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A young woman with lupus
1. CASE OF A WOMAN WITH
SYSTEMIC DISEASE
Dr ENIDA XHAFERI
2. CASE PRESANTATION
This is the case of a 30 years old
woman who developed a wide
range of clinical manifestations
and symptoms over a period of
12 months.
3. Chief complains on
admission
❖ Severe fatigue
❖ Symmetrical non erosive arthritis of the knee,
carpal and proximal inter phalangeal joints
❖ Myalgia and muscle weakness
❖ Photosensitivity, described as development of a
skin rash as an unusual reaction to sunlight
❖ Malar rash in the form of a flat fixed eritema
over malar eminencies. Macupapular lesions on
both arms
❖ Painful buccal ulcerations
❖ Diffuse hair loss
❖ Periungal erythema
❖ Headaches and mood changes
4. History
Patient explains that she reported her
problems to the family physician who
formed his own diagnose and referred
the case to the local rheumatologjist
for more specialized follow up.
He gave her also a short course of low
dose glucocorticoids, nonsteroidal
antiinflamatory steroids, calcium
preparations and vitamins to control
the immediate pathologic symptoms.
5. History
Patient states that in the beginning her
symptoms were mild which did not
prompt an immediate visit to the
specialist ( she occupied herself with
work and family instead…!).
She responded well to the GP therapy
and she felt better until recently, when
her health status has deteriorated
considerably and she has observed also
the development of edemas in her feet
and “redness” over her fingers. At this
moment she thought that it would be
wise to go to the hospital.
6. ADITIONAL INFORMATION
Patient does not have close family members
with known rheumatic or musculoskeletal
disorders, is married and has had one
natural vaginal delivery.
She has not been exposed to known toxins
lately, does not use tobacco, alcohol, or
illicit drugs, has not received any new
medications and has no drug allergies.
7. PHYSICAL EXAMINATION
Vital Signs and General Examination:
Blood pressure 150/100 mm Hg; heart rate 110
beats/minute; respiratory rate 18 breaths/minute; T
37.5° C, Normostenic individual. Patient feels
weakened and fatigued and sweats a lot.
Mental status: Frequent headaches, distressed
face, reduced concentration span and mild seizures.
She states that lately she has had frequent mood
swings and sleep disorders.
Respiratory System : Normal vesicular
respiration throughout, free lungs, no wheezing
Cardiovascular System: Heart rate and
rhythm was regular; normal S1 and S2 sounds with
no murmurs, audible rubs, or positional substernal
chest pain
Abdomen: Abdomen was soft, not tender, and
not distended, Blumberg (-)
8. Physical examination
Gastrointestinal System: Normal bowel
sounds, liver is palpated 2 cm under right
costovertebral angle, no spleen
enlargement. Patient relates that she
experiences frequent bouts of dispnea,
pyrosis and has other digestive disorders,
and weight loss.
Genital & Urinar System: Mild edema
on the feet and face. Pasternacki (+) on
both sides.
Mucocutaneal lesions : Malar rash,
photosensitivity, periungal eritema, oral
lesions (punched out painful ulcers),
presence of a reddish/cianotic eritematous
pattern on the surface of both arms.
Hair loss
9. Physical examination
♦ Musculoskeletal manifestations
Knee, radiocarpal, proximal
Inter phalangeal joints arthritic,
tender, mildly swollen, and painful
when pressed.
Morning stiffness (lasting
several minutes)
Mialgia
Back ache
15. A-P hands and L-L lumbar x-rays
Unremarkable outcome
16. Questions
What do you think would be the
diagnosis in this case?
What other examinations would you
request?
What kind of treatment would you select
(low dose therapy or aggressive
regimens with high dose glucocorticoids
and cytotoxics)
How would you monitor treatment
response?
17. Revised criteria for classification of
SLE
❖ Malar rash
❖ Discoid rash
❖ Photosensitivity
❖ Oral ulcers
❖ Arthritis
❖ Serositis
❖ Neurological disorders
❖ Immunologic disorders
❖ Renal disorders
(proteinuria >0.5g/d
or 3 + if
quantification non
performed or urine
cellular casts
❖ Hematological
disorders
❖ Anti nuclear antibody
Lupus diagnosis is sure when at least 4 of
these criteria are met
18. Results of the consultation
with the nephrologist
• Nephrotic syndrome in the terrain of LES present
(proteinuria, hematuria, hypoalbuminemia,
hyperlipidemia edema), NIH protocol to be
considered.
