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APPROACH TO PERIODIC FEVER SYNDROMES

APPROACH TO PERIODIC FEVER SYNDROMES. Sarah S. Long, M.D. Professor of Pediatrics Drexel University College of Medicine Chief, Section of Infectious Diseases St. Christopher’s Hospital for Children Dr. Long has no conflict to disclose. Case. A 15-year-old girl is referred because of a

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APPROACH TO PERIODIC FEVER SYNDROMES

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  1. APPROACH TO PERIODIC FEVER SYNDROMES Sarah S. Long, M.D. Professor of Pediatrics Drexel University College of Medicine Chief, Section of Infectious Diseases St. Christopher’s Hospital for Children Dr. Long has no conflict to disclose

  2. Case A 15-year-old girl is referred because of a persistent febrile illness. Twelve weeks ago she had the acute onset of fever to 1030, headache, myalgia, sore throat and nasal congestion. A throat culture was negative for group A streptococcus. Over the next week high fever and congestion abated but parents report that she has had persisting low grade fever (to 99.8 every 2-3 days), complains of headache, sore throat, weakness, fatigue, and has poor color, poor appetite. She complains of sudden shooting pains in arms and legs.

  3. Case continued On attempt to return to school 4 weeks ago she was there only 2 hours when the school nurse reported that the patient felt faint, chilly and sweaty, and was unable to stay in class. She has not returned to school and is now home tutored. What is your next diagnostic step?

  4. APPROACH TO DX PERSISTING SYMPTOMS I Past Hx: No significant medical “A” student Swim team Church choir Local volunteer Current Hx: Temps Peaks 4-8 PM, <1000 Relieved acetaminophen Does not awaken No vomiting Headache

  5. APPROACH TO DX PERSISTING SYMPTOMS II Current Hx: Sore throat Prominent AM only Not worse swallowing Weakness Not prox/distal/truncal Fatigue Asleep < 10 hrs night Not asleep during day Poor color “Pale”, not cyanotic Poor appetite Weight gain/no loss Shooting pains Multiple areas, durations <10 sec

  6. APPROACH TO DX PERSISTING SYMPTOMS III Resp. congestion Eyes “weird” Skin fleeting rashes Heart “jumps” Not hungry Stomach “weird” Feels faint ROS: Family Hx: Mother fibromyalgia, ib Grandmother hospice Father night work Schedules @ patient

  7. APPROACH TO DX PERSISTING SYMPTOMS IV PE: 80%ile Ht, 90%ile Wt, HR 110, BP 100/62 Animated, flushed, helpful Normal general exam Normal musculoskeletal & neuro Normal eye grounds Lab: Hb 12.8, WBC 10,200 (60P), Pl 238K Chem screen WNL ESR 9 UA WNL

  8. What’s the differential diagnosis? What further evaluation should be done?

  9. APPROACH TO DX PERSISTING SYMPTOMS V Signs_____ Summary Symptoms__ Fevers Headache Sore throat Weakness Fatigue Poor color Poor appetite Shooting pains Congest Eyes Rashes Heart Appetite Stomach Faint Chilly Normal weight Normal vital signs Normal exam Normal simple labs

  10. DIFFERENTIAL DIAGNOSIS Prior to vs After Interview Prior to vs After Exam Prior to vs After Lab Tests After all – Abused – Depressed – Fatigued

  11. DEMO & PSYCHO SYMPTOMS BY ILLNESS FatiguedDepressedJIA White race + Female>>male + Low socioeco + High parent ed + Acute onset + Overachiever +

  12. PSYCHOLOGICAL SYMPTOMS BY ILLNESS FatiguedDepressedJIA Social/extra act + + + Sad all the time + Lonely + + Hate/want to hurt self + Not as good as others + Don’t get along/not liked + Feel overtired + Not much energy + Somatic complaints + See doctors alot + Need help + Internalize stress + Carter BD. Dev Beh Ped 1996 Carter BD. Pediatr 1999

