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Systemic Lupus Erythematosus

A CASE PRESENTATION

Roscelie L. Kho
Post-Graduate Intern
OBJECTIVES

History and physical


examination

To discuss the case of SJ, 15


years old, female, presenting Differential diagnosis
with intermittent fever more
than 6 days as to:
Formulation of the
working diagnosis

Course in the
emergency room and
wards
OBJECTIVES

Etiology Epidemiology

To discuss Systemic lupus Clinical


Pathogenesis
erythematosus (SLE) as to: manifestations

Diagnosis Management

Prognosis
GENERAL DATA

• S.J., 15 years old, female, child, Filipino, Roman


Catholic

• Currently residing in Sitio Kalingging Saavedra,


Moalboal Cebu Province.

• Born last October 11, 2003 at Moalboal, Cebu.

• Admitted in Cebu Doctors’ University Hospital for the


first time last November 16, 2018.
CHIEF COMPLAINT

FEVER
HISTORY OF PRESENT ILLNESS

Admitted at Cebu City Medical


Center.
3
WEEKS Managed as a case of Dengue
PTA Fever.
Discharged without any
complications.
HISTORY OF PRESENT ILLNESS

Intermittent low grade fever Tmax 38C.

1
WEEK Associated with dry cough and vesicular rash
on both lower extremeties.
PTA
Paracetamol 500mg tablet, 1 tablet every 4
hours, which brought temporary relief.

(-) colds, (-) joint pain, (-) body malaise and (-


)diarrhea noted.
HISTORY OF PRESENT ILLNESS
Fever (Tmax 38.5), fatigue, erythema and maculo
papular rash on both upper extremities,
aggravated by exposure to heat.
3
Sought consult at CHH OPD, laboratory tests taken:
DAYS CBC and urinalysis all of which showed
PTA unremarkable results as claimed by mother.

THM: Paracetamol 500mg to be taken every 4 hours


or as needed for temperatures 38 and above and
Cetirizine 10mg to be taken once during bedtime.

Medications brought temporary relief.


HISTORY OF PRESENT ILLNESS

(+)undocumented fever, (+)fatigue,


(+)erythema (+)maculopapular rash on
upper extremities and anorexia
MORNING Consult with attending physician done.
PTA

Advised admission for further


management.
PRENATAL HISTORY
• Mother was 40 years old with an obstetric score of
G2P1(1001), at the time of pregnancy.

• Prenatal care was started at 8 weeks AOG.

• Regular prenatal checkups with an obstetrician.

• Unremarkable laboratory test results and ultrasound findings.

• No known maternal illnesses or complications during the


course of pregnancy.
NATAL HISTORY
• Delivered as a live term female neonate via normal spontaneous delivery.

• Attended by an Obstetrician.

• Had good cry, was acyanotic.

• Birth weight of 8.2lbs.

• Birth rank of 2/3.

• No known complications.

• Discharged after 3 days.


POSTNATAL HISTORY
• Patient was exclusively breastfed for 1 month.

• Mixed feeding with formula milk thereafter.

• Semisolids and solids were introduced at 12


months.

• Usual diet: rice, fish, pork and fried foods.


POSTNATAL HISTORY
Immunization History
• Patient was given 1 dose of BCG vaccine and Hepatitis B vaccine at birth.

• Primary vaccinations given from a local health center:


• 3 doses of Pentavalent, pneumococcal vaccine and OPV vaccine
• 1 dose of Measles vaccine at 9 months
• 1 dose of MMR vaccine at 12 months.
• No Rotavirus vaccinations given.
• No booster vaccinations given.
POSTNATAL HISTORY
Growth & Development
• Patient is currently a grade 9 student.
PAST MEDICAL HISTORY
• 2015: Pneumonia and was admitted
at Moalboal district hospital.

• October 2018: Dengue Fever was


admitted at CCMC.

• No other previous hospitalizations


and surgeries.
FAMILY MEDICAL HISTORY
• Father is 49 years old, and is healthy.

• Mother is 55 years old and is healthy.

• Heredofamilial diseases include diabetes mellitus (both)


and Systemic Lupus Erythematosus (maternal).

• No family history of hypertension, bronchial asthma,


cardiac or kidney disorders.
PERSONAL & SOCIAL HISTORY
• Patient’s father is employed as a security guard and is the main
source of family income.

• Patient’s mother is a housewife and manages their small sari2x


store.

• The family of 5 live in their ancestral home, with a clean and


safe environment.

• Their source of drinking water is mineral water.

• Patient has no known allergies.


PHYSICAL
EXAMINATION
PHYSICAL EXAMINATION

• GENERAL SURVEY: awake, alert, not in respiratory distress.


