Musculoskeletal- Joint - Inflammatory Disease Flashcards

1
Q

Rheumatoid Arthritis

A

Chronic autoimmune inflammatory disease of insidious onset with systemic manifestations including skin, blood vessels, heart, lungs, and muscle.

  • Principally produces a symmetric polyarthritis generally affecting mainly small joints in the hands and feet, as well as larger joints such as the wrists and shoulders.
  • The arthritis is a sterile proliferative and inflammatory synovitis progressing to destruction of the cartilage and ankylosis of the joint.

AKA: RA, Rheumatoid disease, Atrophic arthritia

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2
Q

Rheumatoid Arthritis - Epidemiology

A
  • Incidence is 0.36/1000 in women, and 0.14 in men per year
  • Prevalence is 0.05-0.15/1000 in western countries
  • Approx. 1% of adults in the world’s population (including the US) is affected with RA
  • Genetic susceptibility, strongly associated with the HLA-DR4 and to a lesser extent to the HLA-DR1 and HLA-DR14 genotypes
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3
Q

Rheumatoid Arthritis - Etiology

A
  • Origin of the autoimmune inflammatory process is unknown
  • Unknown antigen involved in triggering the disease
  • There is a common genetic varient at the TRAF1-C5 locus on chromosome 9 that is associated with an increased risk of the anti-CCP positive form of RA (TRAF1 gene encodes an intracellular protein that mediates signal transduction involving the TNF receptor)
  • Other gene variants associated with risk for RA:
  • PTPN22 (T-cell receptor signaling)
  • PADI4 (affecting the citrullination of proteins, targets of the anti-CCP antibodies)
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4
Q

Rheumatoid Arthritis - Pathogenesis

A
  • Synovium becomes edematous with perivascular infiltration of myeloid cells
  • Fronds or villous projections develop from the synovium
  • Infiltration by inflammatory cells (CD4+ T-cells and B-cells)
  • release of cytokines IL-1 and TNF-a
  • synovial cells to produce prostaglandins and matrix metalloproteinases
  • Articular cartilage destruction
  • increased vascularity
  • Aggregation of fibrin
  • Accumulation of neutrophils
  • Osteoclastic activity in underlying bone driven by RANKL from T-cells and synovial fibroblasts
  • Pannus formation
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5
Q

Rheumatoid Arthritis - Pathology

A
  • Disordered cytoarchitecture in the synovium
  • hyperplasia of the resident stromal cells such as fibroblasts
  • heavy infiltration of hematopoetic cells such as T and B lymphocytes
  • lymphocyte accumulations strongly resemble lymph nodules
  • Rheumatoid nodules occur in the dermis but can form in other organs as well
  • Vasculitic changes can occur in severe cases
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6
Q

Rheumatoid Arthritis - Presentation

A
  • Usually subacute (20%) or insidious (70%) onset of arthritic symptoms of pain, swelling and stiffness
  • Prominent morning stiffness, often lasting hours
  • Affected joints are often warm, sometimes reddened
  • Can be accompanied by low grade fever and fatigue
  • CRITERIA FOR DIAGNOSIS:
  • morning stiffness lasting at least 1 hour
  • soft tissue swelling of three or more joints
  • swelling of the PIP, MCP, or wrist joints
  • symmetric arthritis
  • Subcutaneous nodules
  • postive test for RA factor
  • Radiographic erosions or periarticular osteopenia in hand or wrist joints
  • joints involved include:
  • most common: MCP, wrist and PIP
  • knee, shoulder, ankle, hip, and elbow present later
  • MTP C-spine, and TMJ can also be involved
  • Hand deformities associated with RA include:
  • fusiform swelling
  • boutonniere deformity
  • swan-neck deformity
  • ulnar deviation of fingers
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7
Q