  13. TIMELINE EVIDENCE BASE FATIGUE 1990s NIH definition CFS Case series pediatrics Immunologic studies EBV, other ID 2000s Lyme, other ID Follow-up case series Non Dx pediatrics Endocrine – hypoadrenia Cardio – neurally med. hypotension – postural tachy “syndrome” Joint hypermobility – ?Ehlers Danlos

  14. EVIDENCE BASE FATIGUE Orthostatic instability in a population-based study of chronic fatigue syndrome. Jones JF et al. Am J Med, December 2005 – 58 adults CFS & 55 controls – Inpatient 2-day evaluation – Head-up tilt test pos. 30% CFS vs 48% controls Endurance exercise training in orthostatic intolerance: a randomized controlled trial. Winker R et al. Hypertension Mar 2005 – Military recruits 36 orthostatic intol – Randomized to “training” vs “control” x 3mo – Outcome OI: 6/11 training vs 10/11 control – Symptom scores sig in trained

  15. TYPICAL FINDINGS IN PATIENTS WITH “DECONDITIONING” • Age > 12 years • Preillness achievement high • Family expectations high • Acute febrile illness with onset easily dated • Family and outside attention high • Lengthy list, but vague complaints • Odd complaints (eg, “shooting” pains; 30- second “blindness”; stereotypic sporadic, brief unilateral tremors, jerks, or “paralysis”) • No daytime sleep • Preserved weight • Extreme cooperation with examination • Normal physical and neurologic examination • Normal results of screening laboratory tests

  16. MANAGEMENT “DECONDITIONING” Patient: Validate symptoms as accurate Incremental, forced return to school Exercise Predict symptoms No school absence w/o ped. visit No expectation performance Family: Validate their concern Validate their medical pursuit Justify your diagnosis Change focus to health Avoid “How do you feel?” No expectation performance 10 Provider = Quarterback Long approach >1975

  17. CASE 4 yr old adopted boy of mixed races Age 1 yr – Febrile illnesses q 30 days Age 2 yr– Characteristic fevers q 30 days Temp 1020 – 1030 x 3 days Swollen cervical nodes Age 3 yrs– Same char & periodicity ROS– No rash, URI, H/A, arthritis No mouth ulcers Completely well between episodes Good growth/development Immunizations fever

  18. Exam – Healthy, no dysmorphic, activity Height & weight 75%ile Normal tonsils, nodes, skin, joints Normal abdomen Tests – Hb 10.2 – 11.8 WBC < 10,000 (healthy) 10,000 – 15,000 (episodes) Neutrophils normal or , mild shift Platelets not elevated ESR 20 (healthy), 50-70 (episode) Normal immunoglobs (IgD=2 mg/dL) Chem, HIV, Sickle, Hb electro

  19. APPROACHING RECURRING FEVER ILLNESSES History History History Prolonged or relapsed single illness Multiple simple illnesses Recurrences at irregular intervals Recurrences regular/predictable/clockwork Duration Response to antipyretics, NSAIDs, antibiotics Occurrence with immunizations Additional Features of Episodes Symptoms URI, AOM Target organ? Rash? Mouth ulcers? Cardinal feature

  20. MORE HISTORY Interval between episodes Lingering symptoms Multiple URIs, asthma Severe bacterial/viral infections Perfectly well, energetic Few “regular” illnesses Good growth & development Family History Genetic background Sibs not ill with patient Autoimmune, -inflammatory dis Amyloidosis Parent w fevers as child

  21. PHYSICAL EXAMINATION Growth chart Thorough general Target organ Mouth ulcers, rashes, joints, nodes TESTS Simple - during episode & interval CBC, Chem, ESR Immunoglobulins Urine culture Imaging only as directed by exam

