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SYSTEMIC LUPUS

ERYTHEMATOSUS
GENERAL DATA
• PM
• 9 y/o
• Female
• EDD/PA
• Roman Catholic
• Born on 22 May 2008
• Zamboanga del Sur
• Admitted for the first time on13 August 2017
CHIEF COMPLAINT
• Increased Blood Pressure (160/100)
HISTORY OF PRESENT ILLNESS

9 MONTHS PTA (+) Bilateral Pitting Edema


of the lower extremities

(+) Abdominal
enlargement

No consult done and no


medications taken
HISTORY OF PRESENT ILLNESS

8 MONTHS PTA (+) Bilateral Pitting Edema


of the lower extremities

↑ in abdominal girth

No consult done and no


medications taken
HISTORY OF PRESENT ILLNESS

6 MONTHS PTA Consult with IM-Nephro

Admitted at a private hospital


inPagadian, Zamboanga del Sur
as a case of NEPHROTIC
SYNDROME

UTZ: Bilateral Renal Enlargement


HISTORY OF PRESENT ILLNESS

6 MONTHS PTA Medications:


Methylprednisolone
OD for 3 days
Lasix
Calci-Aid

Discharged after 2
weeks
HISTORY OF PRESENT ILLNESS

5 MONTHS PTA (+) MoonFacies, Bilateral Pitting


Edema,↑Abdominal
Enlargement

Medications

Self-medicated
withSambongand Banaba
leaves
HISTORY OF PRESENT ILLNESS

4 MONTHS PTA (+) Scaly, erythematous, pruritic


lesions on the face and
shoulders

Consult done with a faith


healer

Given unrecalled herbal


medications
HISTORY OF PRESENT ILLNESS

2 MONTHS PTA (-) Fresh lesions, (+)


Scars from rashes

Recurrence of bilateral
edema and ascites

No meds taken and no


consult was done
HISTORY OF PRESENT ILLNESS

1 MONTH PTA

Progression of bilateral
pitting edema and ascites
HISTORY OF PRESENT ILLNESS

9 DAYS PTA Consult with IM-


Nephro

Consult with
Pediatrician

Admitted for 2 days


HISTORY OF PRESENT ILLNESS

3 DAYS PTA (+) Headache, vomiting,


seizure, diarrhea

Admitted atPagadian, noted


increase in BP and was treated
with Albumin

Decrease in abdominal girth


HISTORY OF PRESENT ILLNESS

1 DAY PTA Advised to continue


albumin treatment

Advised to transfer to
AFPMC

Admission at our
institution
PAST MEDICAL HISTORY
• (-) Hypertension • No allergies to food and drugs
• (-) Diabetes Mellitus • No previous hospitalizations
• (-) Bronchial Asthma • No previous surgeries
• (-) Blood Dyscrasia • No history of blood transfusion
• (-) PTB
• (-) Thyroid Diseases
• (-) Cancer
FAMILY HISTORY
PATERNAL SIDE MATERNAL SIDE
• (+) Hypertension • (+) Hypertension
• (+) Diabetes Mellitus • (+) Diabetes Mellitus
• (+) Bronchial Asthma • (-) Bronchial Asthma
• (-) Cancer • (-) Thyroid Diseases
• (-) Cancer
FAMILY PROFILE
• Father: 35 y/o, active military for 14 years (SSG), asthmatic
• Mother: 37 y/o, housewife, apparently well
• Brother: 8 y/o, NSD, Grade I, apparently well
SOCIAL AND ENVIRONMENTAL HISTORY
• Lives with parents, grandparents, and brother in
Zamboanga del Sur
• Currently in Grade III
• Drinking water: Distilled Water
• Has no food preference
• Water supply: Zamboanga del Sur Water District
Physical Examination
General Survey
• Conscious, coherent not in cardiorespiratory distress
• GCS 14 (E4V4M6)
• BP- 110/70
• PR-130
• RR- 25
• Temp- 37.0
• O2 Sat- 96%
Visual Acuity
• Patient has difficulty on verbalizing

• Pupils- 5-6mm ERTL


External Eye
• (-) swelling
• (-) erythema
• (-) tenderness
Extraocular Muscles
• Preferential right gaze even on left lateral decubitus but observed one
instance of central and left gaze
Funduscopic Exam
• (+) ROM
• Clear media
• Well defined margins
• CDR 0.3
• AVR 2:3
• (-) hemorrhage
• (-) exudates
DIFFERENTIAL
DIAGNOSIS
BLESY GARCIA
OPTIC NERVE GLIOMA

