Rheumatology




(1)
Division of Rheumatology, Department of Medicine, University of Alberta, Edmonton, AB, Canada

 




Septic Arthritis



Differential Diagnosis of Monoarthritis



★ICU RN★





  • I nfections



    • BACTERIAL—Gonococci, Staphylococcus aureus, Streptococcus, Enterobacteriaceae, Borrelia burgdorferi, Syphilis, TB


    • VIRAL—HIV, HBV, Parvovirus, rubella, mumps, enterovirus, adenovirus


    • FUNGALCryptococcus, Blastococcus


    • OSTEOMYELITIS/OSTEONECROSIS EXTENDING TO JOINT


  • C rystalgout, pseudogout, hydroxyapatite, basic calcium phosphate


  • U nclassified



    • TRAUMA


    • OSTEOARTHRITIS


    • HEMARTHROSIS—coagulopathy, thrombocytopenia, pigmented villonodular synovitis, trauma


    • NONARTHRITIS



      • BONE—osteomyelitis, avascular necrosis, fracture


      • SOFT TISSUE—tendonitis, ligament tear, bursitis, myositis, meniscus tear


  • R heumatologic (early stage, unusual presentation as monoarthritis)



    • SEROPOSITIVEPSSR★—Polymyositis, Palindromic rheumatism, SLE, Scleroderma, Rheumatoid arthritis


    • SERONEGATIVEPEAR★—Psoriatic arthritis, Enteric arthritis, Ankylosing spondylitis, Reactive arthritis


    • SARCOIDOSIS, POLYMYALGIA RHEUMATICA


  • N eoplastic—chondrosarcoma, osteoid osteoma, metastasis


Pathophysiology



RISK FACTORS

—50% of sexually active adults with septic arthritis are due to gonococcal infections, while most patients with risk factors for septic arthritis listed below are due to non-gonococcal infections (S. aureus, Streptococci, Gram-negative bacilli)



  • COMORBIDITIES—diabetes, chronic kidney disease, rheumatologic disease, cancer, advanced disability


  • TREATMENT RELATED—immunosuppressive therapy (glucocorticoids, cytotoxic agents), prosthetic joint


  • SPECIFICS—IDU (more axial joints with MRSA, Gram negative especially Pseudomonas), endocarditis (sterile fluid as autoimmune process)


GONOCOCCAL ARTHRITIS

—more common in women. Less destructive and has better outcome than non-gonococcal arthritis. The synovial fluid Gram stain is only positive in <10%, and culture is often negative in gonococcal arthritis


COMPLICATIONS

—osteomyelitis (30%), permanent joint damage, sepsis


Clinical Features



HISTORY

—arthritis (location, duration, pain, range of motion, function), adenopathy, fever, rash, oral ulcers, alopecia, Raynaud’s, photosensitivity, sicca, trauma, recent infections, cervical/urethral discharge, sexual encounters, diarrhea, recent travel, past medical history (pre-existing joint disease, gout, rheumatoid arthritis, SLE, IBD, psoriasis, diabetes, IDU), medications (anticoagulants)


PHYSICAL

—vitals (fever), joint examination (tenderness, swelling, range of motion). Look for nail pitting, onycholysis, tophi, rheumatoid nodules, track marks, psoriasis, keratoconjunctivitis sicca, uveitis, conjunctivitis, episcleritis, murmurs, urethral discharge, and penile ulcers. Examine all joints and pay particular attention to the affected one. Soft tissue injuries (bursitis, tendonitis, muscles) usually have decreased active range of motion but normal passive range of motion, while both active and passive range of motion would be affected in joint diseases. Pelvic examination to inspect the cervix and to look for pelvic inflammatory disease


RATIONAL CLINICAL EXAMINATION SERIES: DOES THIS ADULT PATIENT HAVE SEPTIC ARTHRITIS?

















































































































































 
Sens (%)

Spc (%)

