RHEUMATOLOGIC DISORDERS RHEUMATOLOGIC DISORDERS.ppt

RHEUMATOLOGIC DISORDERS

1. Connective Tissue
DISORDERS
BY DR Sakeena Nour Eldine

2. RHEUMATIC FEVER
AN INFLAMMATORY DISEASE THAT
FOLLOWS INFECTION WITH GROUP A
STREPTOCOCCI.
IT MAY AFFECT :
THE HEART
THE JOINTS
CNS
S/C TISSUES

3. RF OCCURS IN 3% OF PTS WHO
CARRY AN INFECTING STRAIN FOR
MORE THAN 3 WEEKS AFTER
CONVALESCENCE.
INDIVIDUALS WHO HAVE ASO TITRE
>250 AFTER STREPTOCOCCAL
INFECTION, HAVE A 5% INCIDENCE OF
RF

4. PTS WITH STREPTOCOCCAL
INFECTION AND HISTORY OF
PREVIOUS RF, HAVE INCIDENCE 5%-
50% GREATER THAN PTS WITHOUT
HIST OF RF.

5. FACTORS AFFECTING THE
INCIDENCE OF RF:
1.ENVIROMENTAL
2.ECONOMIC
3.AGE

6. PTHOLOGIC CHANGES AFFECT ALL
THE BODY , PRIMARILY IN
CONNECTIVE TISSUE AND AROUND
SMALL VESSELS.
THE PATHOGNOMONIC LESION IS THE
ASCHOFF BODY.
ENDOCARDITIS (VALVITIS)

7. VALVES AFFECTED BY ORDER OF
FREQUENCY:
•MITRAL
•AORTIC
•TRICUSPID
•PULMONARY (RARE)

8. JOINTS:
IN CONTRAST TO CARDIAC LESIONS,
PATHOLOGIC CHANGES IN THE
JOINTS ARE COMPLETELY
REVERSIBLE

10. SKIN: S/C NODULES AND ERYTHEMA
MARGINATUM
CNS : CHANGES IN CHOREA NOT
CONSISTANT.

12. CLINICAL FEATURES
MAJOR MANIFESTATIONS:
1.CARDITIS
2.POLYARTHRITIS
3.CHOREA
4.ERYTHEMA MARGINATUM
5.S/C NODULES

13. MINOR MANIFESTATIONS:
CLINICAL:
ARTHRALGIA
FEVER
LAB:
ACUTE PHASE REACTANTS
ESR, C-REACTIVE PROTEINS
ECG :
PROLONGED P-R INTERVAL

14. DIFF DIAGNOSIS
OTHER CONNECTIVE TISSUE
DISORDERS
RHEUMATOID ARTHRITIS, SLE
SICKLE CELL DISEASE

15. LAB EVALUATION
ESR, C-REACTIVE PROTEINS
WBC COUNT
URINE ANALYSIS
THROAT SWAB
ANTIBODIES e.g. ASO, antiDNase B, AH
and ASK.

16. TREATMENT
PRCAINE PENICILLIN FOR 10 DAYS OR A SINGLE
DOSE OF BENZATHINE PENICILLIN
ERYTHROMYCIN FOR 10 DAYS
SALYCILATES FOR FEVER AND ARTHRITIS
STEROIDS FOR CARDIAC INVOLVEMENT (carditis)
DIGITALIS FOR HF
SURGERY
PHENOBARBITONE OR HALOPERIDOL (CHOREA)

17. PROPHYLAXIS
BNZATHINE PENICILLIN.
PHENOXYMETHYL PENICILLIN.
DURATION ?

18. Other Connective Tissue
Disorders

19. INTRODUCTION
REUMATOLOGIC DISEASES ARE
MEDIATED BY INFLAMATORY
PROCESSES OF UNKNOWN AETIOLOGY
ASSOCIATED WITH FORMATION OF
IMMUNE COMPLEXES WHICH CAUSE
TISSUE DAMAGE AFTER DEPOSITION IN
TISSUE OR BLOOD VESSELS

20. THESE IMMUNE COMPLEXES ATTRACT
NEUTROPHILS THROUGH COMPLEMENT
ACTIVATION.
NEUTROPHILS MEDIATE TISSUE
DAMAGE BY RELEASE OF
DEGENERATIVE ENZYMES e.g.
HYDROLASES, PROTEASES, ELASTASES,
COLLAGENASES etc

21. JUVENILE RHEUMATOID
ARTHRITIS
5 MAJOR FORMS OF JRA:
1.SYSTEMIC JRA (STILL DISEASE)
2.Oligo(PAUCI)ARTICULAR JRA
3.POLYARTICULAR JRA
4.SPONDYLOARTHROPATHIES
5.POLYARTICULAR JRA (RF POSITIVE)

22. JRA IS CHARACTERIZED BY THE
PERSISTENCE OF OBJECTIVE
INFLAMMATORY FINDINGS IN ONE OR
MORE JOINTS FOR AT LEAST 6
WEEKS IN A CHILD 16 YEARS OF AGE
OR YOUNGER.

23. AETIOLOGY
NO AB SPECIFIC FOR JRA HAS BEEN
ISOLATED
DESPITE YEARS OF INTENSIVE RESEARCH ,
NO SPECIFIC INFECTIOUS AGENT HAS
BEEN ISOLATED AS A CAUSE OF JRA (EBV)
PROBABLY IT IS AN IMMUNE RESPONSE TO
UNDEFINED ANTIGENS IN GENETICALLY
SUSCEPTIBLE HOSTS.

24. CLINICAL FEATURES
JRA HAS TWO PEAKS OF ONSET:
1-3 YEARS AND 8-12 YEARS
CLINICAL FEATURES DIFFER IN THE
DIFFERENT SUBTYPES.

