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					NOTES: 5/23/08
          Anti-cyclic citrullinated peptide is useful in the early diagnosis of rheumatoid arthritis and is equally
sensitive (@ 67%) but much more specific (95%) than rheumatoid factor (85%) (Nishimura K. Ann Int Med
2007;146:797) and predicts progression of undifferentiated arthritis to rheumatoid arthritis (van Gaalen FA, 2004).
          The earliest lesion in rheumatoid arthritis is an increase in the number of synovial lining cells with
microvascular injury.
          Complications of RA: Hammer toe, swan neck deformity, ulnar deviation, and Boutonniere deformity.
          Eye manifestations of rheumatoid arthritis include sicca complex syndrome, with decreased tear formation
as a result of secondary Sjogren's syndrome, and "corneal melt" with linear ulcerations usually at the limbus, leading
to corneal perforation.
          In RA, DIP joint involvement is distinctly unusual.
The most commonly involved joints in the rheumatoid hand are the PIPs, MCPs, and wrist.
Path: Microvascular injury with increased number of synovial lining cells is the earliest lesion.
RF is IgM gainst Fc portion of IgG. Not pathogenic. RF level is predictive of disease severity.
Anti-cyclic citrullinated peptide antibody detected by ELISA has a S & S of 62 & 92%.
RA has highest incidence of infective arthritis (more than Osteoarthritis, renal failure, or DM).
The first treatment is NSAIDs. If these fail, use methotrexate, which is the 1 st choice because of relatively rapid
onset. However, gold, pencillamine, antimalarials, sulfasalazine, low dose prednisone, and TNF blockers
(infliximab, etanercept, adalimumab) are also effective.
For methotrexate: frequent non-life threatening side effects: GI sx's. Less common: alopecia and stomatitis. Potential
effect on sperm: men should discontinue methotrexate 3 to 4 months before attempting conception. Serious adverse
effects (rare): hepato-toxicity, marrow suppression and interstitial lung disease: v WBC, v Platelets, pneumonitis,
and cirrhosis.
Lab monitoring: pregnancy test at baseline and CBC, platelet count, alkaline phosphatase, and AST. Repeat
monthly.

Patients with adult Still's disease are RA negative.
In RA, RA antibodies are specific for Fc portion of human IgG.
Sensitivity: 75%.
RF is an IgM antibody recognizing Fc determinants of autologous IgG. It is not pathogenic.
Specificity low: sarcoid, Sjogren's, SBE, and vasculitic syndromes.
RA extra-articular CMs: vasculitis, RA nodule of aortic valve, cacchexia, pleuritis, and pericarditis.
Pleural effusions is often asymptomatic, exudative, WBC < 5,000 /mm3, very low glucose.


RA course at 10 years:
80%+ have residual disability or joint destruction.
50% have work disability.
Predictors of poor prognosis:
20+ inflammed joints.
^^ ESR
^^ Rheumatoid factor
Rheumatoid nodules
^ Age at onset
Sustained disease for more than 1 year
White females versus white males.
The median life expectancy of patients with RA is shortened by 3 to 7 years.
Contributors to decreased mortality are RA itself, increased CV risk, steroid use.

For early aggressive disease, treat with a combination of MTX and Anti-TNF alpha druge (e.g., etanercept).

MTX induced lung disease usually has fever and abrupt onset (Q 59, p 80).

Anti-TNF alpha has 6 side effects: Serious infections including disseminated TB, pancytopenia,
demyelinating disorders, exacerbations of congestive heart failure (hence contraindication to anti-TNF alpha in those
with EF < 50%), hypersensitivity to the
infusion or injection, and the development of drug-induced systemic lupus erythematosus.

Use capsaicin cream to control pain of arthritis, particularly hands & wrists.

NSAIDs are contraindicated in renal insufficiency.

NSAIDs should be taken 2 hours after ASA in ASH or CVA patients on ASA because they block the ASA-
antiplatelet effect.

