Step Up to Medicine: Chapter 06 Connective tissue and Joint diseases

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Treatment of RA?

1. Principles of treatment
-goals of treatment are to prevent or halt joint destruction and to come as close to clinical remission as possible while avoiding the toxicity of nti-RA medication
-treatment must be individualized to the patient.
-a treatment regimen that works for one patient may not work for another.

2. Exercise helps to maintain range of motion and muscle strength

3. SYmptomatic treatment
-NSAIDs are the drugs of choice for control of pain. They play an important role in controlling inflammatoin and should be part of most treatment regimens
-corticosteroids low dose) - use these if nSAIDS do not provide adequate relief.
-short term treatment may be appropriate
-avoid long term high dose steroids - these can be detrimental
-longterm, low dose corticosteroids may actually alter the course of the disease (have been shown to diminish radiographic progression) - more sudies are needed before this can be considered a disease-modifying drug.

Temporal arteritis

General characteristics:

aka: "Giant Cell arteritis"
-vasculitis of unknown cause; typical patients is >50 YO and twice as common in women as in men
-temporal arteries are most frequently affected but it may involve other arteries such as the aorta or carotids
-associated with increased risk of aortic aneurysm and aortic dissection

Diagnosis of reactive arthritis

Synovial fluid for analysis - this r/o infection and crystals

How is the diagnosis of wegeners made?

chest radiograph is abnormal (nodules or infiltrates)
-laboratory findings: high ESR, anemia (normochromic/normocytic)
-hematuria: positive CANCA in 90% patients both sensitive and specific.
-Thrombocytopenia may be present

CONFIRMATION of dx: LUNG BIOPSY

Clinical features of takayasu's arteritis

-Consitutional symptoms: fever, night sweats, malaise, arthralgias, fatigue
-Pain and tenderness over involved vessels
-Absent pulses in carotid, radial or ulnar arteries, aortic regurgitation may be present
-Signs and symptoms of ischemia eventaully develop in areas suppled by involved vessels.

**TREATMENT: Steroids; treat HTN with antiHTN meds; surgery or angioplasty if recannulation of vessel is required. Can use bypass

How is RA diagnosed?

High titers RF are asociated with more severe disease and are generally nonspeific. RF is eventaully presnt in 80% of patients with RA (may be ansent early in the diese) but it is also present in up to 3% of the healthy population.

1. ELevated ESR, CRP
3. Normocytic normochormic anemia (anemia of chronic disease)

Radiographs
-loss of juxtaarticular bonemass (periarticular osteoporosis) near the giner joints
-narrowing of the joint space (due to thinning of the articular cartilages) is uauly seen late in teh diases
-bone EROSIONS at the margins of the joint

Features that are unique to dermatomyositis?

1. Helicotropic rase (butterly) around eyes, bridge of nose, cheeks
2. Gottron's papules - papular, erythematous, scaly lesions over the knuckles (MCP, PIP, DIP)
3. V sign - rash on the face, neck, anterior chest
4. Shawl sign - rash on shoulders and uper back, elbows, and knees
5. Periungual erytema with telangiectases
6. Subcutaneous calcifications in chidlren can be extremely painful

General characteristics of ANK SPONDYLITIS

-strong association with HLA B27
-3x > males than in females
-look for pos famil hx of ank spondylitis, inflammatory bowel dz, psoriasis
-bilateral sacrolitis is a prereq for mkaing the diagnosis.
-onset in teenage years or early 20s
-characterized by fusion of the lumbar spine that ascendings to the cervical area.

CLincal features of Polymyalgia rheumatica

Hip and shoulder muscle pain (proximal)
-often starts abruptly
-stiffness in regions after period of inactivity is the most prominent symptoms
-pain occurs on moveemnt; muscle strength is normal
-profound morning stiffness is common

Consitutional sypmtoms are present malaise, fever, depression, weight loss, fatigue

Joint swelling
-up to 20% of patients have synovitis in knees, wrist, hand joints (can be confused often with RA)
-synovitis and tenosynovitis around the shoulder may lead to rotator cuff tendonitis or adhesive capsulitis

Signs and symptoms of temporal arteritis (if present)

Diagnosis of Temporal Arteritis

ESR elevation (but can be normal)
-biopsy of temporal artery has sensitivity 90%

What is the recommended treatment for acute gout?

1. bed rest
2. NSAIDS
3. Colchicine - an alt for patients who cannot take NSAIDs or did not respond to NSAIDS; do not useif renal insufficiency or cytopenia
4. Corticosteroids (7-10d course)

AVOID ASA AND ACETOMINOPHEN

What is the treatment for SLE?

avoid sun exposure because ti can exacerbate cutaneous rashes
NSAIDS - for less severe symptoms
local or systemic corticosteroids if for acute exacerbations
cytotoxic agents like cyclophosphamide for active glomerulonephritis
monitor the following and treat appropriately (renal dz and HTN)

Sjogrens syndrome has an increased risk of __________ ____. What is the most common cause of death?

non-hodgkins lymphoma
-malignancy

Gout v. Avascular necrosis

Gout: uric acid deposition in joints. Pain. Great toes, ankles, wrist, elbow effected. Lumby bumby appearance on plain film; punched out lesions with overhangiing rim of corticol bone "rat bite"

Avacasular necrosis: 2/2 bone destruction with lack of blood supply. It is a complication of steroid use, trauma, lupus, SCD, number of oter processes. Affects the proximal and distal femur most often. Pain is the msot common symptom

Vasculitis can be classified into size of vessels:

Large vessel: takayasu;s, TA
Medium vessel: PAN, Kawasakis, Wegeners, Churg Strauss, MPA
Small: HSP, HS vasculitis, Behcets syndrome

*blood vessels are inflammed and vascular necrosis results. Findings depend upon the size of the vessel involved and THEREFORE the location of involvement will indicate the organ of ischemia.

