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Hypothyroidism in SLE

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  • 1. UNMASKING SLE
    GRAND ROUNDS
    February 20, 2006
    PCMC Conference Room
    Darla Kay A. de Guzman, MD
  • 2. GOOD MORNING!
  • 3. General Data:
    M.E.C. 18 10/12 y/o Female
    Filipino, Roman Catholic
    Candaba, Pampanga
    Admitted on January 6, 2006
  • 4. Chief Complaint:
    Difficulty of breathing
  • 5. History of the Present Illness
    5 wks PTA bipedal edema
    no other s/sx associated
    no consultation, no meds
    4 wks PTA persistent bipedal edema
    consult w/ MD
    Imp.: Renal problem?
    u/a, abdominal uts: Normal
    Meds: Spironolactone
  • 6. History of the Present Illness
    3 wks PTA ankle, knee pain, weakness and edema
    no fever, cough or colds
    consult same MD
    Chest x-ray: cardiomegaly, 2d-echo: ? results
    referred to PHC
    2 wks PTA seen at PHC: cardiac problem prob sec to Hypothyroidism
    Advised work-up
  • 7. History of the Present Illness
    11 days PTA fever, cough and colds, joint pain and edema
    Ffup at PHC: 2d-echo
    arterial thrombosis
    Meds: Aspirin
    5 days PTA tachypnea, chest pain. Dx: ATP,Hypothyroidism
    Meds: Amoxicillin
  • 8. History of the Present Illness
    3 days PTA 4 pillow orthopnea, DOB, pallor, gen. weakness
    consult at PCMC
    Labs: dec T3, T4, inc TSH
    Advised admx but refused
    1 day PTA above s/sx persisted, severe respiratory distress
    consulted PCMC
    ADMITTED
  • 9. Review of Systems:
    No headache
    No seizure
    No abdominal pain
    No vomiting
    No constipation
    No dysuria
    No hematuria
    No oliguria
    No bleeding
    No heat/cold intolerance
  • 10. Birth and Maternal History
    23 y/o primigravid
    RPNCU at 3 mos AOG c/o LHC
    No maternal illnesses
    Delivered FT, NSD
    At home, by midwife
    No complications
  • 11. Immunization History
    BCG
    DPT x 3 doses
    OPV x 3 doses
    Hepa B x 1 dose
    Measles x 1 dose
    No boosters doses given
  • 12. Nutritional History
    Breastfed up to 1 yrs old
    Complementary foods at 6 mos.
    At present: picky eater
  • 13. Developmental History
    1 mo:regards
    4 mos: head control
    8 mos: sits w/o support
    9 mos: cruises, utters ‘mama’
    1 y/o: walks w/ support
    2 ½ y/o: runs fast
    -At present, has reached first year high school w/ average grades.
  • 14. Past Medical History
    7 y/o: Dx w/ Hypothyroidism
    Maintained w/ L-Thyroxine
    Poor compliance, no follow-ups
    Admitted at 14 y/o: DHF 2
    Discharged after 5 days
    No previous accidents, surgeries
    No allergies to food or drugs
  • 15. Family History
    43
    42
    15
    A maternal uncle was diagnosed of goiter and presently on medication. No family history for diabetes, CVD, hypertension and tuberculosis.
  • 16. Psycho-Social History
    Lives w/ her family in the province.
    Closest to her maternal grandparents
    Stopped schooling after 1st year high school due to financial problems
    Enjoys watching TV and going out w/ cousins. She has no crushes or suitors at present.
    Never tried smoking, drinking liquor or using prohibited drugs
    No suicidal ideations, but is sad for her health condition
  • 17. Physical Examination
    Gen. Survey: awake, pale, in respiratory distress
    Vital Signs: CR: 150/min RR: 50/min T: 38’c BP: 100/60 Wt: 53 kg (p50)
    Ht: 148 cm (p50), BMI: 24.1
    No active dermatoses, diaphoretic
    Anicteric sclerae, pale palpebral conjunctiva, (+) alar flaring, (+) mucoid nasal discharge, no tonsilopharyngeal wall congestion, (+) CLAD, (+) neck vein engorgement
    Equal chest expansion, (+) SC and IC retractions, (+) decreased breath sounds left lung field, (+) rales bilateral lung fields, tactile/vocal fremiti dullness
  • 18. Physical Examination
    Adynamic precordium, tachycardic, (+) muffled, distant heart sounds, no murmurs.