• VITAL SIGNS:
• BP: 110/70 mmHg
• HR: 82 bpm
• RR: 22 cpm
• Temp: 37.2 C
• O2 sat: 98%

• Weight: 56.5kg (1.46)


• Height: 150cm (0.1)
• BMI: 25.1 (1.8)
PHYSICAL EXAMINATION

• SKIN: (+) flushed, (+) erythema & maculopapular rash on both upper
extremities. Skin warm and moist. Nails without clubbing or cyanosis. No
suspicious nevi. No petechiae, or ecchymoses.

• HEENT: Head—normocephalic/atraumatic (NC/AT). Hair (+) thinning,


noted increased hair fall. Eyes—Visual acuity not initially assessed. Sclera
white, conjunctiva pink. Pupils are 4 mm constricting to 2 mm, equally
round and reactive to light and accommodations. Disc margins sharp; no
hemorrhages or exudates, no arteriolar narrowing. Ears—Acuity good to
whispered voice. Tympanic membranes (TMs) with good cone of light.
Nose—Nasal mucosa pink, septum mid- line; no sinus tenderness. Throat (or
Mouth)—(+) Dry Lips, (+) dry tongue, (+) ulcerations on hard palate. Oral
mucosa pink, dentition good, pharynx without exudates.
PHYSICAL EXAMINATION

• NECK: Trachea midline. Neck supple; thyroid isthmus palpable,


lobes not felt.No cervical, axillary, epitrochlear, inguinal
adenopathy.

• CHEST AND LUNGS: Thorax is symmetric with good expansion.


Lungs resonant. Breath sounds vesicular; no rales, wheezes, or
rhonchi. Diaphragms descend bilaterally.

• CARDIOVASCULAR: Distinct heart sounds, no murmurs.


PHYSICAL EXAMINATION
• BREAST: Symmetric and without masses. Nipples without discharge.

• ABDOMEN: Protuberant with active bowel sounds, soft and non- tender; no
masses or hepatosplenomegaly. Liver span is 7 cm in the right midclavicular
line; edge is smooth and palpable 1 cm below the right costal margin. Spleen
and kidneys not felt. No costovertebral angle (CVA) tenderness.

• GENITOURINARY: No inguinal adenopathy. (-) Kidney punch sign.

• EXTREMITIES: Strong peripheral pulses, (-) edema, CRT<2secs


PHYSICAL EXAMINATION

• NEUROLOGIC: Alert, relaxed, and cooperative. Thought


process coherent. Oriented to person, place, and time.
Detailed cognitive testing deferred. Cranial Nerves: I—not
tested; II through XII intact. Good muscle bulk and tone.
Strength 5/5 throughout. Cerebellar: Rapid alternating
movements (RAMs), finger-to-nose (F→N), heel-to-shin
(H→S) intact. Gait with normal base. Romberg—maintains
balance with eyes closed. No pronator drift. Pinprick, light
touch, position, and vibration intact. Reflexes: 2+ and
symmetric with plantar reflexes downgoing.
SUMMARY OF IMPORTANT
FINDINGS
Pertinent Positives Pertinent Negatives
• History of prolonged • No loose bowel movement.
intermittent fever.

• Dry cough. • No nausea or vomiting.

• Fatigue. • No abdominal pain.

• Erythema and maculo papular • No jaundice.


rash on both upper extremities,
aggravated by exposure to
heat. • No joint pain.

• Heredofamilial disease: SLE


WORKING DIAGNOSIS
FEVER OF UNKNOWN
ORIGIN
FORMULATION

• Prolonged intermittent fever.

• Cause of the fever could not be identified after 2


visits.

• Fatigue
DIFFERENTIAL DIAGNOSIS

DIAGNOSIS RULE IN RULE OUT


COURSE IN THE
EMERGENCY ROOM
• Patient was seen in the emergency room with chief complains of
fever and upper extremity erythema.
• Patient was afebrile, not in respiratory distress and with stable
vital signs.
• Patient was given the admitting diagnosis of Fever of Unknown
Origin.
• Initial plan of management was to hydrate the patient and give
supportive therapy until the cause of the fever can be identified.
• Venocolysis was immediately started with 1 liter of PNSS infused
at KVO rate.
• Medications given include Cetirizine 10mg 1 tab once a day
preferably at bedtime which provided slight relief of generalized
pruritus.
LABORATORY TESTS
• Serum electrolytes (sodium, potassium, chloride and
creatinine)
• serum creatinine of 0.4mg/dL
• serum sodium and potassium were within normal limits.
LABORATORY TESTS
• CBC
• Deceased hematocrit of 27.7
• Leukopenia of 2.9
• Thrombocytopenia of 83,000.
LABORATORY TESTS
• Urinalysis
• unremarkable results.
• Serum creatinine resuts showed.
LABORATORY TESTS
• A chest xray was taken which showed clear lung fields with no signs
of cardiomegaly.

• CRP results were within normal limits

• ESR was taken and results showed increased result of 126mm/hr.