Inhibitors of tumor necrosis factor alpha are effective in rheumatic diseases but infrequently cause infections, CHF,
lymphoma, and demyelinating syndromes (PMFLE). Infection: extra-pulmonary TB (e.g., renal TB) is the most
common form of TB. PPD positivity is set at 5 mm, and patients given INH for 9 months as indicated.
Pneumococcal and influenza vaccines are given to all patients. Patients with fever on anti-TNF-alpha drugs are
diagnosed and treated aggressively as if splenectomized. Afebrile patients are given oral drugs to be given at the
sign of first infection.

Lymphoma: There was a suggestive but not established increase in lymphoma in patients on adalimumab in a clinical
trial.

Celecoxib (Celebrex) has not been withdrawn (as of 2005) from the market, unlike other COX 2
inhibitors, but should be avoided in high risk cardiovascular patients, though it can be used in
patients on anti-coagulants (e.g., ASA).

The treatment of anti-phospholipid antibody syndrome in pregnancy with prior fetal loss criteria
(more than 3 prior to the 10th week, one spontaneous abortion of a normal fetus at or after the
10th week, or one premature birth of a normal fetus at or prior to the 34th week) should be treated
with ASA + LMWH (MKSAP 13 update).

Both SLE and Rheumatoid arthritis patients are at increased risk for CAD (MKSAP 13 update).

Gout: Cyclosporine, treatment of malignancy, meats, diuretics (HCTZ), obesity, beer, and
hypothyroidism are risk factors for gouty attacks and appropriate steps should be taken. All of
these decrease uric acid secretion with the exception of malignancy under treatment and meats.

30 yo man. Intermittent painful swelling of right great toe, left ankle, and left knee every month
relieved by ibuprofen.
Also, he has HPT and Crohn’s disease and takes azathioprine and prednisone 5 mg/day.
He drinks alcohol on weekends.
PE: Joint exam is normal.
Uric acid, CBC, creatinine, RF, LFTs, and electrolytes are normal. ESR=40.
What is the correct management: Watchful waiting, allopurinol, low dose colchicine,
indomethacin, or increase prednisone to 10 mg/day?
Answer:
Ddx: Gout versus arthritis associated with IBD.
Watchful waiting and confirm gout with joint aspiration.
If confirmed, allopurinol is given.
Along with this give a prophylactic anti-inflammatory drug such as colchicine (low dose: 0.6 mg
BID), prednisone, or indomethacin. However, in this patient both indomethacin and colchicine
are contraindicated because of the IBD.

In under excreters (85% of gout patients; defined as less than 800 mg uric acid/day), no renal
insufficiency, no history of renal stones, and no problem with TID compliance, give probenecid.

If all of those criteria are not met, give allopurinol (which is QD). However, adverse effects are
>in 3% to 5%: rash, fever, diarhea, v WBC, and v platelets,
>rarely: a potentially fatal (25%) rash, fever, hepatitis, and renal failure, particularly when
given in standard dose allopurinol with renal insufficiency with diuretics.

What advice do you give patients who are taking allopurinol?
Answer: If a rash occurs, stop the drug and never restart it, else it may precipitate fatal TEN or
hypersensitivity syndrome with hepatitis and renal failure (25% fatality rate).
Tophi can occur on heart valves and spinal canal causing a parapersis.

Foods to eat: cheese, eggs and fruit. Foods to avoid: Diuretics, beer, and meats. Treat ^ TSH.

Pseudogout can be prevented by colchicine.

55 yo man
cc: intermittent attacks of joint pain lasting 1 week for past 10 years
PE: Hallux valgus deformity of both 1st MT joints.
PIP joints: left 1st and 4th and right 2nd and 3rd joints.
HARD NODULES OVER OLECRANON BURSA, swollen, not tender or warm.
X-ray: 1st MP bony erosion and 1st MT joint soft tissue density.
Dx: Is this RA, gout, pseudogout, osteroarthritis, or tenosynovitis universalis (?)
Ans: Gout. NOT RA.
Tophaceous deposits of monosodium urate over olecranon. Also can be found over the ear and
achilles tendon.
        Prednisone (to 30+ mg/d) is associated with alleviating symptoms of an acute gouty
flare within a few days and could be tapered during inter-critical periods.
        Allopurinol can cause TEN, hepatitis, agranulocytosis, and hypersensitivity syndrome
(erythematous rash, fever, hepatitis, eosinophilia and renal failure).