LR+

LR−

History

Age >80

19

95

3.5

0.86

Diabetes

12

96

2.7

0.93

Rheumatoid arthritis

68

73

2.5

0.45

Recent joint surgery

24

96

6.9

0.78

Hip/knee prosthesis

35

89

3.1
 

Skin infection

32

88

2.8

0.73

HIV infection

79

50

1.7

0.47

Joint pain

85
     

Joint edema

78
     

Fever

57
     

Sweats

27
     

Rigors

19
     

Physical

Fever

46

31

0.67

1.7

Investigations

Elevated WBC

90

36

1.4

0.28

Elevated ESR

95

29

1.3

0.17

Elevated CRP

77

53

1.6

0.44

Synovial fluid analysis

WBC >100,000/mL

29

99

28

0.71

WBC >50,000/mL

62

92

7.7

0.42

WBC >25,000/mL

77

73

2.9

0.32

PMN ≥90%

73

79

3.4

0.34




  • APPROACH—“when evaluating a patient with a painful, peripheral, swollen joint, the underlying pathology of a monoarthritis may be difficult to diagnose by clinical history and examination alone due to nonspecific symptoms and signs. Identifiable risk factors and arthrocentesis are most helpful in predicting septic arthritis. In particular, synovial WBC count and percentage of polymorphonuclear cells provide the best utility in identifying septic arthritis while waiting for Gram stain and culture test results. There is no evidence that a patient’s symptoms or the physical examination are useful for predicting non-gonococcal bacterial arthritis”

JAMA 2007 297:13


Investigations



BASIC





  • LABS—CBCD, lytes, urea, Cr, uric acid, ANA, RF, ESR, CRP, INR, PTT


  • IMAGING—joint XR (chondrocalcinosis in pseudogout; the presence of crystals does not rule out sepsis)


  • ARTHROCENTESIS—★3C★ (Cell count with diff, Culture and Gram stain, Crystals)


SPECIAL





  • INFECTIOUS WORKUP—urethral/rectal swabs, blood C&S


Diagnostic Issues



GOLDEN RULE

—patients with monoarthritis have septic arthritis until proven otherwise. Joint infection is a rheumatologic emergency as permanent damage can occur. Presence of crystal does not rule out infection. In up to 75% of patients with septic arthritis, a focus of infection may be found


ARTHROCENTESIS FLUID ANALYSIS





























 
Normal

Non-Infectious

Infectious

Septic

WBC (/mm3)

<200

200–2000

2000–50,000

>50,000

PMNs

<25%

<25%

25–50%

>50%


JOINT ASPIRATIONS/INJECTIONS

—for diagnostic and sometimes therapeutic reasons. Absolute contraindication is infection overlying site of injection. Relative contraindications include significant hemostasis defects and bacteremia (NEJM 2006 354:e19)



  • KNEE—flex 10–15°, enter either medially or laterally immediately beneath the undersurface of the patella slightly above midway


  • ANKLE—foot perpendicular to leg, medial approach immediately medial to the extensor hallucis longus tendon. Lateral approach just distal to fibula


  • WRISTS—flex slightly. Medial approach at dorsal surface between the distal ulna and the carpal bones. Lateral approach at dorsum just distal to the end of the radius, between the extensor tendons of the thumb


  • ADVERSE EFFECTS OF ASPIRATIONS/INJECTIONS—hypersensitivity to anesthetic, pain, infection, tendon rupture, subcutaneous atrophy, post-injection flare, systemic steroid absorption, hemorrhage, steroid arthropathy


Management


REMEMBER TO ALWAYS ASPIRATE BEFORE PROCEEDING TO TREATMENT


SYMPTOM CONTROL

—NSAIDs/opioids for pain


TREAT UNDERLYING CAUSE

empiric (if not at risk for sexually transmitted disease, nafcillin 2 g IV q4h or vancomycin 1 g IV q12h, plus ceftriaxone 2 g IV q24h or cefotaxime 2 g IV q8h. If at risk of sexually transmitted disease, nafcillin 2 g IV q4h for Gram positive organisms on Gram stain; otherwise, give ceftriaxone 2 g IV q24h or cefotaxime 2 g IV q8h if organisms not identifiable yet). Gonococcal (ceftriaxone 1 g IV q24h). Lyme arthritis (amoxicillin 500 mg PO TID, doxycycline 100 mg PO BID, or ceftriaxone 2 g IV daily × 4 weeks). Therapeutic arthrocentesis. Arthroscopic or surgical drainage (if joint inaccessible to needle drainage, organism resistant to antibiotics, or no clinical response in 3–4 days)