Preferential gaze Progressive visual


loss
Retroorbitalpain
Massive swelling of
optic nerve
CRANIOPHARYNGOMA

Nystagmus Headache
Vomiting
Vision loss
Enlarging head
circumference
OPTIC NEURITIS

Preferential gaze
Nystagmus
History of
autoimmune
disorder (lupus
nephritis)
COURSE IN THE WARD
DATE S O PLAN

13 (+) BP: 90/60 • Diagnostics: CBC qPC, Serum Electrolytes (Na, K,


AUG generalized CR: 73 Cl, Ca), BUN, Crea, UA, CXR, ANA quantitative,
17 body crea ratio, Lipid profile, TPAG, KUB UTZ
RR:28
weakness • Medications:
T: 36.9
- Captopril 25mg/tab OD
• bipedal and facial - Furosemide 40mg/tab, ½ tab q12
edema, increased - Prednisone 20mg/Tab BID
abdominal girth - Calcium Carbonate 500mg/tab BID
• Diet Low salt, Low fat
• DX:Nephitic VS • Referral to Pedia Nephro
Nephrotic
Syndrome
IMAGING
Chest X-ray -Hazy opacities are seen on both inner lungs
08/13/2017 -Heart is enlarged
-Aorta is dilated
-Diaphragm and sulci are intact
-The rest of the visualized chest structures are unremarkable

Result Ref range


C3 28.6 mg/dL 12 mos-10yrs: 80-150 mg/dl
ANA >32 (-) : <0.7 ratio
Equivocal:: 0.7-1.0
(+): >1.0
DATE S O PLAN

15 AUG 17 (+) Generalized Wt: 28.2 • Low Salt Low Fat diet
body weakness BP: 130/100 • BP monitoring strictly q2
3RD HD Ti: 390 • Referred to pedia-nephro:
T0: 300  Start albumin 25% 100cc x 4H
UO: 0.44cc/kg  Furosemide 20mg/TIV mid and post albumin
BSA: 0.96 w/ strict bp prec
T: 38.4  Hold captopril
 Start amlodipine 5mg/tab q12
• bipedal and  Nifedine 10mg/tab q6H
facial  Diazepam 8mg TIV for active seizure
edema  Start ceftriaxone 700mg TIV q12H—(50mkd)
• increased • Fluid was restricted to 200cc q8H
abdominal • (Paracetamol 250mg/ 5ml, 6ml q4 (T> 37.8)
girth, • Diagnostic: TPAG now
Date S O Plan
16 AUG 17  Loss of BP: 140/100 Transfer to PICU
vision CR: 96 Medications:
RR: 30 1. Albumin 25% 100c x 4H
T:36.9 2. Furosemide 20mg/TIV mid and post
O2sat: 97% albumin
AC: 73.5 3. Furosemide 20mg/TIV q8
4. Amlodipine 5mg/tab, 1 tab q12
5. Nifedine 10mg/tab q6H
4th HD; DX: Nephrotic 6. Metoprolol 50mg/tab, 1 tab OD
1st PICU Syndrome; t/c 7. Ceftriaxone 700mg/TIV q12
Lupus Nephritis 8. Diazepam 8mg/TIV for active seizure
Date S O Plan
17 (+) bipedal and Wt: 28.2 • Diagnostic: Mantoux test, Repeat UA
AUG facial edema AC: 73.5 • Medications:
17 T:36.6 1. Albumin 25% 100c x 4H
increased UO 1.12cc/hr 2. Furosemide 20mg/TIV mid and post albumin
abdominal girth 3. Furosemide 20mg/TIV q8
4. Amlodipine 5mg/tab, 1 tab q12
discoid rash on the 5. Nifedine 10mg/tab q6H
extremities 6. Metoprolol 50mg/tab, 1 tab OD
(bilateral) DX: Lupus 7. Ceftriaxone 700mg/TIV q12
Nephritis 8. Diazepam 8mg/TIV for active seizure
9. Calcium Carbonate 500mg/tab, 1 tab BID
10.Calcitriol 0.25mg/tab, 1 tab ODHS
11.Prednisone20mg/tab, 1 tab OD in am
12.Paracetamol 250mg/5ml, 6ml q4 for T:37.8
Date S O Plan

23 (+) productive (+)Seizure Patient transfer to PICU


AUG cough BP: 130/90 Diagnostics:
17 (+)fever: 37.8  For cranial CT with contrast
(-) colds, chills,  For renal biopsy once stable
vomiting, loose  Blood GSCS
DX: Lupus
stool,  Repeat Sr Electrolyte
Nephritis, R/I
abdominal pain CNS Lupus
23 AUG Result Ref Method
Anti-ds-DNA 394 IU/ml (-) if <10 IU/ml Fluoroenzyme Immunoassay (FEIA)
Equivocal if 10-15 IU/ml
(+) If >15 IU/ml
Date S O Plan