       Rheumatologic emergencies:
*In RA, loss of PIP or MP Joint Extension. >>>>> Tendon Rupture.
*Acute pain, swelling of single joint + fever. >>>>>>> Septic arthritis
*Tender spinous process.      >>>>>>>> Mets to vertebra. OR Vertebral Abscess.
*Sudden blindness w/HA or jaw claudication in elderly patient >>>>>> Giant cell arteritis.
*Bladder or bowel dysfunction with back pain >>>>> S.C. Compression, transverse myelitis,
cauda equina syndrome, or mets.
*Asymmetric lower leg swelling >>>>> DVT.
Arthritis & skeletal disease can antedate the clinical onset of IBD.

SLE (and RA) are major risk factors for coronary artery disease.

When starting a female pre-menopausal patient on warfarin, always remember to get a pregnancy
test.
Beta blockers are contraindicated in Scleroderma and in Raynaud’s.
Prednisone is contraindicated in Scleroderma as it may facilitate scleroderma renal crisis.
ACEIs must be given in scleroderma to prevent renal failure.

In inflammatory myopathies, a muscle biopsy must be preceded by an MRI to identify the best
site for biopsy.

5 pulmonary renal syndromes:
1) Goodpasture’s syndrome: anti-glomerular basement membrane antibody.
2) Five Pulmonary-renal vasculitides:
        Cryoglobulinemia
        Wegener’s (C-ANCA/antiproteinase-3),
        Microscopic Polyangiitis, and
        Churg-Strauss (last two are P-ANCA/antimyeloperoxidase). (alveolar hem, ^ eos, FSGN,
mono-neuritis multiplex, subcu nodules.

Increased ESR is caused by :
1. Anemia
2. Neutralization of negatively charged sialic acid residues on RBC surfaces >> decreased RBC
repulsion, congregation of RBCs, and more rapid sedimentation. Causes of neutralization are:
a) Increase in fibrinogen (often from synthetic response of liver to inflammation or infection, or
drugs such as OCPs)
b) Increase in the ratio of total protein/albumen (e.g., myeloma)
A DECREASE IN THE ALBUMEN (e.g., from nephrotic syndrome) CAUSES AN INCREASE
IN THE ESR !!!

RHEUMATIC MANIFESTATIONS OF SELECTED DISEASES August 30, 2004
                             joints                                            Other
Disease
                             MP joints (2nd & 3rd digits) & wrists
Hemochromatosis
                             Symmetric, large joints, sacroiliitis.            Tophyrema Whippelii
Whipple’s Disease
                             Carpal tunnel syndrome, polyarthropy of large
Amyloidosis
                             limb joints with subchondral bone cysts &
                             erosions. Destructive arthropy of the C-spine
                             SBE can present as excruciating back pain or as
SBE
                             a peripheral non-septic arthritis.
                             Peripheral or Sacral                              Can Present With Oral Ulcers !!
Inflammatory Bowel disease

Lymphoma                                                                       Without MTX: Increased risk.
                                                                               With MTX: EBV + B cell lymphoma.
                             Ankles bilaterally
Sarcoidosis
                             Hands, wrists, knees, ankles, shoulder            Unlike IBD, GI symptoms are painless
Celiac Sprue
       JOINT PATTERNS OF ARTHRITIS                          August 30, 2004
Rheumatoid Arthritis              Wrist, MP, PIP, Elbow, Shoulder (late),
                                  MT joint of foot, Tarsus, retrocalcineal bursitis, Tibio-talar joint
                                  Knee
                                  Hip (late)
                                  Cervical Spine (watch for long tract findings)
                                  Cryco-aretynoid joint (laryngeal stridor) 30%

Parvo virus B19                   15% of new onset poly arthritis. Joint pattern is identical to RA. Lab Dx: IgM & IgG serology.

