AB0921 JUVENILE-ONSET LUPUS WITH SECONDARY CMV-INDUCED MACROPHAGE ACTIVATION SYNDROME (CASE REPORT)
Authors: Hanan Abd EL-Lateef
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Juvenile-onset lupus (JSLE) is a complex illness with an aggressive course and higher chances of morbidities and mortality. JSLE is extremely rare before the age of 5 years. A sole disease criterion may precede long time before others,making diagnosis rather challenging <h3>Objectives</h3> Identifying JSLE and its subsequent complications <h3>Methods</h3> A three and half years old female patient presented with an established diagnosis of idiopathic thrombocytopenia (ITP) of one year duration.One month ago,the patient developed an acute onset of high grade persistent fever, bilateral knee swelling and significant weight loss. <h3>Results</h3> On physical examination, patient was emaciated and pale with multiple ecchymotic patches allover her body. Vital data showed normal blood pressure and apical heart rate ranges for age. Systemic examination revealed multiple oral ulcers, hepatosplenomegaly,bilateral knee arthritis and bilateral below knee painless pitting lower limb edema. Neurological examination was free. Laboratory evaluation showed a CBC with TLC 1.800/mm<sup>3</sup> NE 0.5/mm<sup>3</sup>, HB 7.9 gm/dl, PLT 79 x10^3/µL.ESR 120 mm/1<sup>st</sup> hr and CRP 96 mg/dl. Routine chemistry showed elevated liver enzymes ( ALT 137 IU/L &amp; AST 280 IU/L), LDH 960 U/L, serum albumin 2.5 g/dl, normal renal functions. The history of chronic ITP together with evolution of other autoimmune manifestations raised the suspicion of an underlying Connective tissue disorder. Immunological profile showed positive immune markers (ANA 1/320 speckled and positive Anti-DNA), positive lupus anticoagulant and anticardiolipin IgM,C3 was consumed(49 mg/dl). Secondary Macrophage activation syndrome (MAS) was suspected and markers revealed serum ferritin 30.000 ng/ml, fibrinogen 250 mg/dl,Triglycerides 350 mg/dl and soluble CD25 12.000U/ml. Bone marrow aspiration confirmed the diagnosis.Cytomegalovirus (CMV) IgM and PCR were significantly high.With regard to all the previous clinical and laboratory features,a CMV-induced secondary MAS in JSLE was confirmed.Patient was managed according to HLH-2004 treatment protocol ( pulsed methylprednisolone 30mg/kg/day,cyclosporine A 6mg/kg/day, IV etoposide 150mg/m<sup>2</sup>)and IVIG.Ganciclovir was also added (5mg/kg IV q12h x7 days). Patient showed marked clinical improvement together with significant diminution of the MAS laboratory markers to normal.Two weeks post discharge on oral dexamethasone and cyclosporine A, the patient showed a severe clinical and laboratory MAS relapse ( serum ferritin 50.000 ng/ml). The previous treatment protocol was restarted together with adding Rituximab 375mg/m<sup>2</sup> x 3 doses two weeks apart.Patient showed marked clinical and laboratory improvement and was maintained on full dose oral prednisone and oral Cyclosporine A. Tight gradual withdrawal of oral steroids was done with close clinical and laboratory follow up. <h3>Conclusion</h3> JSLE is a major diagnostic conundrum in pediatrics owing to its extremely variable clinical manifestations which can mimic many common pediatric conditions(e.g.malignant disease,other auto-inflammatory conditions,malignancy and any specific organ associated disease).Compared to adults JSLE has a more severe disease presentation.MAS is a life threatening condition that requires high index of suspicion and prompt management.In our case, controlling JSLE activity using Rituximab was significantly beneficial in arresting HLH evolution. <h3>References</h3> [1] Amol P.etal : Juvenile systemic lupus erythematosus: A diagnostic dilemma.J Nat Sci Biol Med. 2011 Jul-Dec; 2(2): 229–231. [2] Henter JI etal : diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer (2007) 48:124–31. <h3>Disclosure of Interests</h3> None declared