Dr Theresa Raju, Dr Suma Balan(Dept of Rheumatology), Dr C Jayakumar, Amrita Institute of Medical Sciences
Twelve year old female presented with a 6 month history of evening rise in temperature, initially treated symptomatically with anti pyretics. The fever was intermittent, low grade not associated with chills. The mother also noticed painless a neck swelling over the past year but with weight lost of 5kgs in 6 months and joint pain in small and large joints. Joint pain was more prominent in the evenings and affected various joints without early morning stiffness, redness or swellings
The child also had upper limb erythematous lesions 2 weeks prior which evolved into hyperpigmented macules with associated itching. There were no oral ulcers or Gastro intestinal symptoms. Lymph node was biopseied and reported as reactive lymphadenitis
Bone marrow studies showed 18% atypical cells. Hence, time refresh was made
At admission, the child was febrile. General examination showed palpable multiple bilateral cervical and inguinal lymph nodes Non tender and hyperpigmented rashes in bilateral upper limbs. Systemic examination was normal.
Labs: Hb: 8.3g/dl, T:4.51K/Ul,
RFT, LFT were normal. C3 was low(53), while C4 was normal(17.7). DCT was weak positive and ESR was markedly elevated(121mm/hr) while CRP was 19. Urine analysis showed 3+ proteinuria and urine protein creatinine ratio was7.69.
ANA 4+ fine speckled
Anti dsDNA titre, ANA blot, Antiphospholipid workup was sent. The child was started on Hydroxychloroquine. Marrow slides were rechecked and aspiration showed particulate fairly cellular marrows showing trilineage hematopoiesis, left shifted myeloid maturation, dyspoiesis and 6% blasts/hematogones.
Renal biopsy was consistent with focal proliferative and membranous lupus nephritis class IIIA plus V with acute tubulointerstitial nephritis and uncomplicated vascular deposits.
ECHO and USG Abdomen did were normal. She was given 3 days of pulsed IV Methylprednsiolone ,. later switched to oral prednisiolone . ANA blot showed 3+ dsDNA, nucleosomes and histones and 1+ PM Sc1100. Anti dsDNA titre was 189.
Antiphospholipid workup was triple positivewhich include Anticardiolipin, Anti Beta 2 glycoprotein and lupus anticoagulant. She was then started on Tab Mycophenolate mofetil, as a steroid sparing agent. As the anti-B2 GP1 IgM was positive with high titres, low dose aspirin was also started.
She had 2-3 febrile spikes towards the evening which was managed conservatively. At discharge, the patient is hemodynamically stable and symptomatically better
Discussion:
Antiphosopholipis antibody syndrome(APS) is an autoimmune disease mostly affecting women . In APS ,abnormal proteins can cause formation of clots in the veins and arteries. This clots can cause miscarriage, harm a fetus or lead to heart attacks, strokes or pulmonary embolism. In severe cases multiple organs can be affected. APS may be associated with SLE. The presence or absence of SLE might modify the clinical or serological expression. Apart from the clinical manifestations , APS patients with SLE more frequently display a clinical profile with arthralgia,arthritis ,autoimmune hemolytic anaemia, epilepsy and myocardial infraction
The management of patients with SLE and APS/Apl should include an accurate stratification of vascular risk factors. Low dose aspirin and hydroxychloroquine should be considered as primary prophylaxis. In high risk situation, such as surgery ,prolonged immobilization and puerperium the prophylaxis should be potentiated with low molecular weight heparin. The role of novel anticoagulants in APS patients is still to be clearly defined.
Take home message: The goal of the future is to improve the outcome of these patients means of early recognition and optimal preventive treatment.