berkenalan dengan ragam penyakit autoimun

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