    Globular abdomen, NABS, (+) fluid wave, soft, (+) hepatomegaly, no masses palpated
    Tanner SMR stage 5
    (+) grade 3 bipedal edema, no cyanosis, no clubbing, full and equal pulses, CRT: 2-3 secs.
  • 19. Neurologic Examination:
    Cerebral: awake, conscious, oriented to 3 spheres
    Cranial nerves:
    I: intact
    II: 2-3 mm pupils, ERTL, no papiledema or hemorrhages
    III, IV, VI: intact EOM’s
    V: brisk corneals
    VII: no facial asymmetry
    VIII: intact gross hearing
    IX, X: good gag
    XI: good shoulder shrug
    XII: midline tongue
  • 20. Neurologic Examination
    Motor: good muscle bulk and tone, 5/5 both upper extremities, 4/5 both lower extremities.
    Sensory: intact
    Reflex: +2 all extremities
    No Babinski, no clonus
    Supple neck
    Cerebellar: no nystagmus, good finger-to-nose test
  • 21. Admitting Impression
    Congestive Heart Failure, Cardiac Tamponade probably secondary to Hypothyroidism
    Late Adolescent w/ Psychosocial Issues
    No Wasting, No Stunting
  • 22. Course in the Ward
  • 23. S>DOB, easy fatigability, cough, colds, joint pain.
    First Hospital Day
    O>CBC- n/n anemia, WBC, plt ct, toxic gran
    > CXR: cardiomegaly, pulmonary congestion
    >12 L EKG: sinus tachycardia
    > 2d-echo: severe pericardial effusion, RA, RV MPA collapse
    O>awake
    >febrile, tachycardic, tachypneic
    > pale, neck vein engorgement
    > SC and IC retractions > muffled, distant heart sounds
    > globular abdomen, hepatomegaly
    > grade 3 bipedal edema
  • 24. A>CHF, Cardiac Tamponade prob sec to Hypothyroidism
    First Hospital Day
    > Referred to RICU, Cardio
    • Cefuroxime (50)
    • 25. Dopamine (5)
    • 26. L-Thyroxine
    • 27. “E” Pericardiostomy: 438 cc pericardial fluid
    P>O2 support
    > venoclysis
    > NPO
    >PRBC transfusion
  • 28. 2nd Hospital Day
    S>(+) episodes of DOB
    (+)weakness
    (+) cough
    (+) joint pain
    2nd Hospital Day
    A> CHF w/ Cardiac Tamponade prob sec to Hypothyroidism s/p Pericardiostomy tube Insertion
    Bacterial Pericarditis
    O> awake, tachypneic, tachycardic, febrile, adequate U/O, normotensive
    • 2d-echo: decrease in pericardial effusion, pleural effusion
    • 29. Pericardial fluid analysis: 65% PMNs, (+) g+ cocci, LDH , (-) AFB
    • 30. ABGs- normal
    • 31. Blood c/s- no growth
    • 32. Pericardial fluid drained: 410cc
    P> Referred to PIDS
    > Oxacillin started
    > Dopamine
    > L-thyroxine
  • 33. S> (+) afebrile seizure
    (-) DOB
    (+) fair intake
    3rd-4th Hospital Day
    A> Pleural Effusion sec to Myxedematous Pericardial Effusion sec to Hypothyroidism r/o Parapneumonic Effusion s/p pericardiostomy
    Seizure secondary to Electrolyte Imbalance
    O> (+) fever
    > (+) rales, RLF
    > distinct heart sounds
    > Normal neuro exam’n
    > urine KOH- negative
    > U/A: pyuria
    > S. elec: K, Ca
    P>Feeding resumed
    > Kalium Durule
    > Ca gluconate