Gout


NEJM 2003 349:17


Causes



DECREASED URATE EXCRETION (90%)





  • RENAL DISEASE


  • DRUGSCANT LEAP★—Cyclosporine, Alcohol, Nicotinic Acid, Thiazides, Loop diuretics, Ethambutol, ASA (low dose), Pyrazinamide


INCREASED URATE PRODUCTION (10%)





  • METABOLIC SYNDROME—obesity, hyperlipidemia, hypertension


  • INCREASED METABOLISM—alcohol, hemolytic anemia, psoriasis, Lesch–Nyhan syndrome


  • NEOPLASTIC—myeloproliferative disease, lymphoproliferative disease, chemotherapy


Pathophysiology



IMBALANCE

—decreased urate excretion and/or increased urate production → uric acid crystals deposited in joints, skin, and kidneys → arthritis, tophi, and renal failure. Gout almost never occurs in pre-menopausal women (estrogen promotes higher urinary fractional excretion of urate)


PRECIPITANTS

—surgery, dehydration, fasting, binge eating, binge drinking, exercise, trauma


Clinical Features



SYMPTOMS





  • ARTHRITIS—mono/oligo and asymmetric, especially first MTP joint. Podagra, inflammation of the first MTP joint, is the presenting symptom in 75% of gout patients. However, the first MTP is also commonly affected in pseudogout, psoriatic arthritis, sarcoidosis, osteoarthritis, and trauma


  • TOPHI—yellowish-white nodular urate crystals collection in subcutaneous tissues (particularly colder extremities such as ear, fingers, olecranon bursa, ulnar aspect of forearm), bone, tendons (Achilles), cartilage, and joints. Generally painless but may lead to erosions


  • KIDNEYS—urolithiasis (radiolucent), uric acid nephropathy (reversible acute renal failure secondary to acute lysis), urate nephropathy (chronic renal failure secondary to interstitial deposits)


Investigations



BASIC





  • LABS—CBCD, lytes, urea, Cr, uric acid (sens 75%), AST, ALT, ALP, bilirubin, TSH, urinalysis, 24-h urine uric acid collection (<800 mg/day suggests ↓ excretion)


  • IMAGING—joint XR, dual energy CT


  • ARTHROCENTESIS—★3C★ (Cell count with diff, Culture and Gram stain, Crystal, for gout, sens 85%, spc 100%)


SPECIAL





  • TOPHI ASPIRATION


Diagnostic Issues



SERUM URIC ACID LEVELS

—may be falsely lowered in an acute attack


JOINT X-RAY

—soft tissue swelling, normal joint space, erosions (“punched out” and sclerotic lesions with overhanging edge)


JOINT FLUID

—ALWAYS confirm diagnosis with a synovial fluid tap if possible. Microscopy shows predominantly neutrophilic infiltrate with some intracellular monosodium urate crystals (needle shaped, negative birefringence, i.e. yellow when parallel to plane of polarized light)


Management



ACUTE

NSAIDs (first line, avoid if renal/hepatic failure; naproxen 375–500 mg PO BID × 3 days, then 250–375 mg PO BID × 4–7 days; sulindac 150–200 mg PO BID × 7–10 days; indomethacin 25–50 mg PO TID × 3 days, then 100 mg PO div BID–QID × 4–7 days; celecoxib 200 mg PO BID × 1 day, then 100 mg PO BID × 6–10 days). Systemic corticosteroids (avoid if joint sepsis not excluded; prednisone 30–60 mg PO daily × 3 days, then ↓ 10–15 mg daily × 3 days until discontinuation, triamcinolone 50 mg IM × 1 dose). Intraarticular corticosteroids (for mono- and oligoarthritis only. Methylprednisolone 40–80 mg intra-articularly once). Colchicine 0.6 mg PO daily-BID during acute attack (avoid the approach of giving colchicine q1h until development of diarrhea). Low dose colchicine regimens (≤1.8 g daily) appear as effective and are better tolerated than higher dose regimens