Amyloidosis is probable to cause a nephrotic like picture in patients with which clinical predispositions?

1. Rheumatoid
2. Enlarged kidneys
3. Hepatomegaly

Diagnosed?

-renal amyloid deposits that show apple-green birefringence under polarized light after staining with Congo red

How does Baker's cyst present? What does it consist of?

Result of excessive fluid production by an inflamed synovium as occurs in cases of rheumatoid arthritis, osteoarthritis and cartilage tears. The excess fluid accumulates in the popliteal bursa which expands, creating a tender mass in the popliteal fossa. Bake cysts bursa and release contents into calf resulting in a "DVT" appearance.

What are the variants of RA?

1. Feltys syndrome: anemia, neutropenia, splenomegaly, and RA
2. Juvenile RA: begins before 18 years of age. Extra-articular manifestatins may predominat (Stills disese) or arthritis may predominate

10% of patients with polymyalgia rheumatica also have ___ ___ whereas up to 40-50% of patients with ____ ____ have coexisiting polymyalgia rheumatica.

Temporal arterities
Temporal arterities

Thus half of patients with TA have PR, but <10% pts with PR have TA

AA female, photodistriuted skin rash, artralgias. Low range proteinuria and abnormal urinary sediment. Biopsy findings are consistent with focal proliferative glomerulonephritis. COMPLETE CBC shows:

dec erythrocyte
dec platelet
dec leukocyte

Hematological findings?

pancytopenia 2/2 SLE. Formation of autoAB against blood cells form a type II hypersensitivity reaction

Sjogrens syndrome is an autoimmune disease most commonly seen in women. _________ infiltrate and destroy the lacrimal and salivary glands. This is a multisystem disease (involves the ----,----,---,---,---

WHat is primary v. secondary sjogrens?

LYMPHOCYTES
-skin, liver, lungs, thyroid, vessels

Primary sjogrens syndrome: dry eyes, dry mouth, lymphocytic infiltration of the minor salivary glands (on histology); patients do not have another rheumatologic disease.

Secondary sjogrens syndrome: dry eyes and dry mouth along with a connective tissue disease (RA, systemic sclerosis, SLE, polymyositis)

Prognosis for SLE?

Most patients do not acheive normal life expectancy.
-with proper treatment, severe organ damage can be prevented and symptoms controlled in many cases
-the most common causes of death are oppourtunistic infections and renal failure

SLE:
Contstiutional
Cuteneous
MSK
Cardiac
Pulm
Hematologic
Renal
Immunologic

-fatigue, malaise, fever, weight loss
-butterfly rash, photosensitivity, discoid lesions *erythematous eevated patch); raynauds,
-joint pain, athritis (inflammatory AND symmetric but NON erosive like RA)
-pericarditis, endocarditis (libb man sacks NBSE), myocarditis
-pleuritis, pleural effusion, penomitis leading to fibrosis, pulm HTN (rare)
-hemolytic anemia with anemia or retics of chronic dz, leymphopenia, trombocytopenia
-proteinuria > 0.5 g/day with nephrotic syndrome; cellular casts; GN (may have hematuria); azotemia, pyuria, uremia, HTN
-immunologic; impaired immune response due to many factors including autoAB to lymphocytes abrnomal T cll function and immunosuppressive meds

What is the pathogenesis of GOUT?

1. Increased UA production --> hypoxanthine-PP synthetase deficiency (lesch nyhan); phosphoribosyl pyrophosphate synthetase overactivity; increased turnover assoicated with a number of conditions, including cancer chemotherapy, chronic hemolysis, and hematologic malignancies.

2. Decreased excretion of UA (accts for 90%)
-renal dz; NSAIDSs/diuretics; acidosis

Treatment of temp arteritis once diagnosed

1. HiD prenisone early to prevent blindness
2. Start rx immedicately, dont wait for biopsy results
3. If dz confirmed, continue rx for 4 weeks the taper gradually. Maintain therapy for 2-3 years. Relapse is likely if stop prednisone early.
4. F/u with ESR levels to monitor effectiveness of treatment
5. Visual loss in one eye may be temporary or permanent.
6. Even is untreated the disease is self limiting in most patients but the vision can be a permanent issue

What is GOUT?

Inflammatory monoarticular arthriti caused by crystallization of monodosium urate in the joints. Hyperuricemia is a hallmark of the dz but it can present without indication of gout.