    > Antibiotics
    > Referred to Pulmo
    > UTZ guided Thoracentesis
  • 34. S> recurrence of afebrile seizure, same character
    > joint pains
    > fair intake
    5th-6th Hospital Day
    A> Pleural Effusion sec to Myxedematous Pericardial Effusion sec to Hypothyroidism r/o Parapneumonic Effusion s/p Pericardiostomy
    Seizure sec to Electrolyte Imbalance
    O> awake, (+) fever
    > tachypnea
    > tachycardia
    > (+) rales BLF
    • Normal Neuro exam’n
    • 35. S. electrolytes: Ca
    • 36. CBC- normal
    • 37. Chest UTS- no fluid R hemithorax, minimal fluid L
    P> Midazolam IV
    > Calcium gluconate
    > Oxacillin
    > L-Thyroxine
    > Amikacin
    > Thoracentesis- deferred
  • 38. S> no seizure recurrence
    > no DOB
    > fair intake
    > joint pain
    8th Hospital Day
    A> Pleural Effusion 2 to Myxedematous Pericardial Effusion sec to Hypothyroidism r/o Parapneumonic Effusion
    Seizure sec to Electrolyte Imbalance, resolved
    t/c SLE
    S > low to mod grade fever
    > tachypneic, tachycardic
    > painless, oral mucosal lesions
    > CXR: haziness R, consolidation vs pleural thickening
    P> Trans out to 121
    • Antibiotics continued
  • S> no seizure recurrence
    > no DOB
    > cough
    > poor intake
    10th-11th Hospital Day
    A> Myxedematous Pericardial Effusion s/p Pericardiostomy w/ Pleural Effusion prob sec to Hypothyroidism Sepsis Unspecified
    O> moderate to high grade fever
    > tachypneic, tachycardic
    > (+) rales BLF, distinct heart sounds
    > chest uts: bilat. Pleural fibroses, min pleural & pericardial effusion L.
    P> Oxacillin continued
    > Cefotaxime started
    > Amikacin shifted to Gentamicin
  • 39. S> no seizure
    > no DOB
    > poor intake
    12th-15th Hospital Day
    A> SLE
    Hypothyroidism
    Nosocomial sepsis
    O>mod-high grade fever
    > tachycardic, tachypneic, normotensive
    > oliguria, hepatomegaly
    > 2d-echo: pericardial effusion
    > ESR: 120 mm/hr
    > s. Albumin: decreased
    > s. Creatinine: normal
    P> Increased fluid intake encouraged
    > for ANA, anti-DS DNA
  • 40. S> DOB
    > poor intake
    > weak
    17th-19th Hospital Day
    O> CXR: cardiomegaly, pulm congestion, bilat pleural effusion
    > Abd’l uts: hepatomegaly w/ parenchymal changes
    > U/A: pyuria, (+) FGC
    > sputum AFB: (-)
    > Urine C/S: (-)
    > ABGs- normal
    O> high grade fever
    > oliguric, normotensive
    > tachypneic, tachycardic
    > dry lips, and skin
    > jaundice, icteric
    sclerae, petechial rash
    > (+) rales BLF
    > s. electrolytes: Na, K
    > CBC: n/n anemia
    >Reticulocyte ct:
    > RBS, AST, ALT:
  • 41. 17th-19th Hospital Day
    A> SLE w/ Pericardial Effusion
    Hypothyroidism
    Pre-Renal failure Sec to Dehydration Sec to Poor Intake
    Nosocomial Sepsis
    P>Co-managed w/ Nephro
    > Fluid Resuscitation
    > PRBC transfusion
    > Ceftazidime started
    > Gentamicin cont.