Osteoarthritis                    Knee, Hip, Base of Thumb (squaring), PIP (Heberden’s) & DIP (Bouchard’s–most
                                  common)joints
                                  1st Meta-Tarsal joint (Hallus Valgus & Rigidus)
                                  Shoulder (acromioclavicular joint)
                                  Spine

Gout                              1st MT joint, knee, ankle, instep( 80% of all attacks in lower extremity)
                                  Wrist, finger, shoulder, hip, sternoclavicular joint, and even spine.

Pseudo-gout =                     Knee, Ankle, 1st MT joint (i.e., weight bearing joints), wrist.
calcium pyrophosphate             Hypothyroidism, hemochromatosis, hyperparathyroidism, hypomagnesemia, hypothyroidism
deposition disease (CPPD)         Chondrocalcinosis refers to finding calcification on x-ray.

Hemochromatosis                   MCP joints, 2nd & 3rd digits; Pseudo gout (particularly the knees)

Arthritis with inflammatory       Central: Spondylitis & sacroiliitis.
bowel disease                     Peripheral: Knee, MCP (ankle, elbow, shoulder, wrist, PIP)

Arthritis with psoriasis          Several types:
(= psoriatic arthritis)           *DIP (Distal type) (nails have pitting)
                                  *Oligoarticular asymmetric, Small & large type.
                                  *Symmetric poylarthritis (rheumatoid like)
                                  *Dactylitis (arthritis mutilans)
                                  *Spondylo-arthropathy (sacroiliitis & spondylitis)

Infectious arthritis              *Site of prosthetic surgery.
(monoarticular)                   *Site of Adjacent surgery (e.g., GYN operation can lead to pubic symphysis infection)
                                  *Site of prior non-infectious arthritis (e.g., RA [most common] or OA)
                                  *Overall, the knee is the site in 50%, then the wrist, ankles & hips.
                                  *Gonoccocal arthritis: Knee, wrist & ankle. (May have multiply infected joints.)

SLE                               Symmetric: PIP, MCP, wrists, knees. (Like RA) Effusions are common, deformities unusual
                                  (10% develop swan neck at MCP joints), and erosions are rare.
                                  HIP: aseptic necrosis if receiving steroids (also in shoulder & knee)

Reactive arthritis                MTP, PIP, DIP (sausage digits), wrists, sacro-iliitis, knee, achilles tendon.

Sarcoid                           Ankles & knees (less so: PIP joints, wrists, elbows). Acute onset.

Subacute bacterial endocarditis   can present as excruciating back pain or as a peripheral non-septic arthritis.

       Criteria for RA: (4+ of 7):
       1. Morning stiffness
       2. Symmetric arthritis
       3. Typical joint distribution (PIP, MP,
       wrists)
       4. Multiple joints
       5. Subcutaneous nodules
       6. Radiographic erosions
       7. +ve Rheumatoid factor or Anti-CCP

       Remember to check for Hepatitis C, which
       can give a positive rheumatoid factor and
       joint pain!!!
CLASSIFICATION OF SCLERODERMA                                   August 30, 2004
                    Skin involvement with      Pulmonary HPT           Internal Organs               AB
Name
                    Raynaud’s & dilated
                    nailfold capillary loops

Diffuse Cutaneous   YES.                       No                      Early:                        ANA in 98%
Scleroderma         Truncal & acral skin                               Interstitial Lung disease.    Anti-Scl-70
                    involvement                                        Renal disease (ACEI block)    (Anti-
                    Puffy, hidebound skin.                             Wide mouth diverticulae of    topoisomerase
                    Tendon friction rubs.                              Colon.                        I) in 30%
                                                                       Cardiomyopathy.
                                                                       Late:Ca of lung increased.

Limited Cutaneous   YES.                       YES but late in 15%.    Late, if at all:              Anti-
Scleroderma.        Limited to hands, face,                            GI disease or                 Centromere Ab
                    feet & forearms.                                   Interstitial Lung Disease.    in 70%.
                    CREST* in some.
                    YES. With evidence of                                                            Any of the
Pre-scleroderma
                    digital ischemia.                                                                above.
                    NO, except may have                                Early: Interstitial Lung      Any of above
Scleroderma sine
                    Raynaud’s                                          Disease, Renal disease, GI
scleroderma
                                                                       disease, myocardial disease
                    Either Diffuse or          SLE, RA,
Overlap syndromes
                    Limited.                   dermatomyositis,
                                               vasculitis, Sjogren’s
*CREST= Calcinosis, Raynaud’s, Esophogeal dysmotility, Sclerodactyly, Telangiectasia. This
occurs in some, but not all, patients with Limited Cutaneous Scleroderma.