LONG-TERM MANAGEMENT

purine-restricted diet (↓ red meats, ↓ seafood, ↑ low-fat dairy products, ↑ fruit and veggies) have limited supportive evidence. Avoidance of beer and sugar-laden beverages is beneficial. Allopurinol 50–300 mg PO daily (first line, xanthine oxidase inhibitor, renal dose adjustment required, urate-lowering therapy generally not started in acute attack; however, continue allopurinol if already on it prior to acute attack). Febuxostat 80 mg PO daily is a newer xanthine oxidase inhibitor that can be used in patients intolerant of allopurinol or in those with mild-to-moderate renal failure. Probenecid 250–1000 mg PO BID (first line, ↓ renal urate reabsorption. Ensure normal renal function). Sulfinpyrazone 50–200 mg PO BID. Colchicine 0.6 mg PO BID × 6 months (for prophylaxis against recurrent attacks only. Do not give colchicine IV)


Treatment Issues



LONG-TERM THERAPY

—consider if patients have frequent attacks (≥3/year, tophaceous deposits, overproduction of uric acid, or continued cyclosporine treatment)


ALLOPURINOL OR FEBUXOSTAT TREATMENT

—remember to start colchicine or NSAIDs prior to allopurinol and to overlap therapy to prevent precipitating flare. Allopurinol alone can cause an abrupt decrease in serum uric acid → breakdown and release of synovial urate crystal deposits → inflammation. Aim to decrease serum uric acid level below 362 μmol/L [5.1 mg/dL]. Do not start or stop allopurinol during an acute attack


Specific Entities



CALCIUM PYROPHOSPHATE DEPOSITION DISEASE (CPPD, pseudogout)

—associated with normal urate levels and chondrocalcinosis that may be visible radiographically. Crystals appear rhomboid and have positive birefringence (blue when parallel to polarized light, yellow when perpendicular). Risk factors include old age, advanced osteoarthritis, neuropathic joint, gout, hyperparathyroidism, hemochromatosis, diabetes, hypothyroidism, hypomagnesemia, trauma, and symptoms


BASIC CALCIUM PHOSPHATE CRYSTALS

(BCPC)—crystals appear snowball-like with Alizarin red S stain. Implicated in bursitis, inflammation superimposed on osteoarthritis, and calcinosis cutis in systemic sclerosis and CREST


DIALYSIS PATIENTS

—develop destructive arthritis and tendonitis from calcium oxalate, monosodium urate, calcium pyrophosphate, and basic calcium phosphate crystals. Amyloidosis may also contribute to arthritis


Polyarticular Joint Pain and Fever


NEJM 1994 330:11


Differential Diagnosis



★RICE★





  • R heumatologic



    • SEROPOSITIVE—SLE, rheumatoid arthritis


    • SERONEGATIVE—psoriatic arthritis, enteric arthritis, reactive arthritis, ankylosing spondylitis


    • VASCULITIS—polymyalgia rheumatica, granulomatosis with polyangiitis, Behcet’s disease, Still’s disease


  • I nfections



    • BACTERIAL—septic (Gonococci), meningococci, endocarditis, Lyme disease, Whipple’s disease, mycobacteria


    • VIRAL—Parvovirus, rubella, HBV, HCV, HIV, EBV


    • FUNGAL


    • POSTINFECTIOUS/REACTIVE—enteric infections (Salmonella, Shigella, Campylobacter, Yersinia), genitourinary infections (Chlamydia), rheumatic fever, inflammatory bowel disease


