Dr Anakha V Ajay,Dr Suma Balan(Paed Rheumat)DrC Jayakumar
AIMS, Kochi.Kerala ,India
Ten year old female child presented with bilateral elbow and ankle joint pain associated with swelling over bilateral feet of one week duration .Subsequently she developed erythematous rashes over both lower limbs of 4 days duration.
History was negative for fever, reccurent oral ulcers,haematuria, shortness of breath.
With the above symptoms she was evaluated at a local hospital, which revealed nephrotic range proteinuria.
She was initiated on Naproxen after which joint pain and swelling subsided and was referred to AIMS for further management. Past history of rashes bilaterally over knees which was treated as pyoderma ,6 months back. Child was born as first to a non consanguineous couple, via normal delivery with a birth weight of 2.9kg, with uneventful post natal history. Family history was negative for any rheumatic disease.
On examination, child was conscious, oriented with BP-136/84mmHg(high) and other stable vitals. stable vitals.
All peripheral pulses felt.
Pallor was present along with bilateral pitting edema noted upto ankle..
paeditric Gait, Arms ,Legs And Spine (pGAL )was normal. Gait was normal. Systemic examination was within normal limits.
Labs
Hb-9.1g/dl), ESR(40mm/hr),
normal counts
CRP of 2.97 mg/L,
normal LFT,
low complements(C3-83, C4-2.7)
Creatinine level of 0.79.
Urine showed 2+ proteinuria,
Urine protein to creatinine ratio of 12.
Differentials
1.IgA Nephropathy
2. Systemic Lupus Erythematosus
3.Post streptococcal GN
Ophthalmology evaluation showed grade 2 hypertensive changes.
Hypertension was managed with oral Amlodipine.
Renal biopsy revealed diffuse proliferative lupus nephritis, specifically Class IV A, with activity index of 7/24 and a chronicity index of 0/12.
ANA test immunofluroscence revealed a 2+ fine speckled pattern, although antiphospholipid syndrome work up revealed negative results.
Consequently she was diagnosed with Systemic Lupus Erythematosus(SLE) with lupus nephritis.
She has received a pulse of IV methylprednisolone at a dose of 10mg/kg for 3 days, followed transition to an oral dose methyl pred of 2mg/kg with divided doses. Mycophenolate mofetil was added as a steroid sparing agent.
During the hospital stay, strict monitoring of fluid intake and output as well as BP measurements was done every 6 hours. She received antihypertensive medications and enalapril was added due to her proteinuria and hypertension. She was discharged and planned to keep umder regular follow up.
Systemic Lupus Erythematosus(SLE) is a systemic autoimmune disease with multisystemic involvement with unknown etiology.
However several genetic, immunological, endocrine and environmental factors play a role in the etiopathogenesis of SLE.
SLE predominantly affects women with female to male ratio of 9 to 1.
SLE tends to be more severe in children.
Constitutional symptoms such as fatigue,fever and weight loss may be present.
Arthralgia & arthritis may be present in 90% of patients and are often one of the earliest manifestations.
Most patients develop skin and mucocutaneous lesions, of which most common is malar rash i.e presents as erythema in a malar diustribution over cheeks and nose.
Other manifestations are cardiac,vascular, gastrointestinal, renal, thromboembolic, pulmonary, hematological & ophthalmological.
Diagnosis of SLE can be challenging, and no single clinical feature or lab abnormality can confirm SLE diagnosis.
SLE is diagnosed based on constellation of signs, symptoms and appropriate lab work.
Treatment aims at preventing organ damage and achieve remission.
The criteria for diagnosis of SLE is based on American College of rheumatology classification criteria for SLE. Patients should fulfil 4 or more of the below either at a time or at some point of time
Mentioned criterias:
1. Malar rash
2. Discoid rash
3. Photosensitivity
4. Oral ulcers
5. Arthritis
6. Serositis
7. Renal disorder(Persistent proteinuria>0.5g/dl or cellular casts)
8. Neurological disorder(Seizures/ Psychosis)
9. Hematological disorder (Hemolytic anemia/ Leukopenia/ Lymphopenia)
10. Immunological disorder
11. Antinuclear antibody
The choice of treatment is indicated oragn system/systems involved and severity of involvement and ranges from minimal treatment(NSAIDS, Antimalarials) to intensive treatments(cytotoxic drugs, corticosteroids). Despite advanacement in therapeutic options, SLE patients suffer from significant morbidity and carry a high mortality. Survival rates are 85-90% during first 10 years of life.
Poor prognostic factors
African American ethinicity,
Renal disease( especially diffuse proluferative GN),
Male sex,
Young age,
Hypertension,
Antiphospolipid syndrome (APLA)
High overall disease activity.
Take home message: Each case of SLE is unique, so individualised care plans are crucial.Regular follow ups with healthcare providers to adjust treatment is needed and monitor disease activity are essential for the best outcomes.