24 > Irritable BP: 1140/90  Patient was referred to Pediatrics-Neurology


AUG CR: 150 regarding CNS lupus
17 RR: 30  Plain cranial MRI was advised which was
T: 38.8 scheduled on September 26, 2017 at 0900H
 Diazepam dose was decreased 5mg TIV if
UO: 0.38cc/kg active seizure

Facial edema Patient was referred to Pediatrics-Nephrology


(+) Neurologic lapses service
• Enalapril 5mg/tab 21 tab Q12 was started
• Amlodipine dose was increased to 10mg
once a day
• Metorprolol was discontinued
• Furosemide was on hold
Date S O Plan

25 • GCS 15 • Referred to Ophtha for further evaluation


AUG • (+) edema • 1 ‘u’ of PRBC was transfused
17 • (+) tachypnea • Diphenhydramine 112/30 mg TIV 30 mins
• (+) fever (T:37.9C) prior to blood transfusion
• Furosemide 200 mg TIV post blood
transfusion were given
• Referral to Rheumatology once stable
• DIAGNOSTICS:
• For lumbar tap once stable
• For MRI (cranial- plain) once with funds
OPHTHALMOLOGY NOTES
Date S O Plan

25 2 days PTC: • GCS: 12 (E3V6M3) • For referral to: Neuro service for Acute Visual
AUG • preferential • VA: No visual threat Loss probably Central in origin
gaze, left • Pupils: 5-6mm dilated NRTL • For Cranial MRI with Orbital Cuts
17 • EOM: Preferential gaze, right • Refer back once with MRI Result or if once
1 day PTC: even on L lateral decubitus but stable for full Ophtha exam or re-assessment
• preferential observed some central and
gaze, right left gaze
• Fundus: (+ROR), clear media,
well defined margin, AVR 2:2,
CDR 0.3

DX:
• Normal fundus finding at time
of examination
• Preferential right gaze
• T/C Acute Visual Loss probably
Central
Date S O Plan

08 Awake, • (-) Edema, CRT <2s Rheuma consult was done w/ following
Sept not in • CR: 107 suggestions:
17 distress • RR: 22-24, O2Sat:98%  Increase: Prednisone 20mg/tab, 2
• (-) fever (36.1- 36.7) tabs OD after meal
• BP: 110/80  Secure Cyclophosphamide & start
• (-) bleeding 500mg IV once a month for 6 months,
• GCS 15 then 3 mos there after

DX: SLE Nephritis


16 Sept 25 Sept

Hgb 101 86

Hct 0.290 0.26

RBC 3.41 2.90

WBC 26.8 18.5

Seg 0.91 0.73

Lymp 0.05 0.15

Eo 0 0.01

Mono 0.03 0.10

Baso 0.01 0.01

Plt 247 256

Crea 93.80 60.80

K 5.9
Imaging
Cranial MRI with Orbital - Moderate diffuse parenchymal loss involving the cerebral hemispheres bilaterally.
cutz This is frequent intracranial manifestation of systemic lupus erythematous or maybe
09/18/2017 a consequence of a prior episode of lupus cerebritis

- There is a focal cortical encephalomacia involving the left parietal lobe, which is
probably a residua from a prior inflammatory or vascular injury.

- Small nonspecific signal abnormalities involving the subcortical white matter


bilaterally. There is no other focal brain parenchymal signal abnormality.

- MRI of the orbits demonstrate abnormal enhancement involving the left optic
nerve, consistent with left optic neuritis. There also appears to be mild abnormal
enhancement of the intraorbital segment of the right optic nerve. These are of
uncertain etiology.
KUB Ultrasound -Negative ultrasound study of both kidneys and urinary bladder
09/20/17
OPHTHALMOLOGY NOTES
Date S O Plan

20 (+) MRI • Started on: Oral or IV Steroids


SEPT Result (1mg/kg/body weight) then taper
17 every 5 days by 5mg
• For referral to Neuro-Ophtha Service
on 27 Sept 17 at 1030H
OPHTHALMOLOGY NOTES
Date S O Plan

25 • OU: cannot be assessed • Maintain on prescribed oral steroids


SEPT due to patient difficulty • Ff-up at Ophtha ward on 27 Sept 17
17 verbalizing for Neuro Ophtha Service
• Pupils: 4-5mm RTL
• Fundus: (+ROR), clear
media, DDB, AVR 2:3,
CDR 0.2, Good foveal
reflex