  • 42. S> (+) DOB
    (+) irritable
    (+) weakness
    20th Hospital Day
    A> CHF, Pericardial Effusion sec to SLE
    Nosocomial Sepsis
    O> high grade fever
    > tachypneic, tachycardic
    > adequate U/O
    > (+) distant heart sounds
    > hepatomegaly
    > Chest uts: min pleural effusion
    > T4: normal
    > CBC: leukopenia
    > ANA: +4
    > Anti-DS DNA : (+)
    P> “E” pericardiocentesis
    > Dopamine, Dobutamine
    > Fluids at 75% BSA
    > Antibiotics continued
  • 43. S> (+) DOB
    (+) weakness
    (+) poor intake
    21st-23rd Hospital Day
    O> SLE w/ Pericardial Effusion
    Hypothyroidism
    Nosocomial Sepsis
    O> mod grade fever
    > tachypneic, tachycardic
    > jaundice, petechial rash
    > rales BLF, distant heart sounds
    > hepatomegaly
    P> Methylprednisolone pulse therapy, x 3 doses
  • 44. S> no DOB
    > improved appetite
    > better well-being
    25th Hospital Day
    A> SLE
    Hypothyroidism
    Nosocomial sepsis
    O> afebrile
    > stable vital signs
    > jaundice resolved
    > fine rales BLF
    > distinct heart sounds
    > Serum bilirubin, CBC: normal
    P> seen by ophthalmology svc
    >CBC
    > s. bilirubin
    > 2D-echo, CXR
  • 45. Final Diagnosis
    Systemic Lupus Erythematosus With Serositis (Pericardial Effusion, Severe, s/p Pericardiocentesis, Pericardiostomy, Pleural Effusion, Bilateral)
    Congestive Heart Failure, Resolved
    Hypothyroidism
    Nosocomial Sepsis, resolved
    Late Adolescent w/ Psychosocial Issues
    No Wasting, No Stunting
  • 46. 18 y/o F
    -bilateral edema
    -edema
    - joint pain
    - weakness
    - cardiomegaly
    - fever -edema
    - cough/colds – joint pain
    5 wks PTA
    3-4 wks PTA
    11 days PTA
  • 47. 18 y/o, F
    - above s/sx
    - chest pain
    - tachypnea
    - orthopnea
    -pallor
    - weakness
    -respiratory distress
    5 days PTA
    3 days PTA
    Day of Admission
  • 48. Past Medical History
    Dx w/ Hypothyroidism at 7y/o
    Poor medical compliance
    No follow-ups
  • 49. Pertinent PE Findings
    Pale, RD
    Alar flaring, neck vein engorgement
    SC, IC retractions, rales BLF, decreased BS left
    Tachycardic, muffled and distant heart sounds
    Globular abdomen, (+) fluid wave, hepatomegaly
    Grade 3 bipedal edema
  • 50. Difficulty of Breathing
    Unlikely Causes
    CNS
    Trauma/Injury
    Most Likely Causes
    Respiratory
    Cardiovascular
    Metabolic
  • 51.
  • 52. Pericardial disease
    Pericarditis
    Pericardial Effusion
    Cardiac Tamponade
  • 53. Cardiac
    Tamponade
    CARDIAC
    ARF/ RHD
    METABOLIC
    HYPOTHYROIDISM
    RENAL
    UREMIA
    INFECTIOUS
    TB, VIRAL
    PULMO
    PNEUMONIA
    CONNECTIVE TISSUE DSES
    JRA SLE
  • 54. Differential Diagnoses
    Rheumatic Fever
    Viral Pericarditis
    Tuberculosis
    Pneumonia
    Hypothyroidism
    SLE
  • 55. Differential Diagnoses
    Acute Rheumatic Fever
  • Differential Diagnoses
    Viral Pericarditis
    - Rarely progresses to cardiac tamponade
  • 61. Differential Diagnoses
    Tuberculosis
    - no exposure
  • 64. Differential Diagnoses
    Pneumonia
  • Differential Diagnoses
    Hypothyroidism
    • (+) history
    - rarely cardiac tamponade
  • 67. Differential Diagnoses
    SLE/ JRA
  • Course in the Ward
  • 71. Day of Admx-Days 1-2
    - pale
    • fever
    • 72. generalized weakness
    • 73. joint pain
    • 74. respiratory distress
    • 75. tachypneic, tachycardic
    • 76. N/N anemia