  • C rystal induced—gout, pseudogout


  • E tc



    • MALIGNANCIES—acute leukemia


    • SARCOIDOSIS—Lofgren’s syndrome


    • F AMILIAL M EDITERRANEAN FEVER


    • POLYMYALGIA RHEUMATICA


    • MUCOCUTANEOUS DISORDERS—dermatomyositis, erythema nodosum, erythema multiforme, pyoderma gangrenosum, pustular psoriasis


Clinical Features



DISTINGUISHING FEATURES





  • TEMPERATURE >40 °C [>104 °F]—Still’s disease, bacterial arthritis, SLE


  • FEVER PRECEDING ARTHRITIS—viral arthritis, Lyme disease, reactive arthritis, Still’s disease, bacterial endocarditis


  • MORNING STIFFNESS—RA, polymyalgia rheumatica, Still’s disease, some viral/reactive arthritis


  • MIGRATORY ARTHRITIS—rheumatic fever, gonococcemia, meningococcemia, viral arthritis, SLE, acute leukemia, Whipple’s disease


  • EPISODIC RECURRENCE—palindromic rheumatism, Lyme disease, crystal-induced arthritis, IBD, Whipple’s disease, Familial Mediterranean fever, Still’s disease, SLE


  • PAIN DISPROPORTIONATELY GREATER THAN EFFUSION—rheumatic fever, Familial Mediterranean fever, acute leukemia, AIDS


  • EFFUSION DISPROPORTIONATELY GREATER THAN PAIN—tuberculosis arthritis, bacterial endocarditis, IBD, giant cell arteritis, Lyme disease


  • SYMMETRIC SMALL JOINT SYNOVITIS—RA, SLE, viral arthritis


  • LEUKOCYTOSIS (>15 × 109/L)—bacterial arthritis, bacterial endocarditis, Still’s disease, systemic vasculitis, acute leukemia


  • LEUKOPENIA—SLE, viral arthritis


  • POSITIVE RHEUMATOID FACTOR—RA (sens 70%, spc 70%), viral arthritis, tuberculosis arthritis, bacterial endocarditis, SLE, sarcoidosis, systemic vasculitis


  • POSITIVE ANTI CYCLIC CITRULLINATED PEPTIDE ANTIBODIES ( ACPA )—RA (not sensitive but highly spc 95%)


Investigations



BASIC





  • LABS—CBCD, lytes, urea, Cr, AST, ALT, ALP, bilirubin, uric acid, TSH, ESR, CRP, RF, anti-CCP, ANA, serologies (Borrelia burgdorferi, Streptococci, Parvovirus, HBV, HCV, HIV), c-ANCA, urinalysis


  • IMAGING—CXR, X-rays of affected joints


SPECIAL





  • ARTHROCENTESIS—★3C★ (Cell count with diff [>2000 WBC/mm3], Culture and Gram stain, Crystal)


Management


TREAT UNDERLYING CAUSE

SYMPTOM CONTROL


Specific Entities



STILL’S DISEASE





  • PATHOPHYSIOLOGY—unknown. Most consider this as a diagnosis of exclusion


  • DIAGNOSIS—major criteria include fever ≥39 °C [≥102.2 °F] (quotidian vs. diquotidian), salmon color maculopapular rash, arthralgia/arthritis ≥2 weeks, leukocytosis. Minor criteria include pharyngitis, lymphadenopathy, abnormal liver enzymes, hepatomegaly/splenomegaly, negative ANA, and RF. Need at least 2 major criteria and 3 minor criteria to make diagnosis (sens 93%). Important to exclude infections, malignancy, and acute rheumatologic disease. Significantly elevated serum ferritin


  • TREATMENTS—NSAIDs, corticosteroids, methotrexate, recombinant IL-1 receptor antagonist (anakinra)


Rheumatoid Arthritis



Differential Diagnosis of Polyarthritis



★RICE★





  • R heumatologic (>6 weeks)



    • SEROPOSITIVEPSSR★—Polymyositis, Palindromic rheumatism, SLE, Scleroderma, Sjogren’s syndrome, Rheumatoid arthritis


