berkenalan dengan ragam penyakit autoimun
TRANSCRIPT
Mengenal ragam autoimun
Any organ . . .Autoimmunity can affect ANY organ/organ system in the human body
Pemphigus
Multiple Sclerosis
SLE
Rheumatic Fever
Autoimmune Hepatitis
Ulcerative Colitis
Goodpasture’s Syndrome
Diabetes
Autoimmune Uveitis
Autoimmune hemolytic Anemia
Addison’s Disease
Rheumatoid Arthritis
Autoimmune Oophoritis
Autoimmunity Classification
Can be classified into clusters that are either organ-specific or systemic
Rheumatoid Arthritis
Images from: www.hss.edu/conditions_an-in-depth-topic-review-of-rheumatoid-arthritis.asp
Rheumatoid Arthritis“A chronic autoimmune disease characterized by the inflammation of the synovial joints”
Has a symmetrical bilateral effect on joints
Results in joint deformity and immobilization
Multiple factors increase one’s risk
(The Arthritis Society, 2012; Gulanick & Myers, 2011; Firth, 2011)
Symptoms•Morning stiffness lasting more than half an hour•Simultaneous symmetrical joint swelling•Not relieved by rest•Fever•Weight loss•Fatigue•Anemia•Lymph node enlargement•Nodules•Raynaud’s phenomenon(The Arthritis Society, 2012; Firth, 2011; Oliver, 2010; Day et al., 2010)
Nodules
(Arthritis Foundation, 2012; Day et al., 2010; American College of Rheumatology, 2009)
Disability in Early RA
• Inflammation– Swollen– Stiff– Sore– Warm
• Fatigue
• Potentially Reversible
Periarticular OsteopeniaJoint Space Narrowing
ErosionsMal-Alignment
RHEUMATOID ARTHRITIS – SYSTEMIC AUTOIMMUNE / AUTO INFLAMMATORY DISEASE--
How dose RA affect all these organ systems ?
By causing… 1. Synovitis
2. Serositis 3. Nodules4. Vasculitis
5. Autoantibodies
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RHEUMATOID LUNG - MOST SERIOUS VISCERAL ORGAN AFFECTED BY RHEUMATOID ARTHRITIS
**LUNG Most common small
bilateral pleural effusions
Cause of lung fibrosis Pulmonary vasculitis Caplan’s syndrome
Rheumatoid nodules
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CorticosteroidsExamples General Use Side Effects Nursing
Considerations
Cortisone, hydrocortisone, prednisone, betamethasone,dexa-methasone
• Used in the management inflammatory conditions •When NSAIDS may be contraindicated•Promptly improve symptoms of RA
•Increased appetite•Weight gain•Water/salt retention•Increased blood pressure•Thinning of skin•Depression•Mood swings•Muscle weakness•Osteoporosis•Delayed wound healing•Onset/worsening of diabetes
•Take medications as directed (adrenal suppression)•Used with caution in diabetic patients•Encourage diet high in protein, calcium, potassium and low in sodium and carbohydrates•Discuss body image•Discuss risk for infection
(The Arthritis Society, 2011; Day et al., 2010)
Disease-modifying anti-rheumatic drugs(DMARDS)
Examples General Use Side Effects Nursing Considerations
Methotrexate (the gold standard), gold salts, cyclosporine, sulfasalazine, azathioprine
•immunosuppressive activity•Reduce inflammation of rheumatoid arthritis•Slows down joint destruction•Preserves joint function
•Dizziness, drowsiness, headache•Pulmonary fibrosis•Pneumonitis•Anorexia•Nausea•Hepatotoxicity•Stomatitis•Infertility•Alopecia•Skin ulceration•Aplastic anemia•Thrombocytopenia•Leukopenia•Nephropathy•fever•photosensitivity
•May take several weeks to months before they become effective•Discuss teratogenicity, should be taken off drug several months prior to conception•Discuss body image
(The Arthritis Society, 2011; Day et al., 2010)
Biologic Response Modifiers (“Bioligics”)
Examples General Use Side Effects Nursing Considerations
Etanercept, anakinra, abatacipt, Adalimumab, Infliximab
• Used in the management inflammatory conditions •When NSAIDS may be contraindicated•Promptly improve symptoms of RA
•Increased appetite•Weight gain•Water/salt retention•Increased blood pressure•Thinning of skin•Depression•Mood swings•Muscle weakness•Osteoporosis•Delayed wound healing•Onset/worsening of diabetes
•Take medications as directed (adrenal suppression)•Encourage diet high in protein, calcium, potassium and low in sodium and carbohydrates•Discuss body image•Discuss risk for infection
(The Arthritis Society, 2011; Day et al., 2010)
Alternative Medicine
Olive leaf extract
Aloe Vera
Green Tea
Omega 3
Ginger Root Extract
Cats Claw
Omega 3 interferes with blood clotting drugs!