90% of men over 30YO most likely to be diagnosed. Women are not affected until post menopause

Polymyalgia Rheumatica

Age:
Population:
Major association:

autoimmune process (likely) with a genetic link (HLADR4) most common in elderly patients - rarely before age 50 but usually dominant in 70YOF.
-cause is unknown
-self limited disease (1-2) years!***

Diagnosis of FMS

1. Diagnostic criteria: widespread pain including AXIAL pain for >3 months, pain in at least 11/18 tender points
2. Before confirming FMS dx, must r/o myofascial syndromes, rheumtoid, PR, SLE, AS, chronic fatigue syndrome, lyme disease, hyPOthyroidism, polymyositis

Diagnosis of PAN

-bipose of involved tissue or mesenteric angiography
-high ESR
-pANCA pos
-test for fecal occult blood

Wegeners granulomatosis (granulomas with polyangiitis) clinical features?

upper respiratory symptoms (sinusitis) purulent or bloody nasal discharge
-oral ulcers may be painful
-pulmonary symptoms (cough, hemoptysis, dyspnea)
-renal involvement (GN - may have rapidly progressive renal failure)
-eye dz *conjunctivitis/scleritis)
-MSK arthralgias, myalgias
-tracheal stenosis

When should uricouric agents be admin to patients with GOUT?

1. Uricosuric drugs (probenecid, sulfinpyrazone) if the 24hr ua level is < 800 mg/day then this indicates an undersecretion or URATE. THese durgs increase renal excretion of UA and should be used only in pt with normal renal function.
2. Allopurinol - is a xanthine oxidase inhibitor that decreases UA synthesis - if the 24hr uric acid is >800 m/day this indicates overproduction. Never give this for acute gout; it maks it worse. Use once-daily dosing. IT is well tolerated

Associated findings with dermatomyositis?

1. Vasculities of the GI tract kidneys lungs and eyes (more common in children)
-increased risk of malignancy in oler adults (lung, breast, ovary, GI tract, and myeloproliferative disorder)
-once dermatomyositis is diagnosed make an effort to uncover an OCCULT MALIGNANCY. Dermatomyositis is associated with malignancy and will remit once tumor removed.

What is the treatment for Sjogrens syndrome?

1. Pilocarpine (enhances secretions)
2. Artificial tears for dry eyes
3. Good oral hygiene
4. NSAIDS, steroids for arthralgias, arthritis
5. Patients with secondary Sjogrens syndrome- therapy for connective tissue disease

HLA associations with rheumatic diseases: RA

HLA-DR4

Prognosis of CHURG STRAUSS

poor with a 5 year survival of 25% (death is usually due to cardiac or pulmonary complications) with treatment (steroids), the 5 year prognosis improves to 50%

Normal synovial fluid analysis:

Clear fluid
<200 WBC
<25% PMNs

What cervial spine involvement is associated with RA?

Cervical spine involvement is common at C1-C2 subluxation and instabiliy, but it is less common in the lower cervical spine.
-instability of the cervial spine is a potentially life-threatening complication of RA. Most patients do not have neurologic involvement, but if they do, it can be progressive and fatal if not treated surgically.
-this is seen in 30-40% of patients
-all patients with RA should have cervical spine radiographs before udnergoing any surgery (due to risk of neurologic injury during intubation)

what does the term "undifferentiated" spondyloarthropathy used to indicate?

when a pt has features of reactive arthritis but there is no evidence of previous infection (in the FI or GU tract) and the classic findings of reiter's syndrome are absent

What is the most common finding (usually present at diagnosis) of lupus? What are the types?

Lupus nephritis
1. Type 1 - 5 % - minimal lesions, renal failure is rare
2. Type 2 - 20 % - mesangials lupus GN - rarely renal failure
3. Type 3 - 25 % - focal proliferative GN -renal failure is uncommon
4. Type 4 - 40 % - diffuse proliferation GN - renal failure is common
5. Type 5 - 10% - membranous GN - renal filures is uncommon

HLA associations with rheumatic diseases: HLA DR2 and DR3

SLE

Precipitation of an acute gouty attack

decreased in temp
dehydration
stress
excessive alcohol intake
starvation

Takayasu's arteritis
General characteristics

1. Most common to young asian females
2. Vasculitis of aortic arc and its major branches - potentially leading to stnsosis or nrrowing of the vessels
3. Diagnosed via ateriogram

Buergers Disease (aka Thromboangiitis Obliterans)

Occurs mostly in young men who smoke cigarettes
-acute inflammation of small and medium sized arteries and veins, affecting arms, and the legs
-can lead to gangrene
-clinical features include ischemic claudication: cold, cyanotic, painful distal extremities, parethesias of distal extremities and ulceration of the digits.
-smoking cessation is mandatory to reduce progression

What organisms are most commonly associated with REACTIVE arthritis?

Salmonella, Shigella, Campylobactor, Chlamydia, Yersinia

What is the diagnostic criteria for POLYMYOSITIS?

If two of first four --> possible polymyositis
If three of first four-->probably polymyositis
If all four --> definite polymyostis
-symmetric and proximal muscle weakness
-elevation in serum CPHOS
-EMG findings of myopathy
-BIOPSY evidence of myositis
-caracteristic rash of dermatomyositis

What is BEHCETs syndrome?