Rheumatoid Arthritis - DIagnostic testing

A
  • ESR and CRP levels
  • Rheumatoid factor
  • Anti-cyclic citrullinated peptide antibody
  • Diagnostic Imaging, findings include (ABCDEs):
  • characteristic Alignment abnormalities with no ankylosing
  • periarticular osteoporosis seen in associated Bones
  • Cartilage shows uniform joint space loss
  • Deformities present in hand
  • Erosions present but are marginal
  • soft tissue swelling
    Additional tests can include:
  • CBC
  • Serum electrolytes and creatinine levels and liver function tests to monitor therapy
  • stool guaiac test to rule out gastrointestinal bleeding
  • urinalysis to rule out renal disease if needed
  • synovial fluid analysis to rule out crystal deposition diseases in appropriate
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8
Q

Rheumatoid Arthritis - Treatment

A
  • Acetaminophen
  • NSAIDs
  • Corticosteroids
  • DMARDs
  • methotrexate
  • antimalarial drugs
  • sulfasalazine
  • leflunomide
  • Etanercept
  • Infiximab
  • Adalimumab
  • Rituximab
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9
Q

Rheumatoid Arthritis - Management with compliations

A
  • Depression can be a major issue in patients with RA
  • Physical therapy and occupational therapy when needed
  • Surgical intervention and joint replacement
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10
Q

Rheumatoid Arthritis - Prevention

A
  • Evidence exists to support the use of complementary therapies involving the elimination of food allergies, vegetarian diet
  • Patient Education
  • Physical modalities to reduce pain and maintain function such as manipulation, occupational therapy, and physical therapy
  • Psychological support and referral for counseling when appropriate
  • Dietary modification
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11
Q

Juvenile Idiopathic (Rheumatoid Arthritis)

A

idiopathic synovitis of the peripheral joints with associated soft tissue swelling and joint effusion. It is one of the most common rheumatic disorders of childhood.

AKA - JRA, Juvenile Rheumatoid Arthritis

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12
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - History

A
  • prevalence of JRA has been estimated to be 57-113 cases per 100,000 children under the age of 16. (may vary by location)
  • Most common form of childhood arthritis
  • Three types:
  • Systemic (10% of cases)
  • Polyarticular (40% of cases)
  • Pauciarticular or oligoarthritis (50% of cases)
  • Pauciarticular and polyarticular disease occur more frequently in girls, while both sexes are affected with equal frequency in systemic onset disease
  • Altered bone mineral metabolism
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13
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - Presentation

A
  • Less then 16 years old
  • Persistent arthritis in one or more joints for period of 6 weks
  • exclusion of all other types of arthritis
  • Four KEY POINTS:
  • Arthritis: defined as swelling, effusion and two or more of the following:
  • Limited range of motion
  • Tenderness
  • Pain on motion
  • Joint warmth
  • consistently present for 6 weeks
  • more than 10 other causes must be excluded
  • Systemic JRA
  • number of joints variable
  • 1-2 daily fever spikes exceeding 101
  • rash
  • systemic manifestations
  • Polyarticular JRA
  • 5 or more joints are involved
  • Pauciarticular JRA
  • 4 or less joints are involved
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14
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - Diagnostic Testing

A
  • DIagnosis is based o history and clinical presentation
  • No lab test can establish diagnosis
  • Elevated WBC count
  • ESR and C-reactive peptide
  • Elevated ANA in 40-85% of cases with poly- or pauarticular JRA
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15
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - treatment

A
  • Pyramid approach:
  • beginning with NSAIDs adequate for a minority of children
  • many children require:
  • Corticosteroids
  • Methotrexate
  • Sulfasalazine
  • Ethanercept
  • Infiximab
  • Adalimumab
  • Adalimumab
  • Rituximab
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16
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - Complications

A
  • Disease can persist into adulthood
  • Chronic uvitis
  • Anemia
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17
Q

Juvenile Idiopathic (Rheumatoid Arthritis) - health promotion

A
  • Psychosocial problems

* Physical and occupational therapies

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18
Q

Reactive Arthritis

A
  • An asymmetrical sterile arthritis occuring usually within one month of an infection elsewhere in the body. Most likely an autoimmune reaction set off by the preceding infection.

