pfapa 09.04.2013

7/27/2019 PFAPA 09.04.2013

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Morning Report

September 4th, 2013

Carrie Johnson

7/27/2019 PFAPA 09.04.2013


HPI•

In Urgent Care in August• You see a 3 yo Caucasian female with fever,

sore throat and swollen lymph nodes for 2days

• Fever reach 104 on day of presentation

• Complaining of throat pain, discomfort withswallowing

• Decreased oral intake

• Also complains of generalized stomach ache;no vomiting or diarrhea

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HPI continued

• Some body aches, head aches intermittently

• No altered mental status

•

No cough, congestion, rhinorrhea, ear pain, orsnoring.

• No joint pain, rash, night sweats, weight loss.

•

No dysuria, no back pain• Using ibuprofen which seems to help fevers,

pain

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• PMH/PSH:

– Bilateral Grade III VUR, no prophylaxis, no history of UTI.

– Has had a few similar episodes of lymph nodeenlargement and fever “fairly regularly” throughoutpast year. Always with a negative strep test. Had bloodwork done at PCPs office, slight elevation of WBC (20-

21), normal differential, platelet, hemoglobin, normalUA/culture. Received a course of antibiotics eachtime. Between episodes feels well.

• Unremarkable social history and family history.

No sick contacts, no daycare.• No medication use other than Ibuprofen during

illness

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Physical Exam• Temp 102° F, HR 110, RR 20, BP 86/52

• Wt: 73%ile for age, Ht: 84%ile for age• Gen: Well nourished, non-toxic

• HEENT: EOMI, no conjunctival injection, TMs normal, naresclear. Posterior OP with erythema, thin tonsillar exudate.No lesions, masses, or ulcerations. Discomfort with

swallowing. • Neck: Full ROM, tender bilateral submandibular lymph

node enlargement (1.5 cm). No overlying erythema

• CV: RRR, no murmurs, rub, or gallop. Good perfusion

•

Resp: CTAB, no wheezing or crackles, no increased WOB• Abd: soft, no tenderness to palpation, normoactive BS

• Extrem: full ROM, nontender

• Skin: no rashes

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Differential

• 3 year old female with recurrent episodes of

fever and tender lymph node enlargement.

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DifferentialID

Lymphadenitis

Pharyngitis

-Group A strep (carrier in home?)

- viral (rhinovirus, parainflu, coronavirus, adenovirus,coxsackievirus, Human metapneumovirus)

- H flu, M catarrhalis, streppneumo, mycoplasma

Retropharyngeal AbscessPrevertebral Abscess

Atypical mycobacterium

Bartonella Henselae

EBV/CMV/HIV

Onc

Lymphoma

Neuroblastoma

Heme

Cyclic Neutropenia

Rheum/Imm-Serum Sickness

-PFAPA

-Familial Mediterranean fever

-Tumor Necrosis Factor

Receptor-associated PeriodicSyndrome (TRAPS)

-Hyperimmunoglobulin D

Syndrome

-SLE

-Sarcdoidosis-Histiocytic necrotizing

lymphadenitis (Kikuchi Disease)

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More Past Medical History

• When digging through the chart more…

• First presented with similar illness with high fever andswollen lymph nodes nine months ago.

• Since then, doctor’s office visit every 4-8 weeks for

same symptoms.• Always with a negative rapid strep and culture. Often

diagnosed with Cervical lymphadenitis or AOM.Received 5 courses of antibiotics

•

Resolution of symptoms in between• Patient lovingly nicknamed her lymph nodes “my

rocks,” and anticipated their reappearance every 4-6weeks.

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Periodic Fever with Aphthous

stomatitis, Pharyngitis, and Adenitis

• Abrupt onset of high fever (38.9-41.1°C),

malaise, irritability, sore throat.

• Aphthous ulcers in first 12-24 hours

on lips or buccal mucousa

• Pharyngitis (+/-) exudate

•

Tender cervical adenopathy• May also have HA, myalgia, abdominal

discomfort

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Natural Course of Illness

• Onset age 2 to 5 years old

• Benign, self limited (unknown etiology)

• Mean duration of attacks ranges from 4.5 to 8

years.• With time, episodes are of shorter duration and

occur less frequently.

•

Within 5-7 years of onset, most childrenasymptomatic.

• Limited cases occurring in adulthood

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Laboratory Findings

• May have mild leukocytosis (15-19), Elevation

of ESR ( 49 +/- 30 mm/hr), CRP increase (~ 2.5

to 7 mg/dl).

• No increased presence of ANA above baseline

population estimates ( 1 in 30 children)

• All lab values normalize between episodes

• Strep test should be negative.

– If positive may represent benign carriage

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Diagnosis

Based on Clinical Presentation

• 3+ episodes of fever no more than 5 days at

regular intervals (on average Q 3-6 weeks)

• Pharyngitis + aphthous ulcers OR tender

cervical LAD

• Normal growth parameters, good health

between episodes (no B symptoms)

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Treatment

• Optional

• NSAIDS, tylenol for fevers

• Tonsillectomy (Controversial) – Small study (N 39): Remission in 63%,

• Prednisone (benefit vs. harm) – Single dose of 1-2 mg/kg has dramatic response on fever, pharyngitis

within hours

– Repeated doses shorten the interval between attacks in 25% of cases

– Can try Colchicine to extend interval between attacks but effect isshort lived.

•Cimetidine (Case Reports) – 20-40 mg/kg divided BID

– May help with reducing or eliminating recurrent episodes

– Continue drug therapy for 6 months if initial benefit

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PFAPA must be excluded when…

• Family history of recurrent fever (consider

hereditary autoinflammatory syndromes)

• Elevated CRP/ESR between attacks (suggests

chronic inflammation)

• Atypical symptoms (are not consistent with

previous pattern)

• Neutropenia (consider cyclic neutropenia)

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Cyclic Neutropenia

• Rare

• Onset in first year of life

• Recurring fevers and neutropenia ANC <200

(very consistent time frame for the individual;range 14-35 days)

• Familial pattern (AD inheritance)

• Symptoms: malaise, fever, aphthousstomatitis, gingival/mucosal infection,sub/cutaneous infections

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Periodic Fever/Autoinflammatory Syndromes

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References

• Donald Goldsmith. Periodic Fever Syndromes.Pediatrics in Review Vol 30 No.5, May 2009.

• Shashi Sahai. Lymphadenopathy. Pediatrics in

Review 2013;34;216. DOI: 10.1542/pir.34-5-216• Thomas KT, Feder HM Jr, Lawton AR, Edwards

KM. Periodic Fever syndrome in children Journalof Pediatrics. 1999; 135: 15.

• Shai Padeh, MD. Periodic Fever with aphthousstomatitis, pharyngitis, and adenitis (PFAPAsyndrome). Uptodate.com

pfapa 09.04.2013

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