia include the following: ANA, CBC, ESR,
CRP, TSH, CK, AST, and ALT. Tests for Lyme disease, Epstein-Barr
virus infection, and endocrinologic status are usually unnecessary.
·
Polymyalgia rheumatica = when the shoulder girdle
is the weakest
o
Pain in
the shoulder girdle (actions of waving, combing hair)
o
Tender trigger points
o
very high incidence of Temporal Arteritis (Giant cell)
·
temporal headache
·
very high ESR
·
Rule of
60’s
·
age>60
years old
·
ESR >60
sed rate
·
Need more than 60mg of Prednisone to treat it.
·
Diagnose with temporal artery biopsy (MUST DO)
·
Complications
– blindness due to involvement of ophthalmic artery.
·
Calcium oxalate deposition disease is usually
seen in patients with end-stage renal disease; calcium phosphate deposition
disease causes calcific tendinitis or Milwaukee
shoulder.
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