    • SERONEGATIVEPEAR★—Psoriatic arthritis, Enteric arthritis, Ankylosing spondylitis, Reactive arthritis, undifferentiated


    • VASCULITIS—polymyalgia rheumatica, granulomatosis with polyangiitis, Behcet’s disease, Still’s disease


  • I nfections (<6 weeks)



    • BACTERIAL—sepsis, endocarditis, Lyme disease, Whipple’s disease, mycobacteria


    • VIRAL—Parvovirus, rubella, HBV, HCV, HIV


    • FUNGAL


    • POSTINFECTIOUS/REACTIVE—enteric infections, genitourinary infections, rheumatic fever, inflammatory bowel disease


  • C rystal—gout, pseudogout, hydroxyapatite, basic calcium phosphate


  • E tc



    • MALIGNANCIES—leukemia


    • SARCOIDOSIS—Lofgren’s syndrome


    • F AMILIAL M EDITERRANEAN FEVER


    • MUCOCUTANEOUS DISORDERS—dermatomyositis, erythema nodosum, erythema multiforme, pyoderma gangrenosum, pustular psoriasis, polymyalgia rheumatica


Pathophysiology



CLASSIFICATION OF ARTHRITIS





  • MONOARTHRITIS—1 joint involved


  • OLIGOARTHRITIS—2–4 joints involved


  • POLYARTHRITIS— ≥ 5 joints involved


DESTRUCTION OF CARTILAGE

—T-helper 1 mediated process → proteases produced by synovial cells destroy proteoglycans in the articular cartilage → irreversible damage 6 months to 1 year from disease onset


POSSIBLE TRIGGERS

—viruses (Parvovirus, EBV, HTLV), super-antigens (from bacteria/viruses), autoantigens (QKRAA)


RISK FACTORS

—age >50, female (3:1), first-degree relative with rheumatoid arthritis, smoking, low level of education


Clinical Features



JOINT SYMPTOMS

—symmetric polyarthritis with joint pain, swelling, redness, morning stiffness (>1 h), and dysfunction



  • HANDS—MCP, PIP, and wrist joints most commonly involved. Deformities include Boutonniere, swan neck, Z (thumb), ulnar deviation at MCP joint, volar subluxation of proximal phalanx from MCP head, radial deviation of carpus, compression of the carpal bones, subluxation at the wrist


  • FEET—MTP joint involved. Deformities include valgus of the ankle and hindfoot, pes planus, forefoot varus and hallux valgus, cock-up toes


  • LEGS—knees (80%), ankles (80%), hips (50%)


  • ARMS—shoulders (60%), elbows (50%), acromioclavicular (50%)


  • ATLANTOAXIAL—subluxation may lead to spinal cord compression (cervical myelopathy with hand weakness/numbness)


  • TEMPOROMANDIBULAR (30%)


  • OTHERS—related disorders include Baker cyst, tenosynovitis, carpal tunnel syndrome


Related Topics

Gout (p. 311)

Inflammatory Myositis (p. 319)

Lupus (p. 317)

Scleroderma (p. 318)


EXTRA-ARTICULAR MANIFESTATIONS

—only in seropositive patients



  • RHEUMATOID NODULES (20%)


  • PULMONARY—pleural effusion (exudates, low glucose), pulmonary nodules (Caplan’s syndrome), acute interstitial pneumonitis, bronchiolitis obliterans


  • CARDIAC—valvular abnormalities, myocarditis, pericardial effusion, constrictive pericarditis


  • GI—elevated transaminases (especially ALP), nodular hyperplasia (portal hypertension, hypersplenism)


  • HEMATOLOGIC—anemia of chronic disease, Felty syndrome (triad of seropositive rheumatoid arthritis, neutropenia often associated with anemia and thrombocytopenia and splenomegaly. Patients at risk of life-threatening bacterial infections). Large granular lymphocyte leukemia, lymphoma


  • NEUROLOGIC—peripheral sensory neuropathy (not motor), myelopathy from cervical vertebral subluxation