AKA - Reiter’s syndrome (old term, inapporpriate); Enteropathic Arthritis, Acute nonpurulent arthritis, postinfectious arthritis

19
Q

Reactive Arthritis - History

A
  • Approx. 3.5 - 4.0 cases per 1000 men peryear
  • Predominately occuring in men, and most often affecting knee or ankle
  • Presentation is strongly associated with presence of HLA-B27 genotype (>80%)
  • Age range is typically in the 20-30 year group
  • Preceding infection commonly involves the mucosa of the Genitourinary system and gastrointestinal system (chlamydia, Shigella, Salmonella, Yersinia, Campylobacteria)
  • Waxing and waning pattern of inflammation
  • Reoccurence is common
  • Non-articular manifestations can include:
  • skin and mucosal lesions
  • Eye: conjunctivitis
  • Enthesopathy
20
Q

Reactive Arthritis - Presentation

A
  • Triad (often referred to as Reiter’s syndrome
  • Arthritis
  • Urethitis or cervicitis (nongonococcal)
  • conjunctivitis
  • Joint stiffness and low back pain
  • Ankles, knees and feet, often asymmetric in distribution
  • Sausage finger or toe secondary to synovitis of the tendon sheath
  • Spondylitis
21
Q

Reactive Arthritis - Diagnostic Testing

A
  • Diagnosis is primarily clinical in nature
  • Arthrocentesis and aspiration to rule out other causes
  • Radiographic imaging, findings include (ABCDEs):
  • Ankylosis
  • Bone Reactivity
  • cartilage loss and joint space narrowing
  • Distribution primarily in the lower extremity
  • Erosions are common in the MTP joints
  • Sift tissue swelling
  • HLA-B27 determination has very low diagnostic value, but has good prognostic value correlating with the severity of the disease and its non-articular manifestations.
22
Q

Reactive Arthritis - Treatment

A
  • Non-pharmaceutical management involves bed rest, hot and cold treatment followed by gradual return to function
  • Pharmaceutical management involves eliminating the infection and the use of NSAIDs and analgesics when appropriate
  • Glucocorticoid therapy if appropriate
23
Q

Arthritis of Inflammatory Bowel Disease

A
  • Arthritis occuring in association with inflammatory bowel disease, which includes Chron’s disease and ulcerative colitis

AKA - Enteropathic arthritis

24
Q

Arthritis of Inflammatory Bowel Disease - Epidemiology

A
  • In patients with IBD, the prevalence of peripheral arthritis and/or sacroilitis/spondylitis is 10-20%
  • the incidence of ulcerative colitis (UC) is 6-8 cases per 100,000 per year, and prevalence is 70-150 cases per 100,000
  • Affects makes and females equally
25
Q

Arthritis of Inflammatory Bowel Disease - Pathogenesis

A
  • Studies suggest that there is a direct trafficing of lymphocytes from the gut wall to the synovium of the joints
26
Q

Arthritis of Inflammatory Bowel Disease - Pathology

A
  • Aseptic inflammation of the joint
27
Q

Arthritis of Inflammatory Bowel Disease - Presentation

A
  • Symmetric, persistent or migratory polyarthritis affecting primarily the joints of the lower extremity
  • Spondylitis can be present involving stiffness in back and/or buttocks
28
Q

Arthritis of Inflammatory Bowel Disease - Diagnosis

A
  • Rheumatoid factor negative
  • X-Rays of affected joints do not usually demonstrate the process
  • Non-specific joint inflammation inflammatory on synovial biopsy
29
Q

Arthritis of Inflammatory Bowel Disease - Treatment

A
  • Responds to successful treatment of the bowel disease

* Treatment with NSAIDs can exacerbate the underlying inflammatory bowel disease

30
Q

Ankylosing Spondylitis

A
  • Chronic, progressive autoimmune inflammatory disease of insidious onset that involves a loss of range of motion in the joints of the spine, sacrum and proximal ribs.

