1. Laboratory examination in Immune disorders
Umi S. Intansari
Bag. Patologi klinik FK UGM/ RSUP Dr. Sardjito
Yogyakarta

2. Autoimmune diseases

3. c c c c c c c c
Mackay IR, 2000, BMJ 321: 93-96

4. A critical function of the immune system is to discriminate self from non-self

5. Requirement for autoimmunity
Infection and environmental exposure
Genetic factors
Breaking tolerance
Autoimmunity

6. Cluster of autoimmune diseases

7. Faktor genetik DM tipe 1
HLA DR3/4 increase susceptibility of disease in type 1 DM
Autoimmunity is more common in female
Shared HLA in sibling increase susceptibility of disease in type 1 DM

8. Prevalence of autoimmune diseases
Rheumatoid arthritis: 1% of general population, but female excess
SLE: 0.12% of general population, but female excess
Myasthenia gravis: 0.0% of general population, but female excess
Multiple sclerosis: 0.1% of general population but female excess
Primary Sjogren Syndrome: 0.6-3% of adult women
Thyroid disease: >3% of adult women
Type 1 diabetes: 0.1% of children
Mackay IR, 2000, BMJ 321: 93-96

9. Deteksi autoantibodi
Ada bermacam-macam antibodi terhadap antigen intraseluler dan nuklear
Deteksi dapat dilakukan secara kualitatif, kuantitatif dan dilanjutkan identifikasi spesifikasi imunologisnya
Secara umum dikarakterisasi oleh;
Ada tidaknya antibodi spesifik
Perbedaan level kuantitatif/titer dari autoantibodi

10. Revised ARA Criteria for the Classification of Rheumatoid Arthritis (RA)
For classification purposes, a patient is said to have RA if he or she has satisfied at least 4 of the following 7 criteria. Criteria 1 through 4 must have been present for at least 6 weeks. Patients with 2 clinical diagnoses are not excluded. Designation as classic, definite, or probable RA is not to be made.
Morning stiffness: Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement.
Arthritis of 3 or more joint areas: At least 3 joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician; the 14 possible joint areas are right or left proximal interphalangeal (PIP) joints, metacarpophalangeal (MCP) joints, wrist, elbow, knee, ankle, and metatarsophalangeal (MPT) joints.
Arthritis of hand joints: At least I area swollen (as defined above) in a wrist, MCP or PIP joint.
Symmetric arthritis: Simultaneous involvement of the same joint areas (see 2 above) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry).
Rheumatoid nodules: Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician.
Serum rheumatoid factor: Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in <5% of normal control subjects.
Radiographic changes: Radiographic changes typical of RA on posteroanterior hand and wrist radiographs, which must include erosions or unequivocal bony decalcification localized to or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify).

11. Systemic Lupus Erythematosus (SLE)
Autoantibody-mediated inflamation can lead to release of autoantigens from damaged tissue and promotes further activation o f autoreactive B cells
Chronic stage

12. Classification Criteria for the Diagnosis of Systemic Lupus Erythematosus (SLE)
Malar rash: Fixed erythema, flat or raised, over the malar eminences
Discoid rash: Erythematous circular raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur
Photosensitivity: Exposure to ultraviolet light causes rash
Oral ulcers: Includes oral and nasopharyngeal ulcers, observed by physician
Arthritis: Nonerosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion
Serositis: Pleuritis or pericarditis documented by ECG or rub or evidence of effusion
Renal disorder: Proteinuria >0.5 g/d or 3+, or cellular casts
Neurologic disorder: Seizures or psychosis without other causes
Hematologic disorder: Hemolytic anemia or leukopenia (<4000/L) or lymphopenia (<1500/L) or thrombocytopenia (<100,000/L) in the absence of offending drugs
Immunologic disorder: Anti-dsDNA, anti-Sm, and/or anti-phospholipid
Antinuclear antibodies: An abnormal titer of ANA by immunofluorescence or an equivalent assay at any point in time in the absence of drugs known to induce ANAs
Any combination of 4 or more of 11 criteria, well-documented at any time during a patient's history, makes it likely that the patient has SLE (specificity and sensitivity are 95% and 75%, respectively).
Note: ECG, electrocardiography; dsDNA, double-stranded DNA; ANA, antinuclear antibodies.

13. Lupus eritematosus sistemik (SLE)
Pem urin kuantitatif
Urin rutin
The American College of Rheumatology ( ACR ) established 11 criteria in 1982, which were revised in 1997

14. Darah lengkap, urin rutin, Comb’s test,
Morfologi Darah Tepi
Retikulosit, LDH, bilirubin
Sel LE, Anti Sm,
anti Ds DNA, VDRL, TPHA
ANA tes
The American College of Rheumatology ( ACR ) established 11 criteria in 1982, which were revised in 1997

15. Immunofluorescence microscope
Indirect immunofluorescence of ANA (homogenous pattern
Courtesy: Stanford Clinical chemistry web
Fluorescence imaging of renal biopsy of lupus nephritis patient (SLE
Courtesy: NUS home page

16. Autoimmune Haemolytic Anemia (AIHA)

17. Autoimmune Haemolytic Anemia (AIHA)

18. Anti immunoglobulin antibodies18

19. Coombs tests and the detection of Rh incompatibility
Use anti-immunogobulin antibodies (Coombs reagent) to detect antibodies that cause disease
Direct : directly detect Ab bound to the surface of fetal red blood cells
Indirect: detect nonagglutinating anti-Rh Ab in maternal serum 19

20. Coombs tests and the detection of Rh incompatibility20

21. Figure A-13 21

22. Allergic diseases

23. Alergi dan hipersensitivitas definisi
Hipersensitivitas adalah reaksi imun terhadap antigen lingkungan yang sebenarnya tidak berbahaya yang mengakibatkan kerusakan jaringan dan/ atau penyakit
Alergi adalah salah satu dari reaksi hipersensitivitas: reaksi hipersensitivitas tipe 1 (type 1 hypersensitivity atau immediate-type hypersensitivity reactions)

24. Contoh – contoh Reaksi Alergi

25. Lab Examination

26. Ke-4 Jenis Reaksi Hipersensitivitas
Figure 12-2
alergi

27. Alergi
Reaksi alergi terjadi bila individu yang sudah mempunyai antibodi IgE terhadap suatu antigen (alergen) tertentu (SUDAH TERSENSITISASI) terpapar pada alergen yang sama
Antigen yang mengakibatkan alergi disebut alergen
Alergen memicu aktivasi IgE yang terikat sel mast, mengakibatkan suatu rentetan kejadian yang disebut reaksi alergi

28. sensitisasi
reaksi alergi

29. Immunodeficiency

30. Figure 11-20

31. Figure 11-29

32. Distribution of lymphocyte subpopulation in human peripheral blood
Figure A-24 32

33. Subset limfosit
B CD19+
T-helper
CD3+ CD4+
T-cyt.
CD3+ CD8+ 33

34. 34