An autoimmune, multisystem vasculitis disease, cause is unknown
-clinical features: recurrent oral and genital ulcerations that are usually painful, arthritis in the nees and ankles, eye involvement (evwitis, optic neuritis, iritis, conjunctivitis), CNS involvement (meningoencephalitis, intracranial HTN, fever and weight loss
-Diagnosis: made by biopsy of involved tissue - no labs are helpful
-Treatment - steroids

In RA, changes in the joint are usually more extensive than in OA because the entire synovium is involved in RA. Notes that ______ is a characteritic of OA and NOT that of RA.

Osteophytes

Prophylaxis for gout?

1. Wait until pt has two acute gouty attacks before starting therapy. Two attacks per year sometimes used as a rough guidline. This is because the second attack may take years to occur if at all, so the risk to benefit ratio for prophylactic medications (allopurinol) is not favorable after one gouty attack.

2. When given prophylaxis, add either colchcine or NSAID for 3-6 mo. The colchicine or nSAID can be d/c and the pt can remain on the uricouric agent or allopurinaol indefininely.

3. The choise of whether to use uricosuric drugs or allopurinaol depends on how much UA is exceted in the urine in a 24 hour period.

What is the diagnosis of Sjogrens?

ANAs are pressent in 95% of the cases --> 50-75% of patients with seondary disease
-Ro (SS-A) present in 55% of patients, and La (SS-B) Abs present in 40% of patients.
-nonspecific findings: increased erythrocyte sedimentation rate (ESR), normocytic, normochromic anemia, leukopenia (normal ESR 10-20)

Patietns with antibodies to Ro (SSA) are at increaed risk of having a child with neonatal SLE (with congenital heart block).

Which cardiac abnormalities are involved with RA?

Pericarditis, pericardial effusions, conduction abnormalities, and valvular incompetence

Fibromyalgia
General characteristis:
1. Population
2. Age:
3. Key to dx:

1. Adult women 80-90% of cases
2. Chronic nonprogressive course with waxing and waning in severity; many patients improve with time
3. Key to dx: multiple TRIGGER points (points that are tener to palpation)
-symmetrical
-eighteen characteristic locatoins have been identified, including OCCIPUT, NECK, SHOULDER, RIBS, ELBOWS, BUTTOCKS, KNEES
4. Etiology is unknown however somatization is still NOT a proven cause

What the clinical features of FMS?

-stiffnes, body aches (MSK); fatigue
-pain is constant and aching, and is aggreavates by weather changes, stress, sleep deprivation, and cold temperatures. Worst in the morning
-sleep patterns disrupted and sleep is unrefreshing
-anxiety and depression common

What are the clinical features of Sjogrens?

dry eyes --> burning, redness, blurred vision
dry mouth
arthralgias, arthritis, fatigue
many extraglandular manifestations (more common in primary disease) such as chronic arthritis, interstitial nephritis, and vasculitis

Pathophys of PAN

PMN invasion of all layers and fribinoid necrosis plus resulting intimal proliferation leading to reduced luminal area which results in ischemia, infarction and aneuryms

What is the treatment for poly/dermatomyositis?

Corticosteroids intially
-taper slowly p to 2 years

-immunosuppressive agensts for patients who do not respond to steroids (MTX, cyclophosphamide, chloambucil),
-physical therapy

Common laboratory markers in which disease: ANAs

SLe, Scleoderma, SS, Polymyo
-highly sensitive for SLE but not for the others

Course of SLE

chornic disease characterized by exacerbations and remissions
-malar rash
-joint pain
-fatigue
-most common initial findings with more advanced disease - renal, pulmonary, CV, and nervous system are affected.

What surgical procedures can be done on patients diagnosed with RA?

1. Synovectomy (arthoscopic) decreases joint pain and swelling but des not prevent xray progression and does not imporve joint ROM
2. Joint replacement surgery for severe pai unresponsive to conservative management

Septic arthritis synovial fluid analysis

Turbid and purulent
>50,000 WBC
>70% PMNs
synovial fluid culture positive for most cases of bacterial arthritis except gonococcal (only 25% are positive)

Diffuse versus limited scleroderma:

1. Diffuse: widespread skin involvement; rapid onset of symptoms (skin and other comlications occurs rapidly after osnet of Raynauds phenomenon); significant visceral involvement (fibrosis of internal organs - lungs - heart - gi - kidneys); associated with ANAs but absense of anticentromere antibody; poorer pronosis - 10 year survivl is 40%-65%; peripheral edema, polyarthritis, faitgue andweakness, carpel tunnel syndrome, renal failure can occur, but now rare; interstitial lung disease more common

2. Limited. Skin involvement limited to distal extremities and face and neck - sparing of the trunk; delayed onset, skin involvement occurs slowly after the osnet of raeynauds phenomemon. Threfore the patient ahs a long history of rayneuds phenomenon before other symptoms begin.; visceral involvement occurs LATE (pulmonary HTN rater than fibrosis, ischemi vascular disease; minimal contiustional symptoms); Anticentromere antibody is found in most patients; better prognosis than diffuse form; normal life span is expected in most cases, unless severe pulmonary HTN develops. Crest syndrome is a variant (CREST - calcinosis of the digits, raynauds phenomenon, esophageal motilitty dysfuntion, sclerodactyly of the fingers, telangientases (over the digits and under the nails).