AKA - AS, Rheumatoid spondylitis, Marie-Strumpell Disease, Bechterew’s disease

31
Q

Ankylosing Spondylitis - History

A
  • Frequency is est. at 0.1-0.2% in the general population (129 out of 100,000)
  • Typically presents in the 2nd and 3rd decade of life, male to female ration of 2-3 to 1, but can present in childhood (11-86 in 100,000)
  • Chronic inflamatory disease of axial joints, especially the SI joint
  • Approximately 90% of patients with AS are HLA-B27 positive; however, the majority of people positive for HLA-B27 do not have AS
  • Absence of B27 in patients with definitive AS increases the likely hood of associated inflammatory bowel syndrome
  • Etiology might involve previous enteric infection triggering an immune response
  • Chronic synovitis precedes bony ankylosis of the joints
  • Enthesitis lead to ossification of tendoligamentous insertions forming bony outgrowths
  • Fracture of the spine, uveitis, aortitis and amyloidosis are recognized complications
  • Cauda equine syndrome secondary to inflammation and stenosis in the spinal canal.
32
Q

Ankylosing Spondylitis - Presentation

A
  • Insidious onset of back pain prior to 40 years of age
  • Morning stiffness in axial spine greater than 1 hour duration
  • Bilateral sacroiliac tenderness secondary to sacroilitis
  • Loss of flexibility in lumbar spine
  • Loss of chest expansion
  • Loss of usual spinal curvature
  • progressively worsening back pain
  • Spinal fractures secondary to osteoporosis
  • Extra axial manifestations:
  • Iritis
  • chest pain, shortness of breath
  • pulmonary fibrosis
  • atrioventricular bolck
  • weight loss, fatigue and anorexia
  • Enthesitis in other areas (Achilles enthesitis, sausage toe)
  • large joint synovitis
33
Q

Ankylosing Spondylitis - Diagnosis

A
  • x-ray of spine and pelvis, AP and lateral - bamboo spine
  • Fusion (ankylosis) of the SI joints
  • CT of spine and SI joint (usually not necessary for diagnosis)
  • RI and CT can detect changes earlier than radiography
  • MRI shows edema surrounding the SI joints
  • ESR can C-reactive protein
  • HLA-B27 test (not necessarily for diagnosis)
  • Bone scintigraphy (rarely used in diagnosis)
  • Schober’s test for lumbar mobility
34
Q

Psoriatic Arthritis

A
  • chronic, inflammatory spondyloarthritis of insidious onset involving the spine and peripheral joints that develops in patients with psoriasis.
  • Patients are usually negative for rheumatoid arthritis

AKA - Arthopathic psoriasis, seronegative arthritis

35
Q

Psoriatic Arthritis - History

A
  • About 1-3% of the population has psoriasis and of that group, 5-10% of psoriatic patients have psoriatic arthritis
  • First presents between 30 to 50 years of age (note, juvenile psoriatic arthritis presents between 9-12 years of age)
  • Males and females are affected equally, except males are more likely to have SI joint involvement
    I In most cases there is a large time of two decades between the onset of psoriasis and the presentation of the arthritis
  • Psoriasis is a Th1 disorder similar to RA
  • Histologically similar to RA with less joint destruction
  • Can be monoarthritis (most common), oligoarthritis, or polyarthritis in distribution
  • Genetic predisposition has been noted; high concordance in twin studies
  • HIV patients have a predisposition to developing psoriatic arthritis
  • Genetic factors: 50% are positive for HLA-B27
36
Q

Psoriatic Arthritis - Presentation

A
  • Insidious onset in 70% of patients; acute onset in the others; fourth or fifth decade of life
  • Psoriatic lesions (thickened, scaly rash) can be hidden: check the scalp, perineum or umbilicus
  • Asymmetric joint involvement, typically a monoarthritis, but can be symmetric
  • Warm, swollen joints with restricted ROM
  • Morning stiffness and lethargy
  • DIP joints of hands and feet are often first to present (95%)
  • Large joints such as ankles, knees hips and wrists follow
  • Axial spine, especially sacroilliac joint involvement can also occur (5%)
  • Sausage finger, due to inflammation of the digital tendon sheaths
  • Nail pitting
  • Severe joint destruction can occur in the variant termed arthritis multilans
  • Can be accompanied by conjunctivitis and iritis (<20% of cases)
  • Pitting of nails in cases with peripheral arthritis (80% of cases)
37
Q