  22. PFAPA SYNDROME PF Periodic Fever 100% A Aphthous stomatitis 70% P Pharyngitis 72% A Adenopathy 88%

  23. CHARACTERISTICS OF PFAPA Add’l SCHC Clockwork cycles Pro 2h “glassy” Fever is cardinal Well between Happy parents Happy child Fewer “colds” Parent hx fevers Vanderbilt Male : Female 1.2 Onset PFAPA 2.8y Max temp 40.50 Duration fever 3.8d Duration epi 4.8d Episodes/yr 11.5 Well interval 28.2d Mean WBC 13,000 Mean ESR 41

  24. FOLLOW-UP PFAPA Natural History Mean follow-up 3.3y (1m – 9.4y) Mean age F/U 8.9y Char. episodes As at diagnosis Resolution 41% Mean duration 4.5y Treatments Cimetidine Rx and prophylaxis+ Prednisone Aborts epi but freq Tonsillectomy Resolution in 64% Thomas KT etal J Pediatr 1999;135:15-21

  25. HISTORY OF PFAPA 1987 Syndrome described (TN) 1992 Cimetidine Rx (CT) 1989 Tonsillectomy Rx (NC) 1990s Cases > 100 TN Cases > 100 PA Cases Israel, Spain, France, Italy, Greece

  26. Resolution of PFAPApost tonsillectomy

  27. WHAT IT ISN’TWHAT IS IT? “K I N E C T I O N”

  28. MANAGEMENT PFAPA Diagnosis – Clinical “best” (Tests: IgA&D, WBCs) – Re-visit – Beware “extra” S&S – Must be well between Management (Sequential) – Support +Cimetidine +Pred dx trial + Pred special circ + Tonsillectomy GET HELP IF “OFF” EXPECTED

  29. CYCLIC NEUTROPENIA Onset < 5 yrs Common Often < 1 yr Length fever epi 5-7 days Interval between 3 weeks Assoc. symptoms Ulcers* Gingivitis* Rare clostridia Growth & dev Ethnic/geographic None Auto dominant Lab findings Neutropenia Etio/Diagnosis Neutrophil elastase Apoptosis myeloid Twice wkly WBC Treatment G-CSF

  30. Autoinflammatory syndromes – Seemingly unprovoked inflammation without significant autoantibodies and autoreactive T lymphocytes McDermott

  31. PERIODIC FEVER SYNDROMES Periodicity Other Sympt Inher Etio_______ PFAPA Yes No No No Cyc Neutro Yes Gingivitis AD ELA2 AOM, Sinus Apop marrow FMF No Polyserositis AR MEFV Rash;scrotal Pyrin HIDS Yes/No Abdom pain AR MVK Rashes MVKase Mood swings Isoprenoids Immun trigger TRAPS No Myalgia AD TNFRSF1A Pseudo cell TNF receptor Periorb edema

  32. HYPER IMMUNOGLOBULIN D (HIDS) Onset < 5 yrs Common Length fever epi 4 days Interval between 4-8 wks, non-periodic/P Assoc. symptoms Abdom pain/diarrhea Arthralgia Rashes, splenomegaly Immun. trigger Ethnic/geographic Dutch, French, others Auto recessive Lab findings IgD*, IgA* Etio/Diagnosis Mutations MVK Mevalonic kinase Urinary mevalonate Treatment ? Etanercept ? Simvastatin

  33. TNFR ASSOC. PERIODIC FEVER (TRAPS) Onset < 5 yrs Uncommon Length fever Days to weeks Interval between Not periodic Assoc. symptoms Pseudo cellulitis rash Arthritis Ethnic/geographic Irish, Scot Others (FMF) Auto dominant Lab findings Serum TNFR1 Etio/Diagnosis Mutation TNFRSF1A Treatment Corticosteroid Etanercept

  34. Case Onset < 5 yrs Yes Length fever epi 3 days Interval between 4 wks, well Assoc. symptom Mood change Abdominal pain/V Immunization trigger Ethnic Mixed racial Lab findings Acute phase react Treatment Cimetidine no effect Tonsillectomy no effect Age 7 years IgD 5 mg/dL, IgA 72 mg/dL

  35. Missense mutations MVK HIDS

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