What are the consitutional symptoms can be prominent in RA?

1. Morning stiffness (present in all patients) - improves as the day progresses
2. Low grade fever, weight loss
3. Fatigue can be prominent because this is a systemic disease.

What is the clinical course of SLE?

1. chronic disease charactertized by exacerbations and remissions
2. malar rash, joint pain, and fatigue are the most common intiial findings with more advances disease, renal, pulmonary, CV and nervous systems are affected.

When should one suspect takayasu's arteritis?

In a young women with:
1. Decreased peripheral pulses
2. Discrepancy of BP (arms) versus (legs)
3. Arterial bruits

Polyarteritis Nodosa (PAN)
General:

1. VAsculitis of medium sized vessels
2. Involves the nervous system and GI - NOTHING TO DO WITH PULMONARY
3. Can be associated with HBV, HIV, and drug reactions

What are the four stages of gout?

1. Asymptomatic hypruricemia
2. Acute gouty arthritis
3. Intercritical gout
4. Chronic tophaceous gout

What are the four classes of rheumatoid arthritis?

1. Patient has an acute attack, which subsides, and never has an attack again (10%)
2. Undulating course, periods of exacerbations and remission (20%)
3. Patient has periods of exacerbations and "remissions" but "remissions" are really improvements, as patients are symptomatic at all times to some extent (65%)
4. Severe, progressive course (5%)

Most common joints affected in PSEUDOGOUT?

THe knees and wrists
-classically MONOARTICULAR but can be poly

What are te clinical features of ank spondylitis?

Low back pain and stiffness (2/2 sacroilitis) limited motion in lumbar spine
-neck pain
-limited motion of cervical spin - occurs later in course of disease
-ethesitis (INFLAMMATION OF THE TENDINOUS INSERTIONS INTO BONE (achilles tendon and surprspinatus tendon most common)
-with spinal involvement, the spine becoes brittle and prone to fracture with minimal truama.
-chest pain and diminised chest expansions
-shoulder and hip pain (most common)
-constitutional syptoms (fatigue, fever, malaise)
-Extra articular involvment (ACUTE ANTERIOR UVEITIS OR IRIDOCYCLITIS is the most commone other features that can occur can be cardiac, renal , pulm
-loss of normal posture as disease progresses

Treatment for acromegaly, carcinomid syndrome and VIPoma

octreotide

Features of raynauds?

-vasospams and thinked vessels
-leads to digital ischemia
-cold temperatures and stress bring about color changes of fingers, blancing first then cyanotic, then red. (WHITE BLUE RED)

cutaneous fibrosis
-tightening of the skin face and extrmeities (sclerodactyly)
-lead to cutaneous contractures, disability and disfigurement

GI involvement
-occurs in most patients (both diffuse and limited)
-findings include dysphagia/reflux from esophageal immobility with delayed GI empting, abdominal distention, and pseudoobstruction. Prolonged acid reflux may eventaully lead to esopageal strictures

Wegeners granulomatosis:
General

Vasculitis predominantly involving the kidneys and upper and lower respiratory tracts *sometimes other organs

What is antiphospholipid antibody syndrome?

A hypercoaguable state that can be idiopathic or associated with SLE (or other CVD)
-findings include venous thrombosis - PE is a risk
-recurrent arterial thrombosis
-recurrent fetal loss (abortions)
-thrombocytopenia
-livedo reticularis

Laboratory findings: presense of lupus anticoagulant, anticardiolipin antibody, or bot. Prolonged PTT or PT is not corrected by additing normal plasma

-treatment is long term anticoagulation with an INR 2.5-3.5 (lupus anticoag is the anti-thrombin AB)

What are the clinical findings associated with NEONATAL LUPUS?

skin lesions
cardac abnormalities (AV BLOCK) - transposition of the great vessels
valvular septal defects

What are rheumatoid nodules?

subcutaneous nodules ocer the extensor surfaces (elbows sacrum occiput) that can also occur in visceral structures (eg lungs, pleura, pericardium)

What conditions is ANA elevated?

SLE
RA
Scleroderma
SS
MCTD
Polymyositis and dermatomyositis
Drug induced lupus

Common laboratory markers in which disease: ESR

conditions: infection (acute/chornic); malignancy, rhumatiologic diseases, miscellaneous (tissue necrosis, pregnancy)

comments: low sensitivity, and specificity. Major uses: diagnose/rule out inflammatory process and monitor course of inflammatory conditions

Diagnosis of Polymyalgia Rheumatica

clinical diagnosis
-ESR usually elevated and aids in diagnosis
-almost always >50, frequently >100
-Correlates with disease activity

Pathophys of inflammation in GOUT

PMNs play the role in the acute inflammation
-develops when the UA crystals collect in the synovial fluid as the extrecellular fluid becomes saturdated with uric acid.
-IgGs coat monosodium urate crystals, which are phagocytied by PMNs, leading to the release of inflammatory mediateors and proteolytic enzymes from the PMNs which then result in inflammation

Clinical finidings associated with NEONATAL LUPUS

skin lesions, AV BLOCK, transposition of the great vessels
-valvular and septal defects

Clinical findings of PAN:

1. Early symptoms are fever, weakness, weight loss myalgias and arthralgias, and abdominal pain (bowel ANGINA)
2. Other findings are HTN, mononeuritis muliplex and livedo reticularis

Common laboratory markers in which disease: RF

RA (70%) of patients
Healthy population 3%

-neither sensitive OR specific for RA

What disease modifying drugs are associated with RA?