(American College of Rheumatology, 2012)
PainPain is subjective and influenced by multiple factors
HelplessLack of control
Stressful events can increase symptoms of arthritis
(Day et al., 2010; Canadian Psychological Association, 2009)
Consider drugs such as Paxil, Elavil or Zoloft
ExerciseBeing overweight strains joints and leads to further inflammation
(Arthritis Foundation, 2012)
4 times a week for 30 minutes
•Walking•Light jogging•Water aerobics•Cycling•Yoga•Tai chi•stretching
Nutrition
(Johns Hopkins Arthritis Center, 2012)
The most commonly observed vitamin and mineral deficiencies in patients with RA are:o folic acido vitamin Co vitamin Do vitamin B6
o vitamin B12
o vitamin Eo calciumo magnesiumo zinc o selenium
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Spondyloarthritis, Psoriasis and PsASpondyloarthritis, Psoriasis and PsASpondyloarthritis (SpA)
• The prevalence of SpA is comparable to that of RA (0.5–1.9%)1,2
Psoriasis (Pso)
• Psoriasis affects 2% of population
• 7% to 42% of patients with Pso will develop arthritis3
Psoriatic Arthritis
• A chronic and inflammatory arthritis in association with skin psoriasis4
• Usually rheumatoid factor (RF) negative and ACPA negative5
– Distinct from RA
• Psoriatic Arthritis is classified as one of the subtypes of spondyloarthropathies– Characterized by synovitis, enthesitis, dactylitis, spondylitis, skin and nail
psoriasis4
1Rudwaleit M et al. Ann Rheum Dis 2004;63:535-543; 2Braun J et al. Scand J Rheumatol 2005;34:178-90;3 Fitzgerald “Psoriatic Arthritis” in Kelley’s Textbook of Rheumatology, 2009;
4Mease et al. Ann Rheum Dis 2011;70(Suppl 1):i77–i84. doi:10.1136/ard.2010.140582;5Pasquetti et al. Rheumatology 2009;48:315–325
Juvenile SpA
Reactivearthritis
Arthritis associated with
IBD
PsA
UndifferentiatedSpA (uSpA)
Ankylosingspondylitis (AS)
RA: Rheumatoid arthritis
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Psoriatic ArthritisPsoriatic Arthritis
ACR Slide Collection on the Rheumatic Diseases; 3rd edition. 1994.Data on file, Centocor, Inc.
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A Disease of Antiquity:A Disease of Antiquity:Ankylosing Spondylitis Ankylosing Spondylitis
• Amenhotep II (1439-1413 BC)1
• Rameses the Great (1298-1232 BC)1
1Rheumatol Int. 2003; 23:1-5.
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Ankylosing Spondylitis (AS)Ankylosing Spondylitis (AS)• AS is a chronic, progressive immune-mediated
inflammatory disorder that results in ankylosis of the vertebral column and sacroiliac joints1
• The spine and sacroiliac joints are the common affected sites1
– Chronic spinal inflammation (spondylitis) can lead to fusion of vertebrae (ankylosis)1
1 Taurog JD. et al. Harrison‘s Principles of Internal Medicine, 13 th Ed. 1994: 1664-67.
26Ankylosing Ankylosing SpondylitisSpondylitis
“Bamboo Spine”“Bamboo Spine”
Repeated process of healing and bone formation leads to
formation of syndesmophytes ‘bone bridges’
ACR Slide Collection on the Rheumatic Diseases; 3rd edition. 1994.
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Bridging syndesmophytesBridging syndesmophytes
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AS: A Debilitating Rheumatic DiseaseAS: A Debilitating Rheumatic DiseaseOver time, joints in the spine can fuse together and cause a fixed, bent-forward posture
1Linden VD et al. Chapter 10. In: Firestein, Budd, Harris, McInnes, Ruddy and Sergent, eds. Kelley’s Textbook of Rheumatology: Spondyloarthropathies. 8th ed. Saunders Elsevier;2009:p.1171
2 Braun J & Sieper. J Rheumatology 2008;47:1738-40
AS patients have an important impact on health care and non health-care resource utilization, resulting in a mean total cost (direct and productivity) of about $6700 to $9500/year/patient1
More than 30% of patients carry a heavy burden of disease and have a decreased QoL2
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Peripheral manifestations
Enthesitis Peripheral arthritis Dactylitis
AS: Signs and SymptomsAS: Signs and Symptoms
50% patients with enthesitis1
1Cruyssen BV et al. Ann Rheum Dis 2007;66:1072-10772Sidiropoulos PI et al. Rheumatology 2008;47:355-361
Up to 58% patients ever had arthritis1
Much smaller number of patients2
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Why are Dactylitis and Enthesitis Important?
The first abnormality to appear in swollen joints associated with spondyloarthropathies
is an enthesitis2
Likelihood of erosions is higher for digits with dactylitis than
those without1
1Brockbank. Ann Rheum Dis 2005;62:188-90; 2McGonagle et al. The Lancet 1998;352.