• Typhidot test was also done and results showed non-reactive


Samlonella IgG and IgM antibodies.
TYPHOID
• Blood culture and sensitivity with ARD was also taken. FEVER
ruled out.
LABORATORY TESTS
• A chest xray was taken which showed clear lung fields with no signs
of cardiomegaly.
COURSE IN THE WARDS
FIRST HOSPITAL DAY DENGUE
FEVER
• Intermittent febrile episodes (Tmax 39.1).
ruled out.
• Loss of appetite and slight burning epigastric pain, PS 4/10.
No nausea and vomiting was noted.
• Repeat CBC, CK-MB, Peripheral Smear Exam, Serum Lactate
Dehydrogenase, Reticulocyte count and Dengue Serology Tests
were done. Repeat CBC showed Hemoglobin of 10.8,
Leukopenia of 2.5 and Thrombocytopenia of 67, 000. Dengue
Serology tests showed Negative Dengue NS1 and Nonreactive
Dengue IgG and IgM. Paracetamol 500mg, 1 tablet was given
every 4 hours for temperatures above 38C and Ranitidine
50mg/2ml, 50mg via IVTT one dose was given to alleviate
epigastric pain. Medications brought temporary relief as
claimed by patient.
COURSE IN THE WARDS
FIRST HOSPITAL DAY
• Paracetamol 500mg, 1 tablet was given every 4 hours
for temperatures above 38C and Ranitidine
50mg/2ml, 50mg via IVTT one dose was given to
alleviate epigastric pain.

• Medications brought temporary relief as claimed by


patient.
COURSE IN THE WARDS
SECOND HOSPITAL DAY
• Intermittent febrile episodes (Tmax 38.8C).

• Malar rash, periorbital edema and ulcerations on the


hard palate.

• Patient also complained of intermittent oral pain PS


2/10 and bilateral intermittent knee joint pains PS
4/10.

• SLE considered.
COURSE IN THE WARDS
SECOND HOSPITAL DAY
• Laboratories taken include ANA-IF, AST, ALT, Coombs
Test, and Urinalysis with RBC morphology.
• Cetirizine 10mg 1 tab at bedtime was discontinued and
patient was started on Prednisolone 20mg tablet, 1 tablet
2 times a day, Hydroxychloroquinole 200mg, 1 tablet
once a day, Multivitamins + Folic Acid + Iron 1 capsule
once daily and Calcium with Vitamin D3 1 capsule once
daily.
• Bacticol oral solution was to be gargled thrice daily.
• Advised to turn off light in room when not needed and
avoid sun exposure as much as possible.
COURSE IN THE WARDS
THIRD HOSPITAL DAY
• Intermittent febrile episodes (Tmax 38.5C).
• (+)malar rash, periorbital edema and hard palate
ulcerations were noted.
• Prednisolone 20mg was shifted to Prednisone 20mg 1
tablet 2x a day after meals or with full stomach.
Furosemide 40mg tablet, ½ tablet orally was given to
correct fluid retention and edema.
• Patient complained of slight blurring of vision and
increased tearing on both sides. Patient was therafter
referred to an ophthalmologist.
COURSE IN THE WARDS
FOURTH HOSPITAL DAY

• Febrile episodes (Tmax 38C).

• (+) malar rash was noted to be resolving.

• (+)Periorbital edema and ulcerations on the hard


palate.

• Examined by an ophthalmologist and Phenylephrine


eye drops, 1 drop on both eyes every 15 minutes was
prescribed.
COURSE IN THE WARDS
FIFTH HOSPITAL DAY
• Afebrile Day 1.
• (+) decreasing periorbital edema and ulceration on the hard
palate.
• (-) malar rash.
• (-) complains of knee joint pains.
• Phenylehrine eye drops were discontinued.
• (+) Error of refraction OU.
• Repeat CBC was done which showed thrombocytopenia of
135,000.
COURSE IN THE WARDS
SIXTH HOSPITAL DAY

• Afebrile Day 2.

• (+) decreasing periorbital edema and ulceration on


the hard palate.

• (-) malar rash & (-) complains of knee joint pains.

• Deemed fit for discharge.

• Follow up was advised.


FINAL DIAGNOSIS
Systemic Lupus
Erythematosus
Systemic Lupus
Erythematosus
• Multisystem inflammation in the presence of
circulating autoantibodies directed against self
antigens.
Systemic Lupus
Erythematosus
• Adults > children

• African American, Asians, Hispanics, Native Americans and


Pacific Islanders.

• Females > males 2-5:1

• Up to 20% of all individuals are diagnosed prior to age 16.


Systemic Lupus
Erythematosus
• Adults > children

• African American, Asians, Hispanics, Native Americans and


Pacific Islanders.

• Females > males 2-5:1

• Up to 20% of all individuals are diagnosed prior to age 16.


Systemic Lupus
Erythematosus
Genetic Predisposition

Etiology

Environmental Factors Hormonal Factors


Systemic Lupus
Erythematosus
Systemic Lupus
Erythematosus

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