Rheumat ology Review

Natalie Nevins, DO

7/24/2015

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Natalie A. Nevins, D.O., MSHPEDirector of Clinical Education

Western University of Health SciencesCollege of Osteopathic Medicine of the Pacific

Common Rheumatologic

Presentations in Primary Care

ACOFP Board Review

Is a chronic, systemic, inflammatory disorder of unknown etiology that primarily involves joints

◦ The most common inflammatory arthritis

◦ Arthritis is symmetrical may lead to destruction of joints due to erosion of cartilage and bone which leads to deformity

◦ Extraarticular manifestations may

be present (nodules, neuropathy, scleritis, pericarditis, splenomegaly)

◦ F>M 2:1, mean age 50-55 (peak age 35-55)

Rheumatoid Arthritis (RA)

Morning stiffness > 1 hour Arthritis of three or more joint groups with

soft tissue swelling Swelling involving 1 or more joint groups:

wrist, proximal IPJ, MCP, MTP Active symmetric joint swelling Hand X-ray (usually normal for 1st two years)

changes typical of RA that must include erosions or unequivocal bony decalcification

Subcutaneous nodules + Rheumatoid factor

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The characteristic joint deformities appear in more established chronic RA. These findings include ulnar deviation swan neck or Boutonniere deformities of the fingers, or the “bow string” sign (prominence of the tendons in the extensor compartment of the hand)

Occasional patients present with extensor tendon rupture, most commonly affecting the thumb, little or ring fingers of either hand.

RA - Hands

DMARDS are divided into two categories: nonbiological and biological◦ The nonbiological: methotrexate, sulfasalazine, lefunomide

(Pyrimidine synthesis inhibitor), all primary options. Hyroxychloroquine: typically used in combination with the other DMARDS.

◦ The biological DMARDS: target specific cytokines or their receptors, such as tumor necrosis factor. Other types of biological DMARDS include B cell depleting agents and Tcellcostimulatory blockers. The use of biologic DMARDS has been referred to as “targeted therapy”

1st line for mild-moderate disease at initial presentation

Possible options:◦ Mild-moderate disease is usually started on a single

DMARD. MTX most common first line drug. Other options leflunomide (LEF), sulfasalazine (SSZ), and hydroxychloroquine (HCQ)

The addition of ONE of the following agents to MTX:◦ tumor necrosis factor (TNF) inhibitor: adalimumab,

etanercept, or infliximab, abatacept (T cell costimulation blocker), rituximab (Pre-B cell depleter), or anakinra (interleukin-1 receptor antagonist)

Treatment

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Most common form of chronic arthritis in children

Onset <16 y/o

Pain, joint swelling, decreased rom > 6 weeks

3 subtypes (per ACR criteria):◦ Systemic: 10-20% fever, evanescent rash

◦ Polyarticular: 30-40%, > 4 joint involvement (large and small)

◦ Pauciarticular: 40-50%, <=4 joints (large), risk for chronic uveitis in girls and axial skeleton in boys.

ANA (baseline test)+ 40% (poly or pauci)

RA factor (2 positive tests required for diagnosis of RF pos PA JIA) + 10-15%

HLA-B27 + 70% of Pauci boys

ESR (maybe normal or elevated) nonspecific

Radiology: soft tissue swelling, periosteal reaction, juxta-articular demineralization

First line PT and OT with lifestyle modifications in conjunction with Nsaids (effective in 50%)

DMARDS if not responsive

Multisystem, Autoimmune inflammatory condition

Genetic Markers: HLA-B8, HLA-DR2, HLA-DR3

Risk factor Age 15-45, F>M 10:1

Increased risk in African American, Hispanic, Asian and Native American

Hereditary Compliment deficiency: C1q, C1r, C1s, C4 and C2

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Fever

Vasculitis

Panniculitis

Myositis

Avascular Necorisis

Endocarditis

Ascites

Venous thrombosis

Pulmonary Fibrosis

Renal failure

Peripheral neuropathy

Stroke syndromes

Pancreatitis/ elevated LFT’s

Infertility

Seizures

1. Malar rash (Butterfly) 2. Discoid rash 3. Photosensitivity rash 4. Oral ulcers 5. Arthritis: Nonerosive Involving 2 or more peripheral joints 6. Serositis: Pleuritis or Pericarditis a) Pleuritis 7. Renal Disorder a) Persistent proteinuria > 0.5 grams per day OR b) Cellular

casts--may be red cell, hemoglobin, granular, tubular, or mixed 8. Neurologic Disorder a) Seizures OR b) Psychosis 9. Hematologic Disorder a) Hemolytic anemia--with reticulocytosis OR b)