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AS: Extra-skeletal Signs and SymptomsAS: Extra-skeletal Signs and SymptomsOther common symptoms seen during the early stages of disease include:
• Anorexia
• Malaise
• Low grade fever
• Weight loss
• Fatigue
1Missaoui B. et al. Ann Readapt Med Phys 2006;49:305-8, 389-391Linden VD et al. Chapter 10. In: Firestein, Budd, Harris, McInnes, Ruddy and Sergent, eds. Kelley’s
Textbook of Rheumatology: Spondyloarthropathies. 8th ed. Saunders Elsevier;2009:p.1176
Fatigue is a frequent complaint of patients with AS1
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AS: AS: Extra-articular Manifestations (EAM)Extra-articular Manifestations (EAM)EAM Prevalence in AS
Patients (%)
Anterior uveitis 30-50
IBD 5-10
Subclinical inflammation of the gut 25-49
Cardiac abnormalitiesConduction disturbancesAortic insufficiency
1-33 1-10
Psoriasis 10-20
Renal abnormalities 10-35
Lung abnormalitiesAirways diseaseInterstitial abnormalitiesEmphysema
40-88 82
47-65 9-35
Bone abnormalitiesOsteoporosisOsteopenia
11-18 39-59
Elewaut D & Matucci MC. Rheumatology 2009;48:1029-1035
Terminal ileitis
Anterior uveitis
Cardiac abnormalities
Psoriatic Arthritis
Examples of Systemic Autoimmunity
Sjogren’s Syndrome
Sjogren’s Syndrome - Investigations
MRI
Typical features of dry eyes, dry mouth and swollen glands
Dryness results in the clinical appearance of keratoconjunctivitis sicca (KCS)
characteristic of Sjogren’s syndrome
Severe Xerostomia with dry tongue
Systemic sclerosis• Mask face• Fish mouth• sclerodactyly
SYSTEMIC SCLEROSISSYSTEMIC SCLEROSISCREST VARIANTCREST VARIANT
CCALCINOSISALCINOSIS
RRAYNAUD’SAYNAUD’S
EESOPHOGEAL DYSFUNCTIONSOPHOGEAL DYSFUNCTION
SSCLERODACTALYCLERODACTALY
TTELANGECTASIAELANGECTASIA
Clinical Features – Raynaud’sClinical Features – Raynaud’s
Almost all (more than 90%) of people with Almost all (more than 90%) of people with scleroderma also have Raynaud's phenomenon. scleroderma also have Raynaud's phenomenon.
Gangguan paruGangguan paru
LUPUS• Penyakit 1000 wajah• Mrs Wolf• Kupu-kupu
Siapa yang dapat mengalami LUPUS
Organ yang dapat dikenai ?
Sistem otot dan tulang Kulit dan rambutMata Ginjal Jantung Pembuluh darah Susunan saraf Paru-paru Komponen darah Hati
Penyebab LUPUS?Penyebab LUPUS?
Faktor Pemicu dari LingkunganFaktor Pemicu dari Lingkungan
• Sinar Ultraviolet • Stres• Obat-obatan • Infeksi
• Bahan kimia• Hormon
Systemic lupus erythematosus: acute facial rash
Acute malar rash
Antiphopspholipid antibody
Blood clotting of antiphospholipid syndrome
Table 1 - Clinical criteria for the diagnosis of APS
Thrombosis VenousArterialSmall vessel (e.g. thrombotic microangiopathy in kidney)
Pregnancy morbidity
≥3 consecutive miscarriages (<10 weeks' gestation)≥1 fetal death (>10 weeks' gestation with normal fetalmorphology)≥1 premature birth (<34 weeks' gestation with normal fetal morphology) due to pre-eclampsia or severe placental insufficiency .
Table 2 - Other recognized features of APSThrombocytopenia Haemolytic anaemia Livedo reticularis Cerebral involvement
Epilepsy, cerebral infarction, chorea and migraine, transverse myelopathy/myelitis mitral valve
Heart valve disease Hypertension Pulmonary hypertension Leg ulcers
About 30-40% of women with SLE have aPL. About 30% of those with aPL have thrombosis. Up to 30% of women with severe early-onset pre-eclampsia may have aPL
Antiphospholipid syndrome. Livedo reticularis
Antiphospholipid syndrome. Arterial thrombosis
Bercak diskoid
Kulit & mukosa
Vaskulitis Rambut rontok
Bercak malar
Peka cahaya
Sariawan berulang
Kulit & mukosa
Radang pembuluh darah
Sumbatan pembuluh darah
Anti –phospholipid syndrome
Livedo reticularis in young woman with APS.
• Livedo reticularis of the upper extremities, which developed as petechiae in the classic lacy, reticular pattern and evolved as a confluent, non blanching, slightly raised purpuric rash in the same reticular pattern.
Livedo reticularis
• Common skin manifestations which may be present with APS include livedo reticularis , purpura and skin ulceration , and skin necrosis.
• Palmar livedo reticularis associated with antiphospholipid antibody syndrome may range from a lacy, flat, reticulated pattern to a more confluent, non -blanching, slightly raised rash (secondary to extravasation of RBCs and plasma).
Patients with APS are often treated with an injectable anticoagulant called heparin. In some cases, the heparin is given into a vein while the patient is in
the hospital. In other cases, heparin is injected under the skin. Or taking a daily small dose of aspirin can help a woman be more fertile