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“Prolonged fever”
EXTERN
CONFERENCE
Supervised by
Prof. Achra Sumboonnanonda MD
Rattanavalai Chantorn MD
Paisarn Parichatiganond MD
Patient profile
 12 years old Thai girl
 CC: Low grade fever for 1 month PTA
 Hx: 4 mo PTA She had persistent
erythematous rash on both cheeks
and active hair loss. She came to a
local hospital and was diagnosed as
dermatitis.
 1Mo PTA. She had low grade fever
relieved by antipyretic drug.
 She had no other symptoms except rash
on her face that sometimes aggravated
by sun exposure and increase excessive
hair loss
 3 wk PTA she became more malaise,
pallor and went to private clinic. She
received parenteral fluid and oral
medications, the symptoms were partial
improved.
 2 wk PTA she still had persistent fever then
she went to a local hospital.
 At the hospital
PE: erythematous rash on malar area
The rash became worse and she
developed painful ulcer on her lips and
oral mucosa, She also had erythematous
macules on her soles and swelling of her
face
 Investigation at the hospital
-BUN/Cr 22/0.7 mg/dl
-CBC: Hb 8 g/l, Hct 27 %, WBC 4,470 /mm3
(N75%, L20 %) Plt 196,000/mm3
-Stool exam : WNL
-UA : WNL
-Urine culture: > 105 Streptococcus Gr. D,
Enterococci spp
 Rx :Ceftriaxone 2 gm OD x 9 d then
Cefotaxime 1 gm IV q 6 hrs x 3d
Doxycycline x 7d
Gentamycin 100mg IV OD x 7d
Symptoms persisted then the patient
was referred to “Siriraj hospital”
 Past history : healthy
 Family history : no family history of
atopy
 Drug history: analgesic drug allergy
Physical examination
 V/S: T 38.3 oC, P110/min, R20/min,
BP118/60 mmHg
 BW 37.5 kg(P25-50), HT 155 cm(P50-75)
 GA: Irritable, look weak, not cooperative,
mildly pale, no jaundice, no dyspnea,
dry lips, good skin turgor, no sunken eye
balls , capillary refill <2 sec, no eschar
 HEENT: findings as figures
Bilateral scaly erythematous to brownish patches
at malar eminence, nasal ridge and nasolabial
folds,scaly edematous erythematous painful lips
Round shape
erythematous macule
with central
hyperpigmentation
single erythematous patch with
peripheral hyperpigmentation on scalp
Painful shallow ulcer at
hard palate with oral
thrush
RS: normal
CVS: normal
ABDOMEN: normal
Extremities: Bilateral symmetrical partially
blanchable erythematous to purplish
macules and papules on both palms
and soles, no sign of joints inflammation
 NS: normal
 No lymphadenopathy
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Investigation
CBC
 Hb 8.6 g/dl, Hct 26.9 %, MCV 71.7 fl, RDW
14.9 %, WBC 2,840 /mm3 (N 72.2, L 20.1),
Platelets 198,000/mm3
 HCMC RBC, no hemolytic blood picture
Urinalysis
 pH7.0, Sp.Gr. 1.015, Protein +++, Occult
blood +, Bilirubin neg, Acetone neg,
WBC 0-1/HF, RBC 1-2/HF, no cast and
bacteria
Blood chemistry
 BUN 13.0 mg/dl, Cr 0.5 mg/dl
 Na 135 mEq/dl, K 4.5 mEq/dl,
Cl 95 mEq/dl, HCO3- 19 mEq/dl
CXR WNL
U/C, H/C : pending
Problem list
 Prolonged fever for 4 weeks
 Active hair loss for 4 months
 Abnormal skin manifestation on scalp,
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face, ears, lips, mouth and extremities
Oral thrush
Anemia: Hypochromic,microcytic
Leukopenia and lymphopenia
Proteinuria
Children with prolonged fever
 Fever in this patient can be defined as fever
of unknown origin (FUO)
 The Petersdorf and Beeson criteria for FUO,
definition in 1961 are:
▪ a body temp ≥ 38.3°C for at least 3weeks;
and
▪ failure to establish a diagnosis after 1 week
of investigation.
Differential diagnosis of FUO
 Infection
 Autoimmune disease
 Neoplasm
 Miscellaneous (drug-related fever,
factitious fever, etc.)
Relation between infection and
autoimmune disease
 Clinical symptoms of infection may be
indistinguishable from those of
autoimmune disease
 Immunosuppressive therapy for
autoimmune disease may lead to
increased susceptibility to infection
Infectious cause of FUO in
children
 Salmonellosis
 Tuberculosis
 Rickettsial disease
 Bacterial endocarditis
 Infectious mononucleosis
Malar rash
Criteria of SLE
Discoid rash
Photosensitivity
Oral ulcer
Arthritis
Serositis
Renal disorder: persistent proteinuria
Neurologic disorder: seizure, psychosis
Hematologic disorder: hemolytic anemia,
leukopenia, lymphopenia, thrombocytopenia
 Immunologic disorder: anti-dsDNA, anti-Sm,
antiphospholipid antibody
 ANA positive
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ACR 1982, updating classification criteria 1997
Characteristic of fever in active SLE
disease
 Non-shaking fever
 Manifestation of active SLE: such as
 Acute cutaneous LE
 Arthritis
 Hypertension, Edema
 Leukopenia with lymphopenia,
Thrombocytopenia
“Active SLE disease”
THE MOST LIKELY DIAGNOSIS
HOW TO RECOGNIZE SLE
PRESENTING SYMPTOMS
IN PEDIATRIC PATIENTS ?
