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
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
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)
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
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
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
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
8
Q
Rheumatoid Arthritis - Treatment
A
- Acetaminophen
- NSAIDs
- Corticosteroids
- DMARDs
- methotrexate
- antimalarial drugs
- sulfasalazine
- leflunomide
- Etanercept
- Infiximab
- Adalimumab
- Rituximab
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
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
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
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
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
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
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
16
Q
Juvenile Idiopathic (Rheumatoid Arthritis) - Complications
A
- Disease can persist into adulthood
- Chronic uvitis
- Anemia
17
Q
Juvenile Idiopathic (Rheumatoid Arthritis) - health promotion
A
- Psychosocial problems
* Physical and occupational therapies
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
Arthritis of Inflammatory Bowel Disease - Pathogenesis
* Studies suggest that there is a direct trafficing of lymphocytes from the gut wall to the synovium of the joints
26
Arthritis of Inflammatory Bowel Disease - Pathology
* Aseptic inflammation of the joint
27
Arthritis of Inflammatory Bowel Disease - Presentation
* 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
Arthritis of Inflammatory Bowel Disease - Diagnosis
* Rheumatoid factor negative
* X-Rays of affected joints do not usually demonstrate the process
* Non-specific joint inflammation inflammatory on synovial biopsy
29
Arthritis of Inflammatory Bowel Disease - Treatment
* Responds to successful treatment of the bowel disease
| * Treatment with NSAIDs can exacerbate the underlying inflammatory bowel disease
30
Ankylosing Spondylitis
* 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
Ankylosing Spondylitis - History
* 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
Ankylosing Spondylitis - Presentation
* 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
Ankylosing Spondylitis - Diagnosis
* 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
Psoriatic Arthritis
* 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
Psoriatic Arthritis - History
* 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
Psoriatic Arthritis - Presentation
* 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
Psoriatic Arthritis - Diagnostic Testing
* 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
Felty's Syndrome
* 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
Felty's Syndrome - History
* 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
Felty's Syndrome - Presentation
* Rheumatoid Arthritis
* Splenomegaly
* Abnormally low white blood cell count
41
Sjogren's Syndrome
* 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
Sjogren's Syndrome - History
* 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
Sjogren's Syndrome - Presentation
* 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
Sjogren's Syndrome - Diagnostic Testing
* Serum autoantibody assay (ANA, SS-A, SS-B)
* Minor lip salivary gland biopsy
* Schirmer's test
* Rose Bengal score or lissamine green dye test