  • OPHTHALMIC—keratoconjunctivitis sicca (Sjogren’s syndrome), scleritis, episcleritis


  • DERMATOLOGIC—vasculitis (digital arteritis, cutaneous ulceration, visceral arteritis)


  • OTHERS—amyloidosis


CONSTITUTIONAL SYMPTOMS

—fatigue (40%), fever (low grade), sweats, weight loss, myalgia


DISTINGUISHING FEATURES BETWEEN INFLAMMATORY AND NON-NFLAMMATORY ARTHRITIS









































 
Inflammatory

Non-inflammatory

Classic example

RA

OA

Morning stiffness

>1 h

+/−

Resting

Worsens

Improves

Activity

Improves

Worsens

Synovitis, redness

+


Fever, weight loss

+


ESR, CRP, platelets


No change


Investigations



BASIC





  • LABS—CBCD, lytes, urea, Cr, AST, ALT, ALP, bilirubin, ESR, CRP, RF (IgM), anti-CCP (more specific), ANA, urinalysis


  • IMAGING—X-rays of affected joints (particularly hands, knees, and ankles; soft tissue swelling, periarticular osteopenia, narrowing of joint space, marginal bony erosions, subluxation, joint destruction, bony ankylosis)


SPECIAL





  • INFECTIOUS WORKUP—serologies (Parvovirus, HBV, HCV, EBV, CMV, Borrelia burgdorferi)


  • ARTHROCENTESIS—★3C★ (Cell count with diff [>2000 WBC/mm3], Culture and Gram stain, Crystal. Cannot make definite diagnosis of rheumatoid arthritis from arthrocentesis)


Diagnostic and Prognostic Issues



ACR DIAGNOSTIC CRITERIA FOR RHEUMATOID ARTHRITIS

—add score of categories A to D, with a score of ≥6/10 classified as definite RA

A.

Joint involvement one large joint (0), 2–10 large joints (1), 1–3 small joints with or without involvement of large joints (2), 4–10 small joints with or without involvement of large joints (3), >10 joints with at least 1 small joint (5);

 

B.

Serology negative RF and negative anti-citrullinated protein antibodies (ACPA) (0), low-positive RF or low-positive ACPA (2), high-positive RF or high-positive ACPA (3);

 

C.

Acute phase reactants normal CRP and normal ESR (0), abnormal CRP or abnormal ESR (1);

 

D.

Duration of symptoms  < 6 weeks (0), ≥6 weeks (1)

 


PROGNOSIS

—increased number of joints involved, presence of rheumatoid nodules, erosions, elevated inflammatory markers and seropositivity all suggest more severe disease


Management



SYMPTOM CONTROL

physical therapy, diet (Ω-3 fatty acids). Joint protection (range of motion exercises, orthotics, splints). NSAIDs (antiinflammatory dose). Intraarticular steroid injections (if severe pain). Patient education


DISEASE-MODIFYING AGENTS OF RHEUMATOID DISEASE (DMARDs)

single agent (methotrexate with folic acid, sulfasalazine, hydroxychloroquine, cyclosporine, gold). Combination triple therapy (methotrexate plus sulfasalazine plus hydroxychloroquine). Selective pyrimidine synthesis inhibitor (leflunomide). TNFα inhibitors (infliximab, etanercept, adalimumab, golimumab). Bcell inhibitor (rituximab, an anti-CD20 monoclonal antibody). Tlymphocyte activation inhibitor (abatacept), IL-6 inhibition (tocilizumab). Surgical intervention


Specific Entities



PALINDROMIC RHEUMATISM

—episodic arthritis with one or more joints being affected sequentially for hours to days, and symptom-free periods in between for days to months. May be anti-CCP positive and occasionally progresses to other rheumatic disorders (RA, SLE). Treatment with hydroxychloroquine can be useful


SJOGREN’S SYNDROME (KERATOCONJUNCTIVITIS SICCA)