Polyarteritis Nodosa:
Hypertension and/or renal insufficiency                    60%
Arthritis, arthralgias or myalgias                         64%
Peripheral neuropathy                                 51%
Abdominal pain, GI bleed, or ischemia or
infarctions of bowel, liver or pancreas                    44%
Rash, purpura, livido reticularis, uclers or Raynaud’s     43%
CHF, MI, or pericarditis 36%
CVA or seizures                                            23%.
Hepatitis B                                                25%
Hepatitis C                                                5%
Best dx for PAN is by biopsy of affected nerve which will show vasculitis.

(Hepatitis C is associated with essential cryoglobulinemia.)

CRITERIA FOR LUPUS
Four or more are required. There are 11 which can be thought of as:

Skin-mucous mb - 4
Arthritis-Serositis - 2
Hem, cns, kidney - 3
Autoimmune & ana - 2

1. Malar rash
2. Discoid rash: PASTE: Plugging, Atrophy, Scarring, Telangiectasias, Erythema
3. Photosensitivity – UVA and UVB so get sun screen that blocks both. Coppertone Spectra 3.
4. Ulcers (painless)
5. Arthritis: HAM: Knees & Hands, Asymmetric, Migratory, Usually Non-Deforming. If
deforming in the hands, they are reducible.
6. Serositis – pericarditis and pleuritis
7. Hematologic – hemolytic anemia (warm autoimmune, Anti-IgG and Anti-C3),
thrombocytopenia, lymphopenia.
8. CNS: Seizures & Psychosis.
9. Glomerulonephritis (proteinuria, RBCs, RBC casts): I. None, II. Mesangial proliferative, III
Focal proliferative, IV Diffuse proliferative GN, V Membranous GN.
10. Autoimmune: Anti-DS DNA, APLA, LAC, False + VDRL, Anti-Smith AB.
11. Positive ANA (usually peripheral pattern).

Other symptoms AND signs:
fatigue 95%
Increased AST and ALT (10 x nlormal) (Liver inflammation; hence increase in fibrinogen and
increase in ESR!!)
alopecia
vasculitis (bleeding or perforation of GI tract in 5%)
panniculitis
endocarditis
ASHD
conjunctivitis or sicca syndrome in 15%
Venous thrombosis in 10%
Cerebrovascular disease in 10%
SLE causes a transverse myelitis manifest by cauda equina syndrome (difficulty urinating,
incontinence, decreased sphincter tone, erectile dysfunciton) and lower extremity weakness (Q
64, p81, Q 27, p y70).

In general, the alopecia associated with active lupus may improve with treatment of the disease,
except with discoid lesions, in which there is follicular plugging and scarring that may result in
permanent alopecia.

SLE eye involvement: retinal vascular disease, cotton-wool spots (cytoid bodies) or retinal
exudates from distended and disrupted axons resulting from anoxia.
Hemorrhage is commonly observed in the retina.

Renal biopsy is not necessary in SLE patients whose renal function is rapidly deteriorating when
they have an active sediment.
Indications for biopsy: a) failure to respond to glucocorticoid therapy and b) patients with mild
clinical disease (to determine if they have active, severe, inflammatory lesions, which might
respond to therapy).

In Antiphospholipid antibody syndrome, keep the INR at 3.0 (Q53, p79)

Athralgias of wrist and clubbing of digits may be hypertrophic osteoarthropathy with periosteal
new bone formation, due to lung cancer, suppurative lung disease, congenital heart disease; relief
of the feet with elevating the legs is said to be pathognomonic. Glucosamine relieves OA pain.
To diagnose sarcoid, obtain transbronchial lung biopsy, showing a mononuclear granulomatous
inflammation.


This is dermatomyositis: heliotrope                          This is Pemphigus Vulgaris

				
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