Study from department of Pediatrics, Faculty of
Medicine Siriraj Hospital, Mahidol University
Suroj Supavekin MD*,Wanida Chatchomchuan MD**, Anirut Pattaragarn
MD*, Vibul Suntornpoch MD*, Achra Sumboonnanonda MD**
J Med Assoc Thai 2005; 88(Suppl 8): S115-23
 From July 1985 to March 2003, 101
patients
 The major clinical presentation of
pediatric SLE are
- Renal (86.2%)
- Skin and mucocutaneous (76.3%)
- hematological involvement (73.4%)
Pediatric Systemic Lupus Erythematosus in Siriraj Hospital
Suroj Supavekin MD*,Wanida Chatchomchuan MD**, Anirut Pattaragarn MD*, Vibul Suntornpoch MD*,
Achra Sumboonnanonda MD**
Signs and Symptoms at Diagnosis
J Med Assoc Thai 2005; 88(Suppl 8): S115-23
The Results of Renal Biopsies
J
Med Assoc Thai 2005; 88(Suppl 8): S115-23
Classification of lupus nephritis
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Class I: Minimal mesangial LN
Class II: Mesangial proliferative LN
Class III: Focal LN
Class IV: Diffused LN
Class V: Membranous LN
Class VI: Advanced sclerosis LN
International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003
MANAGEMENT
Further investigation
Work up for source of infection
 KOH for oral thrush: Pseudohyphae with
budding yeast
 Stool concentration for parasite x 3days
negative
 Urine culture (10/4/50) No growth
 Hemoculture (10/4/50) No growth
 PPD skin test: Negative at 48, 72 hr
 Consult dentist: No dental caries
Further Investigation
 peripheral blood smear (14/4/2550):
hypochromic microcytic RBC, no
anisopoikilocytosis, Plt 10-15/HPF
 Reticulocyte count
0.61%
 Direct coomb’s test: negative
 Serum ferritin (17/4/2550): 1,537 (13-50)
“Iron deficiency anemia”
Further investigation
Autoimmune profile
 ANA
Positive
Positive with Fine-speckled pattern titer >1:2,560
Positive with Coarse-speckled pattern
Positive with Homogeneous pattern
Positive with Peripheral pattern
Positive with Anti-Cytoplasmic Ab
 Anti-ds DNA Positive titer > 1:160
 C3 level
 C4 level
36.8 (N 83-177)
6.56 (N 15-45)
Further investigation
 Total protein 5.4 g/dl, Albumin 1.7 g/dl
 Urine Creatinine 28.3 mg/dl
 Urine Micro-TP
148 mg/dl
 Urine protein/creatinine ratio 5
 Urine protein 24 hr
55 mg/kg/d
“Nephrotic range proteinuria”
 Renal biopsy
Indication for kidney biopsy
 All patient who correlate with criteria
of LN
 Nephrotic patient with undetermined
diagnosed of Diffuse proliferative GN
or Membranous GN
 Patient whose renal function get worse
despite of receiving high dose steroid
TREATMENT
Patient education
 Avoid sunlight
 Avoid physical and mental stress
 Drug compliance
Fever and malaise:
• Low dose NSAIDs with antimalarial drug or
low dose oral corticosteroid
Cutaneous lesion:
• Sunblock cream with topical steroid and
antimalarial drug
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Treatment of lupus nephritis
Base on renal pathology
Class I: no treatment required
Class II: short course treatment of low
dose steroid (prednisolone 0.5-1 MKD)
Class III: prednisolone 1-2 MKD(max60mg/d)
+immunosuppressive drug(Azathioprine
2MKD)
Class IV: prednisolone 2 MKD+pulse
cyclophosphamide
Class Ⅴ: prednisolone 1-2 MKD
Class Ⅵ:slow renal progression,aggressive
immunosuppressive drug not required
Progression
-Continue cefotaxime until 7days(10-13/4/50)
-Cotrimazole troche to treat oral candiasis
- Septic work up: all negative
so non-infectious cause is most likely
Medication
Prednisolone (2mg/kg/d)
Hydroxychloroquine ( 5mg/kg/d)
0.02%TA cream apply to lesions at face
0.1% TA cream apply to lesions on scalp
Progression
 Anemia:FeSO4 (200mg) 1 tab PO tid pc
(5.6 mg/kg/day) (start 17/4/2550)
 New onset HT:BP 128/86(16/4/2550)
max135/73 mmHg (P95 124/81mmHg)
- Enalapril (5mg) 1 tab PO OD pc
 Follow up urinalysis 18/4/2550
pH 7.0, sp.gr 1.010, protein neg,
WBC 0-1,RBC neg, occult blood neg,
others neg
RENAL PATHOLOGY
Renal pathology
 Mesangial hypercellularity and matrix
expansion
 No endocapillary proliferation
 No crescent
 Microthrombi in 1 arteriole
Imp: Lupus nephritis class II with
thrombotic microangiopathy(TMA)
References
• Suroj Supavekin MD,Wanida Chatchomchuan MD, Anirut
Pattaragarn MD, Vibul Suntornpoch MD, Achra
Sumboonnanonda MD Study from department of
Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol
University J Med Assoc Thai 2005; 88(Suppl 8): S115-23
• The Subcommittee for Systemic Lupus Erythematosus
Criteria of the American Rheumatism Association
Diagnostic and Therapeutic Criteria Committee. The 1982
revised criteria for the classification of systemic lupus
erythematosus. Arthritis Rheum 1982;25:1271-7
• Wallco DJ, Halm BH, eds. Dubois’ lupus erythematosus. 5th
edn. Baltimore: Williams and Wilkins, 1997
Thank you