  • PATHOPHYSIOLOGY—CD4 lymphocytic infiltration of salivary and lacrimal glands


  • CAUSESprimary (sicca plus episodic, non-deforming polyarthritis), secondary (RA, SLE, scleroderma, polyarteritis nodosa, polymyositis, HIV)


  • CLINICAL FEATURES—sicca (dry eyes and dry mouth, along with impaired taste, parotid gland enlargement, dental caries), dyspareunia, arthralgia, arthritis, and constitutional symptoms. May be associated with Raynaud’s phenomenon, cutaneous vasculitis, cerebritis, CNS vasculitis, stroke, and peripheral neuropathy


  • INVESTIGATIONS—quantitative Ig (polyclonal IgG), RF, ANA, ENA (SS-A, SS-B). Check for secondary causes


  • TREATMENTS—symptomatic (artificial tears, pilocarpine 5 mg PO QID), hydroxychloroquine, rituximab may have a role


LOFGREN’S SYNDROME

—a benign self-limited form of sarcoidosis. Tetrad of erythema nodosum, hilar lymphadenopathy, arthritis (ankles and sometimes knees), and fever


RESPIRATORY DISEASES IN RHEUMATOID ARTHRITIS





  • AIRWAY—cricoarytenoid arthritis with central airway obstruction, bronchiectasis, obliterative bronchiolitis, chronic small airway obstruction


  • PARENCHYMA—pneumonia (particularly with immunosuppression), interstitial fibrosis, bronchiolitis obliterans with organizing pneumonia, rheumatoid nodules, rheumatoid pneumoconiosis, apical fibrobullous disease, drug-related pneumonitis and fibrosis (methotrexate, gold, penicillamine, NSAIDs, cyclophosphamide, azathioprine, sulfasalazine)


  • VASCULAR—pulmonary hypertension, vasculitis


  • PLEURAL—pleuritis, pleural effusion, pleural thickening


UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE

—overlap syndrome with clinical features of two or more rheumatologic disorders (RA, SLE, Sjogren’s syndrome, scleroderma, inflammatory myopathies) but does not fit the diagnostic criteria for any specific disorder


MIXED CONNECTIVE TISSUE DISEASE

—a specific overlap syndrome with clinical features of SLE, scleroderma, polymyositis, and antibodies to RNP. Characteristically, Raynaud’s phenomenon, myositis, and synovitis are present


Systemic Lupus Erythematosus



Pathophysiology



POPULATION

—typically affects women aged 15–45


AUTOIMMUNE REACTION

—antibody-immune complex deposition in kidneys (glomerulonephritis), autoantibodies against cell surface antigens on hematopoietic progenitor cells (anemia, neutropenia, thrombocytopenia), antiphospholipid antibodies (thrombosis)


ACR DIAGNOSTIC CRITERIA ★4-RASHES★





  • 4 rashes—malar rash, discoid rash, oral ulcers (usually painless), photosensitivity


  • Renal—proteinuria >0.5 g/day or ≥3+, or cellular casts


  • Arthritis ≥2 peripheral joints, non-erosive


  • Serositis—pleuritis, pericarditis


  • Hematologic—hemolytic anemia, leukopenia <4.0 × 109/L, lymphopenia <1.5 × 109/L, thrombocytopenia <100 × 109/L


  • Excitation—seizures, psychosis


  • Serology—ANA, anti-dsDNA, anti-Smith, antiphospholipid antibodies, false-positive VDRL

Need ≥4 of 11 criteria (each rash counts as one criterion and ANA as a separate criterion) to meet classification criteria. Note that many patients may not ever fulfill four criteria until several years into their disease course


Clinical Features



JOINT SYMPTOMS

—symmetric non-erosive polyarthritis with joint pain, swelling, redness, morning stiffness (>1 h), and dysfunction. Sens 88%



  • HANDS—Jaccoud’s arthritis (joint deformities are unusual). Fingers and wrists may be involved


  • LEGS—knees more commonly affected


  • AVASCULAR NECROSIS—hip, shoulder, and knee may be affected


EXTRA-ARTICULAR MANIFESTATIONS



Mar 26, 2017 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Rheumatology

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