Psoriatic Arthritis - Diagnostic Testing

A
  • Seronegative arthritis, thus RA is negative
  • ESR and CRP levels
  • Rheumatoid factor
  • Uric acid levels
  • Radiographic imaging, findings include:
  • asymmetric oligoarticular involvement
  • involvement of the DIP
  • erosions of the terminal tufts of the DIP joints
  • Erosions can be very significant; destructive arthritis (arthritis mutilans)
  • cupping of the proximal portion of the phalanges (pencil-in-cup appearance)
  • proliferation of bone near the erosion
  • Bony ankylosis distal to MCP joints
  • SI and spondylitic changes
38
Q

Felty’s Syndrome

A
  • Felty’s syndrome is a complication of long-standing rheumatoid arthritis, and is defined by the presence of 3 conditions: rheumatoid arthritis, an enlarged spleen (splenomegaly), and an abnormally low white blood count (granulocytopenia).
  • Affects less than 1% of patients with rheumatoid arthritis
39
Q

Felty’s Syndrome - History

A
  • In the US, rheumatoid arthritis effects approximately 1% of the general population and Felty’s syndrome occurs in 1-3% of those with RA
40
Q

Felty’s Syndrome - Presentation

A
  • Rheumatoid Arthritis
  • Splenomegaly
  • Abnormally low white blood cell count
41
Q

Sjogren’s Syndrome

A
  • chronic, slowly progressive, inflammatory autoimmune disease of insidious onset that is characterized by lymphocytic (CD4+ T-cell and B-cell) infiltration of exocrine glands, especially the salivary and lacrimal glands, and sometimes the pancreas

AKA - Sicca syndrome, Mikulicz syndrome, Gougerot syndrome, Autoimmune exocrinopathy

42
Q

Sjogren’s Syndrome - History

A
  • Although recognized in patients of all ages, it primarily affects women in the fourth and fifth decade of life (female to male ratio is 9:1).
  • Rare in childhood, with a 3 year old girl being the youngest reported patient.
  • Second most common autoimmune disorder, after rheumatoid arthritis
  • Between halfa million and 2 million individuals are affected by this disease in the USA
  • Primary form of the disease is termed “sicca syndrome”
  • Secondary form presents with other forms of autoimmune disorders
  • Ribonuclear proteins SS-A (To) and SS-B (La) detected in 90% of patients with primary Sjogren disease and is also present in cases of SLE
  • Secondary form has antibody profile of the associated disease
  • Titer level of SS-A corresponds to the severity and spread of the disease
  • Genetic component related to class II molecules, e.g. HLA-DQA1 and HLA-DQB1
  • Also a suggested viral origin to the autoimune attack:
  • Epstein-Barr virus
  • Hepatitis C virus
  • Human T-cell lymphotrophic virus type 1
  • Periductal and perivascular lymphocytic infiltration in the lacrimal and salivary glands
  • progressing to the development of lymphoid follicules with germinal centers
  • Late stage involves atrophy, fibrosis and hyalinization of the gland
43
Q

Sjogren’s Syndrome - Presentation

A
  • Keratoconjunctivitis (xerophthalmia) and dry mouth (xerosomia)
  • Bilateral parotid gland swelling
  • Redness in the eye
  • Blurring of vision
  • Burning, itching eyes
  • Oral dryness, parched tongue and dysphagia
  • Change in taste
  • Other manifestations include:
  • skin
  • lung
  • kidney
  • liver
  • peripheral neuropathy
44
Q

Sjogren’s Syndrome - Diagnostic Testing

A
  • Serum autoantibody assay (ANA, SS-A, SS-B)
  • Minor lip salivary gland biopsy
  • Schirmer’s test
  • Rose Bengal score or lissamine green dye test