25. SYSTEMIC JRA
LEAST COMMON (10%)
AFFECTS BOTH SEXES EQUALLY.
ALL AGE GROUPS
PERSISTENT HIGH SPIKING FEVER AND
RASH. SPIKES IN LATE AFTERNOON.
RASH MORE PROMINENT ON THE TRUNK
AND INNER ASPECTS OF THE THIGH.

26. ANOREXIA
ARTHRALGIA AND/OR MYALGIA.
IRRITABILITY
GENERALIZED LYPHADENOPATHY.
HEPATOSPLENOMEGALLY

27. LAB. FINDINGS
RF –VE
ANA –VE
PROFOUND ANEMIA
MARKED LEUKOCYTOSIS
THROMBOCYTOSIS
PERICARDITIS OR MYOCARDITIS
PLEURAL EFFUSION OR PNEUMONITIS
PICTURE OF ACUTE ABDOMEN

28. JOINTS FINDINGS APPEAR MONTHS
AFTER THESE SYSTEMIC
MANIFESTATION
THE SYSTEMIC SYMPTOMS
DISAPPEAR GRADUALLY WHILE JOINT
DISEASE CONTINUE

29. OLIGOARTICULAR JRA
MOST COMMON FORM OF JRA (50%)
USUALLY PRESENT WITH INVOLVEMENT
OF A SINGLE JOINT BUT MAY HAVE
INVOLVEMENT OF UP TO FOUR JOINTS
IN THE SUBSEQUENT 6 MONTHS.
INVOLVEMENT OF JOINTS BY ORDER OF
FREQUENCY:

30. KNEE
ANKLE
WRIST
ELBOW
TYPICALLY THIS FORM OF JRA AFFECTS
VERY YOUNG GIRLS (TODDLERS)
EYE INVOLVEMENT (CHRONIC UVEITIS)

31. LAB:
RF –VE
ANA +VE (FREQUENTLY)

32. PROGNOSIS IS GENERALY GOOD.
SMALL PROPORTION USUALLY BOYS
ABOUT 9 YRS OLD, HAVE DIFFERENT
CLINICAL PICTURE AND PROGNOSIS.
HIP ,KNEE, ANKLE AND MTP JOINT.
ENTHESITIS
ACUTE EYE INVOLVEMENT (UVEITIS)
PROGNOSIS:> ANKYLOSING SPONDYLITIS
LAB: RF AND ANA MAY BE –VE.

33. POLYARTICULAR JRA
INVOLVEMENT OF 5 OR MORE JOINTS
IN THE FIRST 6 MONTHS OF ILLNESS
SYMMETRIC INVOLVEMENT OF THE
SMALL JOINTS OF THE HANDS AND
FEET AND VARIOUS LARGE JOINTS.
SOME ARE RF +VE.

36. TREATMENT
MULTIDISCIPLINARY TEAM.
(PHYSIOTHERAPY ,PSYCHOTHERAPY)

DRUGS:
1- NSAIDs
2- STEROID HAVE VERY LIMITED ROLE
ONLY IN SYSTEMIC JRA.

37. SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE)
SLE IS AN AUTOIMMUNE DISORDER
AFFECTING MULTIPLE TISSUES AND
ORGANS.
RESULTS FROM GENERATION OF
DIVERSE AUTOANTIBODIES AGAINST
SUBCELLULAR COMPONENTS
INCLUDING DNA, RNA

38. CLINICAL FEATURES
ANY AGE
VAGUE SYMPTOMS ( FEVER , FATIGUE, WT
LOSS)
TWO MOST COMMON PRESNTING
FEATURES IN CHILDREN ARE SKIN RASH
AND ARTHRITIS.
MOST IMP CONSEQUENCE IS THE RENAL
INVOLVEMENT.

39. ALOPECIA
ARTHRITIS
RAYNAUD PHENOMENON
CNS MANIFESTATION
THROMBOTIC PHENOMENON
PLEURITIS (PULMONARY HAGE)
PERICARDITIS AND MYOCARDITIS

41. GIT: RECURRENT ABD PAIN,
HEPATOMEGALLY, ASCITIS, ULCERS
AND PANCREATITIS
MOST FEATURES BEGIN AFTER
PUBERTY.

42. INCIDENCE IS 6/1000000
FEMALE:MALE RATIO IS 4:1 IN
CHILDREN < 9
10:1 IN CHILDREN >9

43. DIAGNOSIS
DEPENDS ON THE PRESENCE OF 4 OR
MORE MAJOR CRITERIA
1.MALAR RASH
2.DISCOID RASH
3.PHOTOSENSITIVITY
4.ORAL ULCERS
5.ARTHRITIS

44. 6. RENAL DISORDER
7. SEROSITIS
8. NEUROLOGIC DISORDER
9. HEMOLYTIC DISORDER
10. IMMUNOLOGIC DISORDER
11. ANA

45. LAB
HEMATOLOGY:
•COOMBS TEST
•PLATELET COUNT
•WBC COUNT
•ESR ( ACTIVITY OF THE DISEASE)
•C3, C4 ( ACTIVITY)

46. IMMUNOLOGY:
•ANA > 95% OF SLE PTS
•ANTI-Sm ANTIBODIES ( MOST
SPECIFIC)
•ANTI-DNA

47. RENAL FUNCTIONS
•SERUM ELECTROLYTES
•BLOOD UREA
•CREATININE
•URINE ANALYSIS
•RENAL BIOPSY

48. TREATMENT
Steroid therapy:
To continue until ESR and antibody titer falls and
complement level rise.
•When unable to wean from steroids, second line
drugs can be used e.g. azathioprine,
cyclosporine and hydroxychloroquine
Ophthalmologic exam.
Physical therapy.