Kembara Xtra - Medicine - Ankylosing Spondylitis

Kembara Xtra - Medicine - Ankylosing Spondylitis 
Ankylosing spondylitis (AS) is an axial inflammatory spondyloarthropathy (axSpA) characterized by chronic low back pain (lasting more than three months) and sacroiliitis-related radiographic abnormalities (sclerosis, erosions, and changes in joint width).
Musculoskeletal, Ophthalmic, Cardiovascular, Neurologic, and Pulmonary Systems Affected
● Synonyms: Marie-Strümpell illness; "bamboo spine”

Peak age of onset is between 20 and 30 years; after age 40, it is uncommon.
Males outnumber females by a factor of 2 to 3:1.
Incidence
6.3 to 7.3/100,000 personyears, adjusted for age and gender
Prevalence in the United States is 0.55 percent for AS and 1.40 percent for all axSpA.

ETIOLOGY AND PATHOPHYSIOLOGY Autoinflammation at sites of bacterial exposure (such as the intestines) or mechanical stress in genetically susceptible people Enthesopathy, an inflammation at the insertion of tendons, ligaments, and fasciae to bone that results in erosion, remodeling, and the formation of new bone.

Genetics Endoplasmic reticulum aminopeptidase 1 (ERAP1), interleukin-23 receptor (IL23R), and gene deserts on chromosomes 2p15 and 21q22 are further genetic correlations with AS that affect 85–95% of individuals.

RISK ELEMENTS
A child with a positive HLA-B27 family history who has an AS parent has a 10–30% chance of getting the condition.

CONDITIONS OFTEN Associated with
arthritis in the periphery (30%)
Enthesopathy (29%): plantar fasciitis and Achilles tendonitis Uveitis (25–35%)
(10%) Psoriasis
"Sausage digit" Dactylitis (6%)
"IBD" or inflammatory bowel disease (4%)

Aortitis, aortic regurgitation, and cardiac conduction abnormalities are all symptoms of peripheral spondyloarthritis (SpA), which also includes psoriatic arthritis, reactive arthritis, IBD-related arthritis, and juvenile idiopathic arthritis.

DISEASE HISTORY

 Inflammatory back pain - Insidious start usually before the age of 40; length of more than three months; morning stiffness in the spine lasting more than an hour; nightly awakenings brought on by back pain; pain and stiffness worsen at rest and get better with exercise.
Constitutional symptoms (fatigue, poor sleep, weight loss, and low-grade fever) as well as alternating buttock/hip discomfort are frequent.
Other enthesopathy symptoms include dactylitis (sausage digits), iritis (unilateral discomfort, red eye, photophobia, vision changes/blurring), and Achilles tendon and plantar fascia pain.

TENDANCE in the sacroiliac joint, loss of lumbar lordosis, and rotation of the cervical spine on physical examination
A reduction in the lumbar spine's range of motion in all three planes.
Lumbar spine flexion modified Wright-Schober test:
– Mark the L5 spinous process on the patient's back (or the Venusian dimples) and take measurements 10 cm above and 5 cm below this location. Tell the patient to flex their hips forward. Between these two markers on maximal flexion, there should be at least 5 cm of expansion.
Thoracocervical kyphosis, which typically develops after at least 10 years of symptoms
- The distance between the occiput and the wall when one is standing with their back flat against a vertical surface raised (zero is normal).
Chest wall respiratory excursion decreased
- Measure the chest wall's circumference at the fourth intercostal gap. A rise of >5 cm is considered normal at maximum respiratory excursion, while a rise of 2.5 cm is consistent with AS.
Achilles and plantar fascia tendon insertion sites are tender; peripheral oligoarthritis/dactylitis is typically found with peripheral SpA; extra-articular symptoms include uveitis, psoriasis, and inflammatory bowel disease; and aortic regurgitation murmur (1%)

Mechanical low back pain and nonradiographic axSpA (AS characteristics with sacroiliitis evidence on MRI but not on plain radiographs—can progress to AS) are two different diagnoses.
Osteitis condensans ilii: benign sclerotic changes in the iliac portion of the SI joint after pregnancy; Other inflammatory arthritis; Osteoarthritis or erosive osteochondritis of the axial spine; Diffuse idiopathic skeletal hyperostosis (DISH); Infectious arthritis; or discitis, unilateral sacroiliitis: tuberculosis, brucellosis,
Fibromyalgia—tender points can mirror enthesitis findings—vertebral compression fracture, familial Mediterranean fever, and

DETECTION & INTERPRETATION OF DIAGNOSIS
ESR and C-reactive protein (CRP) levels may be just raised or normal; if elevated, they are correlated with the activity and prognosis of the disease.