DX:
• Optic Neuritis, resolving
OPHTHALMOLOGY NOTES
Date S O Plan

27 DX: • Maintain on 20mg Predisone per day


SEPT • Bilateral Optic Neuritis or at least 2 weeks, then taper
17 • Ff-up at Ophtha ward on 04 October
17, 1030H
Date S O p

4 Oct (-) fever (-) cough For possible Renal Biopsy on 11 October 2017 @
2017 (-) weakmess (-) colds PCMC
(-) decreased (-) seizure For possible transfer to PCMC on 10 Oct 2017
vision FOR BT: BT A+
(+) Good
appetite
(+) Good
Activity
OPHTHALMOLOGY NOTES
Date S O Plan

04 DX: • Keep patient on 20mg Predisone per


OCT • Lupus Nephritis; day until 1 week after 1st cycle of
17 • Optic Neuritis, Bilateral; cyclophosphamide
• Hypertensive • May taper Prednisone to 15mg/day 1
encephalopathy; week after starting
• S/p 1st cycle cyclophosphomide
cyclophosphamide • To follow: 5mg taper weekly until
5mg Prednisone per day
09 Oct 09 OCT
Hgb 114
Glucose, FBS 3.61
Hct 0.34
Crea 598.90
RBC 3.73
WBC 17.3 BUN 8.20

Seg 0.69
Lymp 0.22
Eo 0.01
Mono 0.07
Baso 0.01
Plt 289
Systemic Lupus
Erythematosus
Systemic Lupus Erythematosus
• chronic autoimmune disease characterized by
• multisystem inflammation
• presence of circulating autoantibodies directed against self-antigens
• occurs in both children and adults
• disproportionately affecting females of reproductive age
• most commonly involved organs: skin, joints, kidneys, blood-forming
cells, blood vessels, and the central nervous system
• Children and adolescents  more severe disease and more widespread
organ involvement
Etiology
• Factors influencing risk and severity: genetics, hormonal milieu and
environmental exposures
• Specific Genetic Abnormalities: congenital deficiencies of C1q, C2, and C4, as
well as several polymorphisms, familial clustering of SLE or other autoimmune
disease, and human leukocyte antigen (including HLA-B8, HLA-DR2, and HLA-
DR3)
• Hormonal Factors: preferentially affects females during the reproductive
years; Estrogen exposure promotes B-cell autoreactivity
Epidemiology
• Prevalence of SLE
• children and adolescents 1-6/100,000
• Adults 20-70/100,000
• highest among African-Americans, Asians, Hispanics, Native
Americans, and Pacific Islanders
• Female:Male ratio
• Prior to puberty 2-5:1
• during reproductive years 9:1
Clinical Manifestations
• Any organ system can be involved
• Most common presenting complaints of children  fever, fatigue,
hematologic abnormalities, arthralgia, and arthritis
• Course: periods of flare and disease quiesence or more smoldering
course
Diagnosis
• Presence of 4 of the 11 American College of Rheumatology (ACR)
1997 Revised Classification Criteria for SLE
• MUSCULOSKELETAL MANIFESTATIONS
• Intermittent polyarthritis
• characterized by soft tissue swelling and tenderness in joints, most commonly in hands, wrists, and
knees
• CUTANEOUS MANIFESTATIONS
• Lupus dermatitis can be classified as:
• Discoid lupus erythematosus(DLE)
• Systemic rash
• Subacute cutaneous lupus erythematosus (SCLE), or
• Other.
• Discoid lesions
• roughly circular with slightly raised, scaly hyperpigmented erythematous rims and depigmented,
atrophic centers in which all dermal appendages are permanently destroyed.
• Malar Rash
• butterfly rash, one of the most common signs.
• Fixed erythema over the malar eminence, usually spares the nasolabial folds.
• RENAL MANIFESTATIONS
• Nephritis
• Most serious manifestation of SLE
• Classification of lupus nephritis is primarily histologic
• Patients with dangerous proliferative forms of glomerular damage (ISN III and IV)
usually have microscopic hematuria and proteinuria (>500 mg per 24 h)
• One-half develop nephrotic syndrome, and most develop hypertension
• NERVOUS SYSTEM MANIFESTATIONS
• Cognitive dysfunction  most common manifestation of diffuse CNS lupus
• Difficulties with memory and reasoning.
• Headaches
• Seizures
• Psychosis
• VASCULAR OCCLUSIONS
• Prevalence of transient ischemic attacks, strokes, and myocardial infarctions is
increased in patients with SLE

• PULMONARY MANIFESTATIONS
• Pleuritis with or without pleural effusion
• most common pulmonary manifestation
• Life-threatening pulmonary manifestations include
• Interstitial inflammation leading to fibrosis
• Shrinking lung syndrome, and
• Intra-alveolar hemorrhage
• CARDIAC MANIFESTATIONS
• Pericarditis
• most frequent cardiac manifestation;
• More serious cardiac manifestations
• Myocarditis
• Fibrinous endocarditis of Libman-Sacks.