General principles:
1. Can reduce morbidity and mortality (by nearly 30%) - by lmiting complications, slowing progression of disease, and preserving joint function
-should be initiated early (at the time of diagnosis)
-they have a slow onset of actoin (6 weeks or loner for effect to be seen),
-they have a slow onset of action (6 weeks or longer for effect to be seen), so begin treating RA while weaiting for the disease-modifying therapy to take effect. Once the effect is evident, gradually taper and discontinue NSAIDS an corticosteroids and cotinue the disease-modifying program

2. First-line agents
-MTX: most popular treatment right now - first line therapy
--initial improvement is seen in 4-6 weeks
- 80% of treated patients will experience moderate to excellent symptomatic benefit from treatment although remission is rare
--MTX highly associate with interstitial pulmonary fibrosis

Hydroxycloroquine
-this is an alternative first line agent, but usually not as effect as MTX
-requres eye examinations every 6 mo 2/2 retinopathy (although quite rare)

Sulfasalazine - alternative first line agent, but less effective than MTX

Second line agents include: gold compounds, penicillamine, aza, and cyclosporine

Treatment and management of FMS

1. Advise pt to stay active and productive
2. Meds generally ineffevtice. SSRIs and TCAs are beneficial but avoid narcotics
3. Cognitive behavioral therapy, exercise, consider psychiatric eval

Classifications of idiopathic inflammatory myopathies:

1. Polymyositis
2. Dermatomyositis
3. Childhood onset dermatomyositis - subcutatneous calcifications
4. Myositis associated with CVD
5. Myositis associated with malignancy
6. Inclusion body myositis - "oddball" for the following reasons: affects male more than female patients, absense of autoantibodies, distal muscle involvement nd relatively low creatine kinae; poor prognosis

Treatment of GOUT?

Avoid seondary causes of hyperuricemia
-medications that increase uric acid levels (thiazide and loop diuretics)
-obesity
-reduce alcool intake
-reduce dietary purine intake

Complications of gout
-nephrolithiasis - risk is small less than 1% per year
-degenerative arthritis occurs in <15% of patients.
-incidence is decreasing due to effective tretment of hyperuricemia and conseqeunt prevention of tophaceous gout

CLINICAL features of reactive arthritis?

Look for evidence of prior GI or GU infection 1-4 weeks prior
-asymetric arthritis - new joints may be involved sequetially over days. Joints are painful with effusions and lack of mobility.
-fatigue, malaise, weight loss and fever are common
-joint pain may persist or recur over a long-term period

What is usually the first symptom of SLE?

joint pain (MSK)

Cure for systemic sclerosis?

no effective cure
-treatsymptomatically with NSAIDS for MSK pain, H2 blockers or PPI for esophageal reflux
-raynauds phenomenon- avoid cold and smoking - keep hands warml if severes use CCB
-treat pulmonary and renal comlications if present

How is Churg Strauss syndrome dx?

BIOPSY of the LUNG or SKIN
-**PROMINENCE OF EOSINOPHILS***
-associated with P-ANCA

How does the "daily" activity of someone with ANK SPONDYLYTIS PROGRESS?

worse in the morning but gets drastically better withe exercise, hot showers, and worsens with inactivity

What is inclusion body myositis?

Most common in males (elderly)
-insidious onset of slowly progressive proximal and distal weakness that often leads to delay in its diagnosis
-early weakness and atrophy of the quads, forearsm and tibialis anterior muscles. Involvement is assymmetrical. Facial wekaness can occur in 1/3 of patients and dysphagis in one-half of patients.
-patients can also have loss of DTR although nerves are not involved in the poly or demato
-extramusclelar manifestations are rare
-diagnosis is bya slight elevation in CK levels
-poor response to therapy

Complications of ANK SPONDYLITIS

1. Cauda equina syndrome (this is medical emergency)
2. Osteoporosis
3. Spondylodiscitis
4. Restrictive lung disease
5. Spine fracture with spinal cord injury

Common laboratory markers in which disease: CRP

conditions (inflammatory states and infection. Miscellaneous condisitons (MI, vasculitis, trauma, malignancy, pancreatitis)

comments: primarily used for infection - much more sensitive and specific than that of ESR. If markedly elevated (>15, normal is 0-10) then expect a bacterial infection.

How is gout diagnosed?

joint aspiration and synovial fluid analysis (under a polarizing microscope) is the only way to make a definitive diagnosis - needle shaped and negatively birefringent urate cyrstals will appear in synovial fluid

-serum uric acid is not helpful in fiagnosis because it can be normal even during an acute gouty attack.
-radiographs reveal PUNCHOUT erosions with an overhanging rim of cortical bone

What pulmonary involvements are associated with RA?

pleural effusions; interstitial fibrosis may occur

Churg Strauss Syndrome

-Vasculitis involving many organ systems (pulmonary, renal, cardiac, GI, skin, neuro)
-clinical features include constitutional symptoms; prominent respiratory tract findings like (asthma, dyspnea) and skin lesions (subcutaneous nodules, palpable purpura).