    • 77. cardiomegaly, pulm congestion
    • 78. pericardial effusion, severe
  • Days 3-8:
    • painless, oral lesions
    • 79. fever
    • 80. afebrile seizure
    • 81. Pericardial fluid analysis
    - (-) AFB
    • Pleural effusion
  • Days 12-15
    - petechial rashes
  • Day 17-20
    • jaundice, icteric sclerae
    • 85. hepatomegaly
    • 86. shortness of breath
    • 87. cardiomegaly, pulm congestion, bilat pleural effusion
    • 88. N/N anemia
    • 89. Inc. LFTs
    • 90. T4- normal
    • 91. ANA: +4
    • 92. Anti- DS DNA: +
  • Day 21-23
    • decreasing jaundice
    • 93. Lysis of fever
    • 94. Improvement of appetite and well-being
    • 95. Methylprednisolone 3 doses given
    IMPROVEMENT
  • 96. :non-constitutional
    signs and symptoms
  • 97. :non-constitutional
    signs and symptoms
  • 102. :non-constitutional
    signs and symptoms
    Laboratory parameters
  • 107. :non-constitutional
    signs and symptoms
    Laboratory parameters
    SYSTEMIC LUPUS ERYTHEMATOSUS
  • 112. The 1982 Revised Criteria for Classification of Systemic Lupus Erythematosus
    Malar rash
    Discoid rash
    Photosensitivity
    Oral ulcers
    Arthritis
    Serositis
    Renal disorder
    Neurologic disorder
    Hematologic disorder
    Immunologic disorder
    Antinuclear antibody
  • 113. Systemic Lupus Erythematosus
  • 114. FREQUENCIES & CLIN FEATURES OF SLE AT DIAGNOSIS & AT ANY TIME AMONG CHILDREN AND ADOLESCENTS
  • 115. FREQUENCIES & CLIN FEATURES OF SLE AT DIAGNOSIS & AT ANY TIME AMONG CHILDREN AND ADOLESCENTS
  • 116. PCMC
  • Arthritis
    Persistent knee and ankle pain
    Non-erosive, non-deforming
    2 or more peripheral joints
    Tenderness, swelling, effusion
    Anti-inflammatory meds.
  • 122. Mucocutaneous Involvement
    Beseler, Silvermann
    Pediatr Clin N Am
    52 (2005) 443-467
  • 123. Mucocutaneous Involvement
    Petechial rash, ecchymoses
    not as frequent
    r/o other lesions of platelet count abnormalities
    aggravated by sun exposure
    ‘..definite photosensitivity occurs in 16% of children…’
    Downing, Mesina
    N Engl J Med
    1992; 227:408-409
  • 124. Mucocutaneous Involvement
    ‘rashes occur frequently in children w/ SLE but only 30-50% ever manifest the typical butterfly rash.’
    Glidden, Mantzouranis, Borel
    Clin Immunol Immunopathol
    1983; 29;196-210
  • 125. Hematologic Involvement
    Pallor; normocytic/ normochromic anemia
    ‘ …the most common hematologic manifestation of SLE in children and adolescents is anemia…’
    Wallace, Hahn2002
  • 126. Hematologic Involvement
    Anemia, thrombocytopenia, leukopenia: 50-75%
    normocytic normochromic anemia
    microcytic hypochromic
    Coagulation abnormalities
    Menorrhagia
  • 127. Cardiac Involvement
    Pericardial effusion cardiac tamponade
    ‘… Cardiac manifestations rarely are prominent in children and adolescents w/ SLE, but occasionally they are catastrophic…’
    Auito, Stanbouly, Boxer
    Clin Pediatr 1993;32:566567
  • 128. Cardiac Involvement
    Common Cardiac Pathology
    - Pericarditis
    - Myocarditis
    - Mild Valvular Involvement
    ‘…The most common form of cardiac involvement is pericarditis w/ pericardial effusion…’
    Chan, Li, Tam
    Scand J Rheumatol 2003;32:306-308
  • 129. Cardiac Involvement
    • Many children w/ SLE are anemic and develop few murmurs. Libman-sacks endocarditis may occur in childhood however, and this predisposes to bacterial endocarditis..