Leukopenia--< 4,000/mm3

on ≥ 2 occasions OR c) Lyphopenia--< 1,500/ mm

3on ≥ 2 occasions OR d) Thrombocytopenia--<100,000/ mm

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10. Immunologic Disorder a) Anti-DNA: antibody to native DNA in abnormal titer OR b) Anti-Sm: presence of antibody to Sm nuclear antigen OR c) Positive finding of antiphospholipid antibodies on: an abnormal serum level of IgG or IgM anticardiolipin antibodies, a positive test result for lupus anticoagulant using a standard method, or a false-positive test result for at least 6 months confirmed by Treponema pallidumimmobilization or fluorescent treponemal antibody absorption test.

11. Positive Antinuclear Antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs

Adds: Nonscarring alopecia

Low complement

Direct Coombs' test

Subdivides:

◦ Acute vs chronic cutaneous lupus

◦ Oral or nasal ulcers

◦ Hemolytic anemia/ Leukopenia or lymphopenia/ Thrombocytopenia

◦ Anti-dsDNA/ Anti-Sm/ Antiphospholipid antibody

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+ ANA (15% false pos in elderly)

Anti-DS DNA

Anti-SM

False + VDRL

+ LE Prep

Increased creatinine

+ Coombs test

Sed Rate (nonspecific)

Anemia

Anticardiolipin AB

Leukopenia/Lymphopenia/thrombo-cytopenia

Proteinuria

Activated PTT (prolonged with anti phos AB)

Inflammatory reaction to URATE crystals in joints, bones and subcutaneous structures

Crystals in joint fluid is pathognomonic

Hyperacute arthritis◦ Primary: Most common, under-excretion or

overproduction of uric acid

◦ Secondary: related to myloproliferative DZ, treatments inducing hyperuricemia, renal failure/tubluar disorders, glycogen storage dz

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Age 30-60, M>F 20:1

Risks: ETOH, Fam hx, MEDS (diuretics induce 20% of secondary gout), obesity/HTN (50%), diet

S/S: <24 hour onset of severe joint/soft tissue: pain, swelling, redness/warmth in 1-2 joints. 75% monoarticular, 1st MTP in 50% of 1st attack.

Sub q or interosseous nodules 20%, called tophi

X-ray normal 1st year◦ Chronic gout gets “punched out” erosions with

“overhanging edge” of periostium over the erosion.

Arthrocentesis with synovial fluid analysis, Wet mount: synovial fluid strongly negatively birefringent urate crystals on the polarizing exam

Uric acid: get 2 weeks after attack

Resolves, may be false low or normal

during attack (>7mg/dl men, >6mg/dl women)

Risk of kidney stones

Avoid foods high in purines, such as liver and other organ meats, veal, turkey, and some types of fish, including anchovies, shrimp, mackerel, and scallops.

Stop drinking large amounts of alcohol. Alcohol interferes with excretion of uric acid, and alcoholic beverages contain purines

Lose Weight

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Acute: NSAIDs (first line) for 10-14 days Treatment of gout should be initiated with an NSAIDs to

control acute inflammation. At the maximum recommended doses, NSAIDs effectively treat arthritis caused by crystals.

◦ Unlike the newer, equally effective NSAIDs, indomethacinfrequently causes dyspepsia and can cause central nervous system side effects such as headache and mental status changes

Antigout Agents Colchicine (second line), may be helpful with patients who

cannot tolerate or have contraindications to NSAIDs and corticosteroids.

◦ With the availability of other agents, however, there is little role for colchicine in the treatment of acute gout, particularly in elderly patients.

Recurrent Gout: 2-3 weeks post acute episode◦ First line: Urate lowering agent

Allopurinol, Febuxostat

Naproxen Ibuprofen Diclofenac Potassium Meloxicam Celecoxib Febuxostat Triamcinolone acetonide Prednisone/methylpred/ Indomethacin Probenecid Sulindac Allopurinol Colchicine

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Acute inflammatory arthritic disease usually involving large joints

Arthrocentesis Synovial fluid Calcium pyrophosphate dihydrate crystal (CPPD) deposition disease

Associated with chondrocalcinosis 80% > 60 y/o Knee involved 50% of all attacks 50% with fever Elevated sed rate, leukocytosis (may have left

shift) Nsaids

Triad of Arthritis, conjunctivitis and either urethritis or cervicitis. 4th feature may be buccal ulceration or balanitis

Sterile joint inflammation with infection starting at non-articular site

2 forms: ◦ Sexually transmitted: S/S emerge 7-14 days after

sex (Chlamydia usual organism)

◦ Postdysenteric (shigella, salmonella, yersina, campylobacter). More common in Women, children and elderly.