 Lack of the rheumatoid factor
(15%) Mild normochromic anemia
Alkaline phosphatase levels may mildly increase in severe illness. Synovial fluid may have modest leukocytosis.
Oblique projection is better for SI joints.
– Joint abnormalities are categorized as follows:
Grades 0 and 1 correspond to normal, mild erosions or sclerosis without changes to joint width, moderate or severe sacroiliitis with erosions, sclerosis, increased joint space, or partial ankylosis, and grade 4 corresponds to total ankylosis.
Spine: preferable lateral view
– Early radiographic changes include "shiny corners" caused by osteitis and sclerosis at the annulus fibrosus attachment sites to the corners of vertebral bodies and "squaring" caused by erosion and vertebral body remodeling. Contrast-enhanced MRI is more sensitive for detecting early abnormalities.
Ankylosis of apophyseal joints, ossification of spinal ligaments, and/or spondylodiscitis are further late alterations that can cause bone bridging between vertebral bodies (syndesmophytes), providing the distinctive "bamboo spine" appearance.
Peripheral joints may have erosions, sclerosis, loss of joint space, and asymmetric pericapsular ossification.
– Enthesitis can be detected with ultrasound. Initial Tests (Lab, Imaging)
The Assessment of SpondyloArthritis International Society (ASAS) modified Berlin algorithm is used to make the diagnosis:
Obtain an anterior-posterior x-ray of the sacroiliac joints if the patient has experienced low back pain for at least three months and the narrative and physical examination raise suspicion of AS.
- The diagnosis of AS is sufficient if the patient exhibits at least one other typical SpA symptom (as stated below) and the sacroiliitis is judged to be grade 2 bilaterally or grade 3 unilaterally.
Inflammatory back pain, heel pain (enthesopathy), dactylitis, uveitis, positive family history of SpA, inflammatory bowel disease, alternating buttock pain, psoriasis, asymmetric arthritis, positive response to NSAIDs, elevated ESR or CRP are all considered sufficient for the diagnosis of AS if the plain AP x-ray is negative for sacroiliitis.
- If the patient exhibits two to three of the aforementioned symptoms and the simple AP x-ray is negative for sacroiliitis, a positive HLA-B27 test will suffice to make the diagnosis.
Further testing with an MRI of the sacrum to check for sacroiliitis is advised if the plain AP x-ray shows no evidence of the condition and the patient only has 0 to 1 of the aforementioned symptoms and a positive HLA-B27.
Interpretation of Tests
Ossification of the periarticular soft tissues is caused by erosive alterations and new bone development at the bony attachment of the tendons and ligaments.

GENERAL TREATMENT MEASURES
The major therapy objectives are to minimize symptoms, preserve spinal flexibility and normal posture, lessen functional restrictions, preserve work capacity, and lessen illness consequences.
The most significant non-pharmacologic intervention is aggressive physical therapy.

First Line: MEDICATION

The first-line pharmacologic treatment for pain and stiffness in AS is nonsteroidal anti-inflammatory medications (NSAIDs) [A].
- Ibuprofen up to 800 mg TID; naproxen up to 500 mg BID; and celecoxib up to 200 mg BID
NSAIDs' hazards for CVD, GI, and renal disease should be taken into account. Use with caution if a patient is taking anticoagulants or has a bleeding disorder.
Surgery may be an option for patients with atlantoaxial subluxation complicated by neurologic impairment, however intra-articular corticosteroids injected into SI joints and prosthesis can offer momentary relief.
pregnant women's issues
Infants who are exposed to NSAIDs during the first trimester may be more likely to develop heart abnormalities.

Next Line
Tumor necrosis factor (TNF) antagonists are biologic treatments that are advised for treating active disease after at least two NSAIDs have failed to control it for a month. Etanercept, a recombinant TNF receptor fusion protein, infliximab, a fully humanized IgG1 monoclonal antibody to TNF-, golimumab, a human IgG1 kappa monoclonal antibody to TNF-, and certolizumab pegol, a pegylated humanized monoclonal antibody to TNF- are among the AS medications approved by the FDA.
TNF-blockers: use with caution Serious bacterial, mycobacterial, fungal, opportunistic, and viral infections are made more likely by anti-TNFs. Check for hepatitis B and tuberculosis.
– Anti-TNF treatment has been linked to lymphomas, nonmelanoma skin cancers, and other cancers, according to reports.
– Before starting anti-TNFs, vaccinations should be updated, especially live vaccines, as live vaccines are contraindicated once patients have received anti-TNFs.

Disease-modifying antirheumatic medications (DMARDs), such as methotrexate and sulfasalazine, are ineffective for treating axial disease; however, they may be useful for treating peripheral arthritis in patients who cannot take TNF inhibitors.

QUESTIONS FOR REFERENCE
 Coordination of care with a rheumatologist for anti-TNF therapy and diagnosis, monitoring, and management.
A referral to the appropriate speciality may be necessary for the management of aortic regurgitation, uveitis, spinal fractures, pulmonary fibrosis, hip joint involvement, and cauda equina syndrome.

ADVANCED THERAPIES

Taking bisphosphonates if you have osteoporosis or osteopenia

SURGICAL AND OTHER PROCEDURE

Before intubating patients with AS who are undergoing surgery, check for C-spine ankylosis or instability.
Vertebral osteotomy can help individuals with significant cervical or thoracolumbar flexion by enhancing posture.

CONTINUING CARE AFTERCARE RECOMMENDATIONS
patient observation
Check posture and range of motion every six to twelve months; if disease activity is more pronounced, come more frequently.
Measures of disease activity include the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and the Ankylosing Spondylitis Disease Activity Score (ASDAS).
Fall evaluation and prevention
Periodic evaluation of the CRP or ESR

 Using a dual energy x-ray absorptiometry scan to check for osteopenia and osteoporosis

EDUCATION OF PATIENTS
Maintain a healthy level of exercise and posture by engaging in activities like swimming, water aerobics, tai chi, and walking.
 Steer clear of trauma and contact sports.
The severity and rate of ankylosis progression might vary greatly.


COMPLICATIONS
Osteoporosis, spinal fusion causing kyphosis, c-spine fracture or subluxation, and cauda equina syndrome (rare) are all MSK/spine-related conditions.
 Pulmonary: upper lobe fibrosis (rare), restricted lung disease
Cardiac: pericarditis (very rare), aortic insufficiency, aortitis, and conduction abnormalities at the atrioventricular (AV) node
Eye: cataracts, uveitis
IgA nephropathy with amyloidosis (1%) in the kidneys
GI: up to 50% of individuals have microscopic, subclinical ileal and colonic mucosal ulcerations, the majority of which are asymptomatic.