    Wallace, Hahn 2002
  • 130. Pulmonary Involvement
    Pleural effusion, bilateral
    ‘… Pleurisy and pleural effusion are the most common pulmonary manifestations…’
    Delgado, Mulleson, Pine
    Semin Arthr Rheum
    1990; 29: 225-293
  • 131. Pulmonary Involvement
    Other Pulmonary complications:
    - pneumothorax
    - pneumonia
    - chronic restrictive lung dse.
    - pulmonary HPN
    - acute pulmonary hemorrhage
    Most fatal complication in children and adolescents: pneumonia
  • 132. Pulmonary Involvement
    ‘…Pneumonia was the cause of death for 9 of 26 children w/ SLE coming to autopsy…pulmonary hemorrhage contributed to death of 5 others…’
    Nadora, Landing
    Pediatr Pathol 1987: 7;118
  • 133. GIT Manifestations
    Jaundice, hepatomegaly, abnormal LFT’s
    Other s/sx:
    - abdominal pain, anorexia, weight loss
    Often resolve w/ corticosteroid therapy
  • 134. Infections in SLE
    Patient developed nosocomial sepsis due to her immunocompromosed state
    Major cause of mortality and morbidity
    Plat et al: 55 separate infections in 70 patients, over 9 years.
    >Results from:
    1. combined effects of SLE
    2. drugs used to mediate it
  • 135. Infections in SLE
    ‘…The frequency of infection increases w/ increasing steroid dosage…’
    Guizler, Deamond, Kaplan
    Arthr Rheum 1978; 21:37-44
    Careful use and reduced dosage of corticosteroids decreases frequency of infections
  • 136. Laboratory Evaluation
    ANA
    Anti- DS DNA
    Anti- Smith Ab
    Autoantibodies
    Hypergammaglobulinemia
  • 137. Endocrine Involvement
    Thyroid
    Hypothyroidism>Hyperthyroidism
    ‘…Up to 35% of SLE patients have anti-thyroid antibodies, w/ 10-15% of patients developing overt hypothyroidism…’
    Eberhard, Laxer, Eddy
    J Pediatr 1991;119:277-9
  • 138. Treatment
    Depends on target organs and disease severity
    Pericardiostomy tube insertion, antibiotic coverage, immunosuppresive therapy
  • 139. IMMUNOSUPPRESSIVE TREATMENT OF CHILDHOOD SLE
  • 140. Cornerstone of Treatment of Children w/ Rheumatic Disease
    Accurate diagnosis and education of family
    Medications
    Physical medicine and rehabilitation
    Physical and psychosocial growth and development
    Coordination of care
  • 141. Chronic Disease in Adolescents: Issues Involved
    1.Compromised mental health may make management of the physical illness more difficult.
    - increased risk for psychiatric disorders and social adjustment problems.
    - non-compliance w/ medical treatment.
    2. Risk taking in the areas of sexuality and substance use is an important contributor to morbidity.
    - management of high-risk pregnancies, w/c demands a high degree of compliance and medical regimentation.
    3. Functional impairments increase the risk of academic and adjustment problems.
  • 142. Chronic Disease in Adolescents:Trends in Management
    • Needs a biopsychosocial, multidisciplinary approach
    • 143. Improved nutritional management
    better growth and development
    normative social behavior , including risk-taking behaviors
    • Assistance in becoming functioning adults: employment, sexuality and marriage
    • 144. Programs to help them transition from pediatric into adult-oriented health care.
  • Incidence of Adverse Outcomes in 72 Children w/ SLE
    OUTCOME
    renal failure
    severe CNS dse.
    stroke
    chronic thrombocytopenia
    chronic active dse
    death
    INCIDENCE (%)
    15
    11
    1
    7
    56
    18
  • 145. Predictors of Poor Prognosis in SLE
    Persistent anemia: Hb <10g for 6 mos.
    Persistent hypertension: diastolic BP>90mmHg for >6 mos.
    Persistent hematuria: >20 rbc/HPF for > 6 mos.
    Pulmonary hypertension
    Recurrent emergency admissions.
  • 146. CONCLUSION
  • 147. Thank you!