Noninflammatory arthritis synovial fluid analysis:

Clear, yellow opssible red if traumatic
-WBC < 2000
-PMNs <25%
RBCs for trauma

RA v. Osteoporosis

Rheumatoid arthritis: most common cause of polyarticular inflammatory arthritis. Typically affects the hands, wrists, feet and ankles. Xrays of patients with long-standing poorly controlled disease will reveal periarticular osteoporosis, joint errosions and joint space narrowing.

Bone demineralization is hallmark for osteo. Disease predisposes patients to pathologic fractures of the hip and vertebrae but doesnt cause joint destruction itself.

Treatment of Polymyalgia Rheumatica

CORTICOSTEROIDs - will respond within 7 days
-no curative but effective for suppression of inflmammation until disease resolves itself.
-post 4-6weeks, begin to taper slowly
-60-70% of patients can stop corticosteroids within 2 years. A few patients have symptoms for up to 10 years

**self limited - on suppressive and supportive therapy for symptomatic therapy - no cure

What hematological symptoms can occur with RA?

1. Anemia of chronic disease: mild, normocytic, normochromic anemia
2. thrombocytosis

Prognosis and treatment of PAN

poor prognosis
-improved to a limited extent with treatment

Rx:
1. Start with roids
2. If PAN is severe, add cyclophosphamide

Major cause of death in patients with WEGENERS?

renal disease

Common laboratory markers in which disease: P-ANCA

Polyarteritis nodosa
-70-80% sensitive for microscopin PAN - not specific - also present in PBC likely associated with ULCERATIVE COLITIS

What is a mixed connective tissue disorder (MCTD)?

overlap syndrome with clinical features similar to those of SLE, RA, systemic sclerosis and polymyositis. Findings consistent with each of these disases do not necessarrily occur simultaneously. It usually takes some time for a pattern to be identified and a dianosis of MCTD to be made.

Treatment for reactive arthritis?

NSAIDs are first line therapy
-if there is no resoonse then try sulfasalazine and immunosuppreive agents such as AZAthioprine
-antibiotic use is controversial and not given

HLA associations with rheumatic diseases: HLA DR3

SS

What is the course of ank spondylitis?

1, slow progression but he course is highly variable

What is the cause of myopathies?

Dermatomyositis v. POlymyositis and inclusion body myositis
-dermato is humoral immune mechanism
-poly is cell-mediated

The degree involvement in patients with systemic scleroderma predicts the diagnosis: diffuse v. limited

Diffuse: worse prognosis

Which symptoms/clinical features make RA unlikely?

1. Joint distribution is not symmetric OR
2. DIP is involved OR
3. Constitutional symptoms (espeically morning stiffness) are absent

Key to diagnosing TEMPORAL ARTERITIS?

Age > 50YO
New headache
tender/palpable temporal artery
HIGH ESR *ESR 10-20, CRP 0-10
Jaw claudication

Common laboratory markers in which disease: C-ANCA

Wegeners granulomatosis (Polyangiitis with granulomas disease)
=sensitive and specific
=may vary with disease activity

Prognosis and treatment of WEGENERS?

Poor - most die within 1 year post diagnosis
-combination of cyclophosphamide and corticosteroids can induce remissions in many patients but a relapse may occur AT ANY TIME
-consider renal transplantation if the patient has end stage renal disease (ESRD)

What is feltys syndrome?

Triad of RA, neotropenia and splenomegaly:
-also anemia, thrombocytopenia and LAD
-associated with high titers RF and etra articular disease
-increases susceptibility to infection
-usually occurs fairly late in disease process

Psoriatic Arthritis

General characteristics:

Develops in fewer than 10% of patients with psoriasis
-gradual in onset
-patients usually have skin disease for months to years before the arthritis develops
-usuall asymmetric and poly articular
-upper extremties most often involved; small joints are more common than larger joints

Initial treatment: NSAIDS but persistant arthritis may req drugs similar to that of RA

What is the differential for raynauds?

Primary - no other disorder exists
Scleroderma
SLE
MCTD
Vasculitis (Buergers)
Certain meds (BB, nicotine, BLEOMYCIN)
DIsorders that disrupt blood flow or vessels such as thromboangiitis obliterans

Clinical features of RA?

1. symmetrical inflammatory polyarthritis --> symmetrical joint swelling is the msot common sign. THis can involve every joine in the body EXCEPT the DIP joints

2. pain on motion of joints/tenderness in joints

3. joints commonly inolved include joints of the hands (PIP, MCP) and wrists, kneees, ankles, elbows, hips, and shoulders

4. characteristics hands deformities
-ulnar deviation of the MCP joints
-boutonniere deformities of the pIP joints (PIP flexed, DIP hyperextended)
-swan-neck contractures of the fingers (MCP flexed, PIP, hyperextended, DIP flexed)

HLA associations with rheumatic diseases: HLA b27

Ank spondylitis, Reiters syndrome, psoriatic arthritis

Clinical features of temporal arteritis

1. constitutional symptoms
2. headaches
3. Visual impairment (25%-50%) 2/2 opthalmic atery, optic neuritis, amaurosis fugax, can lead to blindess in up to 50% of patients if not treated
4. Jaw pain with chewing
5. Tenderness to palpation over the temp artery without temp pulse
6. Palpable nodules
7. 40-50% patients also have PR

If temporal arteritis is suspected...begin which treatments empirically?