• HEMATOLOGIC MANIFESTATIONS
• Anemia (usually normochromic normocytic)
• most frequent hematologic manifestation
• Leukopenia
• also common
• almost always consists of lymphopenia
• Thrombocytopenia
• may be a recurring problem
• GASTROINTESTINAL MANIFESTATIONS
• Nausea, vomiting, diarrhea
• Diffuse abdominal pain
• caused by autoimmune peritonitis and/or intestinal vasculitis.
• Vasculitis involving the intestine
• Perforations, ischemia, bleeding, and sepsis are frequent complications

• OCULAR MANIFESTATIONS
• About 1/3 of patients with SLE have ocular manifestations
• Sicca syndrome (Sjogren’s syndrome) and nonspecific conjunctivitis
• common in SLE and rarely threaten vision
• Retinal vasculitis and Optic neuritis
• serious manifestations:
• blindness can develop over days to weeks
Etiology:

•The fundamental defect in SLE is a


failure of the mechanism that
maintain self-tolerance.
Etiology:

A. Genetic factors
a. Certain HLA associations are more common in people with SLE than in the
general
b.population (e.g., HLA-A1, HLA-DR3).

c. Inherited deficiencies of certain complement components increase


the risk for developing SLE (e.g., C2 deficiency).
e. As many as 20% of clinically
unaffected first-degree
d. Family members of patients
relatives of SLE patients reveal
have an increased risk of
autoantibodies and other
developing SLE.
immunoregulatory
abnormalities.

Etiology:
Etiology:
• Immunologic Factors
• Recent studies in animal models and patients
have revealed several immunologic aberrations
that collectively may result in the persistence
and uncontrolled activation of self-reactive
lymphocytes
Etiology:

•Failure of self-tolerance in B cells


•CD4+ helper t cells
•TLR engagement by nuclear DNA and RNA
•Type 1 interferons
Etiology:
• Environmental triggers are important in exacerbating SLE or
triggering its initial onset; examples include:

• (1) Infectious agents (EBV)


• (2) Ultraviolet light (see earlier)
• (3) Estrogen (see earlier)
• (4) Medications (e.g., procainamide, hydralazine
Pathogenesis:
• The hallmark of sle is the generation of
autoantibodies directed against self-antigens.
01 02 03
(1) ICs (e.g., DNA–anti- (2) Autoantibodies are •Antipholpholipidantibod
DNA; type III HSR) are important in the y syndrome
most important in pathogenesis of
producing inflammation variouscytopeniasinvolvin
in the skin, g RBCs, neutrophils,
glomeruli/tubules, joints, lymphocytes, and
and small vessels. platelets.

Mechanisms of injury
MANAGEMENT
Treatment for SLE

• Conservative Management of life-threatening disease:


• Patients with fatigue, pain, and auto-antibodies of SLE, but without major
organ involvement
• Suppression of symptoms
• NSAIDs are useful for analgesia/anti-inflammatory
Treatment for SLE
• Proliferative forms of Lupus Nephritis
• The mainstay of treatment for organ-threatening manifestations of SLE is
• systemic glucocorticoids 0.5 – 1 mg/kg/day PO or
• Methylprednisolone sodium succinate IV x days followed by daily Prednisone
Treatment for SLE
• Retinal vasculitis and optic neuritis are serious ocular manifestations
of SLE; blindness can develop over days to weeks

• Aggressive immunosuppression is recommended


Treatment for SLE
• Therapeutics given to the patient:
• Prednisone 200mg/tab 2 tabs OD
• Mainstay treatment for SLE
• Immunosuppressant
• Hydroxychloroquine sulfate 200mg/tab OD
• To treat symptoms of rheumatoid arthritis and discoid or systemic lupus erythematosus
Treatment for SLE
• Erythropoeitin 2,000 IU
• In SLE, inflammatory cytokines inhibit erythropoietin production
• Cyclophosphamide 455 in 100cc D5W
• The gold standard treatment for severe organ threatening systemic lupus erythematosus
(SLE), especially renal and central nervous system lupus.