HLA-B27 in 60-80% 20-40 y/o M>F Ankylosing spondylitis develops in 30-50% in

those + for HLA-B27 Asymmetric arthritis (knees, ankles, MTP) Enthsopathy Urogenital tract: Urethritis/prostatitis etc. Eye: Conjunctivitis/scleritis/keratitis Skin: mucocutaneous ulcers Constitutional: fever, malaise, wt loss

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most common presentation is an asymmetric arthritis, usually a large or medium sized joint of the lower extremities after an infection that the patient may or may not remember

WBC: 10-20,000

Increased neutrophils

Increased sed rate

Normochromic anemia

Hypergammaglobulinemia

Ongoing segmental inflammatory, systemic necrotizing vasculitiswithin the media of medium sized muscular arteries

Multisystem involvement: fever, wt loss, malaise, Skin (livedoreticularis), CNS (HA, sz), Renal, MSK, GI, Lung, Cardiac

Labs: nonspecific, may have RF, endothelial cell AB, high neutrophil, anemia, elevated sed rate & C-reactive protein. Hepatitis surface antigen + in 10-50% of cases. Negative ANA and RF

BX of involved organs: necrotizing vasculitis Angiogram with aneurysmal changes Treatment (Non-HBV related):

Good Prognosis: PrednisonePoor Prognosis Prednisone and DMARDSevere disease is treated with cyclophosphamide

1. Weight loss: of 4 kg or more of body weight since illness began, not due to dieting or other factors

2. Livedo reticularis

3. Testicular pain or tenderness Pain or tenderness of the testicles

4. Myalgias, weakness or leg tenderness Diffuse myalgias (excluding shoulder and hip girdle) or weakness of muscles or tenderness of leg muscles

5. Mononeuropathy or polyneuropathy Development of mononeuropathy, multiple mononeuropathys, or polyneuropathy

6. Diastolic BP >90 mm Hg Development of hypertension with diastolic BP higher than 90 mm Hg

7. Elevated BUN or creatinine Elevation of BUN >40 mg/dl or creatinine >1.5 mg/dl

8. Hepatitis B virus Presenece of hepatitis B surface antigen or antibody in serum

9. Arteriographic abnormality Arteriogram showing aneurysms or occlusions of the visceral arteries, not due to arteriosclerosis, fibromuscular dysplasia, or other noninflammatorycauses

10. Biopsy of small or medium-sized artery containing PMN Histologic changes showing the presence of granulocytes or granulocytes and mononuclear leukocytes in the artery wall

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Widespread pain

Stiffness

Poor sleep

Fatigue

Swelling in soft tissue (especially hands)

Numbness in the extremities

Headaches

Restless Leg Syndrome

Diarrhea Abdominal pain Tender joints Limited range of

motion Jaw pain Memory impairment Menstrual cramping Dizziness Skin and chemical

sensitivities

Restless sleep and fatigue Hx of widespread pain & Pain in 11 of 18 tender point sites on digital

palpation for at least 3 months

Occiput: Bilateral, at the suboccipital muscle insertions. Low cervical: bilateral, at the anterior aspects of the intertransverse spaces at C5-C7.Trapezius: bilateral, at the midpoint of the upper border. Supraspinatus: bilateral, at origins, above the scapula spine near the medial border.Second rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces.Lateral epicondyle: bilateral, 2 cm distal to the epicondyles. Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle.Greater trochanter: bilateral, posterior to the trochanteric prominence.Knee: bilateral, at the medial fat pad proximal to the joint line.

80-95% are women

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First Line: Graded Aerobic Exercise: Walking, Pool, Strength training Cognitive - behavior therapy Good sleep hygiene

Second line: Mind-Body Therapies: Biofeedback, Guided Imagery,

hypnosis

FDA Approved Meds:◦ Tricyclic Antidepresants◦ Cymbalta (Duloxetine HCl)◦ Lyrica (Pregabalin )◦ Savella (Milnacipran HCl)