• Use ACE inhibitors for HTA and proteinuira.
• Renal biopsy indicated
• Recommended renoprotective treatment
• -Ramiprili 5 mg
1 tab per day
• -Dipyridamoli 75 mg
3x1 tab per day
• -Atorvastatini 20 mg
1 tab in the evening
• -Lasixi 40 mg
1 tab per day
19. Renal involvement WHO
Classification
Class I - Minimal mesangial lupus
nephritis
Class II - Mesangial proliferative lupus
nephritis
Class III - Focal lupus nephritis
Class IV - Diffuse lupus nephritis
Class V - Membranous lupus nephritis
Class VI -Advanced sclerosing lupus
Renal biopsy showed the presence of
Class IV G- A Lupus Nephritis
20. Treatment objectives for lupus nephritis
➢ Corticosteroid therapy should be instituted if the patient
has clinically significant renal disease. Use
immunosuppressive agents, particularly cyclophosphamide,
azathioprine, or mycophenolate mofetil, if the patient
has aggressive proliferative renal lesions, as they improve the
renal outcome. They can also be used if the patient has an
inadequate response or excessive sensitivity to
corticosteroids.
➢ Treat hypertension aggressively. Consider angiotensin-
converting enzyme (ACE) inhibitors or angiotensin II receptor
blockers (ARBs) if the patient has significant proteinuria
without significant renal insufficiency.
➢ Restrict fat intake or use lipid-lowering therapy such as
statins for hyperlipidemia secondary to nephrotic syndrome.
➢ Restrict protein intake if renal function is significantly
impaired.
➢ Administer calcium and vitamin D supplementations to
prevent osteoporosis if the patient is on long-term
corticosteroid therapy and consider adding a bisphosphonate.
➢ Avoid drugs that affect renal function, including
nonsteroidal anti-inflammatory drugs (NSAIDs), especially in
patients with elevated creatinine levels. Nonacetylated
salicylates can be used to safely treat inflammatory
symptoms in patients with renal disease.
➢ Patients with active lupus nephritis should avoid
pregnancy, as it may worsen their renal disease.
21. Proliferative glomerulonephritis
(DPGN) induction therapy protocols
-NIH protocol: monthly pulse CYC, 0.75 g/m2, for 6 months and
oral prednisone (PDN) 0.5 mg/kg per day for 4 weeks. PDN is
tapered every other week and than day until a maintenance
dose of 0.25 mg/kg every other day or the minimal dose required
to control disease activity.
- Euro-lupus nephritis protocol: 6 fortnightly CYC pulses at a
dose of 500 mg. Corticosteroids were administered as 3 iv
pulses of 750 mg of methylprednisolone (MP) followed by PDN
0.5 mg/kg/day for 4 weeks, tapered by 2.5 mg every 2 weeks to
a maintenance dose of 5-7.5 mg/day.
- Oral CYC protocols: CYC is administered at doses ranging
from 1 to 2 mg/kg/day for 3 to 12 months. The most recently
published protocol consisted in the administration of oral CYC
for 6-9 months with PDN 0.5-1 mg/kg/day for 6-8 weeks,
followed by tapering to a maintenance dose of 5-10 mg/day .
22. Medications used in this case
❖ - Induction phase
• Prednisone (PDN) 0.5 mg/kg per day (60 mg for
patients weighing less than 80 kg) for 4 weeks, than 50,40,30
mg for the consecutive months . A new tapering regimen
by 5 mg on alternate days is started at this point
until the minimal dose required to control disease
activity is reached.
• Cyclophosphamide Monthly pulse of 0.75
g/m2, for 6 months usually administered with
antiemetic agents and may be administered with
mesna.
❖Response within 3-6 months
❖Maintenance phase
• Azathioprine : 2-3 mg/kg/d PO single or divided
dose. Initial: 1 mg/kg/d; increase depending on
clinical and hematologic response and toxicity
23. Final comments
• Disease activity is checked through
measurement of proteinuria, complement
levels, anti ds-dna antibodies titer, and serum
creatinine levels. At the third month of
treatment the patient has developed some
minor treatment side effects but her biologic
indexes are improved.