PREDNISONE and order a TEMPORAL BIOPSY

How is systemic scleorissi diagnosed?

almost all have elecated ANA - high sensitivity but low specificity
-anticentromere antibody
-anti-topoisomerase i - antischleoderma 70 AB is very specific for the diffuse form
-barium swallow (esophageal dysmotility and pulmonary function test) are used to detect complications

Treatment for Ank Spondylitis

NSAIDS
-indomethacin for symptomatic relief
-physical therapy (maintaining good posture, extension exercises)
-surgery may be necessary in some patoietns with severe spinal deformity
-pts with ank sponylitis who sustain even minor trauma and who complain of neck or back pain should be strictly immobilized to prevent spinal cord injury until thorough imaing studies are obtained

What are the SERONEGATIVE SPONDYLARTHROPATHIES?

1. Ankylosing Spondylitis
2. Reactive Arthritis
3. Psoriatic Arthritis
4. IBD associated

What is reactive arthritis?

A CLINICAL DX. It is an asymmetric inflammatory oligoarthritis of lower extremities (less common to upper) in which is preceded by an infectious etiology that is remote from the site of arthtritis (1-4 weeks prior) - most commonly 2/2 enteric and or UG infections like the clap.
-occurs most commonly in HLAb27
-REITERS syndrome is an example of reactive arthritis but most patients do not have theh classic findings of reiters (cant see cant pee cant climb a tree) so NOW we use REACTIVE ARTHRITIS

Epidemiology of SLE

women, child bearing age acct 90% cases
-AA pateints more frequency than caucasion
-mild in elderly, severe in children.

Pseudogout - general characteristics?

Calcium pyrophosphate crystals deposit in joints, leading to inflammation

Risk factors:
1. Deposition increases with age and with OA of the joints. THerefore pseudogout is common in elderly patients with degenerative joint dz - other conditions that may increase crystal deposition include hemochromatosis, hyper parathyroidism, hypothyroidism and Bartters syndrome

How is Ank Spondylitis diagnosed?

imagig studyies of lumbar spine and pelvis 9plain film, MRI, CT) reveal sacroiliitis 0 sclerotic changes in the sacroiliac area. Eventually, the vertebral columns use, producing "bamboo spine"
-elevated ESR in up to 76=5% of patients (due to inflammation) nonspecific
-HLAB27 is NOT necessary for diagnosis

What antibodies are common to the diagnosis of MCTD?

1. Increases ANA
2. Increased RF
3. Increased anti-RNP AB is a key lab finding

How might polymyositis/dermatomyositis be diagnosed?

1. Labs:
-SK level is significantly elevatd. CK levels correspond to the degree of muscle necrosis, so on can monitor disease severity
-LDH, aldolase, AST, ALT elevated
-ANA in over 50%
-antisynthetase antibodies (antijo1) abrupt onset of fever, cracked hands, raynauds interstitial lung disease, arthritis, doesnt response well to therapy
-Anti-signal recognition particle (cardiac manifestiation - worst prognosis)
-anti-mi-2 antibodes - better prognosis
-EMG - abnormal 90%

2. Biopsy
-shows inflammation and muscle fiber fibrosis in all three
-dermatomyositis - perivascular and perimysial
-polymyositis and inclusion body myositis - endomysial

Common laboratory markers in which disease: Lupus anticoagulant

antiphospholipid antibody syndrome

What features are found in both poly and dermatomyositis?

1. Symmetric proximal msucle weakness
-earlier and more severe muscle weakness n the neck flexors, should girdle, and pelvic girdle muscles.

2. Myalgia in 33% of patients
3. Dysphagia in up to 30% of patients (involvement of esophageal muscles)

What is RA?

chronic inflammatory autoimmune disease involving the synovium of joints. THe inflamed synovium can cause damage to both cartilage and bone. It is a systemic disease tat has many extra-articular manifestations
-it is a systemic disease that has many extra-articular manifestations
-the usualy age of onset is 20-40 years; it is more common in women than in men (3:1)
-disease severity is variable - some patients hae moderate restrictions and are capable of performing activites of daily living, whereas others are confined to a wheelchair or bed. Etiology is uncertain. It may be caused by an infection or a series of infections (most likely viral), but genetic predisposition is necessary.

Classic triad of REITERS syndrome?

1. Arthritis
2. Urethritis
3. Ocular inflammation (conjunctivitis) or (anterior uveitis)

*these symptoms do not always occur together

Inflammatory arthritis (RA, gout, pseudogoat, Reiters syndrome)

cloudy yellow
->5000 WBC
-50-70% PMNs
-positively birefringent crystals with pseudogout, negatively birefringent with gout

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