Antiphospholipid syndrome (APS) describes patients with antibodies targeting phospholipid molecules causing recurrent arteriovenous thromboses, fetal losses, thrombocytopenia along with antiphospholipid antibodies viz. lupus anticoagulant and the anticardiolipin antibodies [1]. It can be either primary, or secondary that is triggered by infections or malignancies [2]. Catastrophic APS (CAPS), also known as Asherson syndrome, is a rare accelerated variant characterized by the rapid appearance/progression of more than three organ thromboses, with microangiopathy leading to multiorgan failure. We describe a child with pulmonary tuberculosis triggering CAPS.

A 14-year-old girl presented with increasing pyrexia and cough for 3 weeks. Mild left upper/middle zone crepitations were present with no respiratory distress. Investigations on admission revealed anemia (hemoglobin 8.5 g/dL), leucocytosis (leucocyte count 13×109/L, polymorphs 85%), thrombocyto-penia (platelet count 45×109/L), with mildly elevated C reactive protein (10 mg/dL) and erythrocyte sedimentation rate (ESR 40 mm/h). Blood cultures were sterile. With an initial chest roentgenogram revealing pneumonitis, she was commenced on antibiotics (amoxicillin and clavulanic acid) with no response. Worsening in the productive cough with persistent fever, despite 7 days of antibiotics, prompted a computed tomo-graphy (CT) scan, which revealed a left upper consolidation with cavitatory changes and necrotic mediastinal lymphadenopathy. Sputum tested positive for Mycobacterium tuberculosis and anti-tubercular therapy (ATT) was commenced.

While on ATT, she developed severe bifrontal headache, vomiting and worsening respiratory distress. Magnetic resonance imaging of brain was performed, which was suggestive of thrombosis of superior sagittal venous sinus, right transverse sinus and sigmoid sinus with thrombus extending to proximal internal jugular vein. Repeat investigations revealed anemia, falling leucocyte counts (5.8×109/L), thrombo-cytopenia (platelet count 60×109/L), increasing CRP (107mg/dL), high ESR (120 mm/h) and transaminitis. Activated partial thromboplastin time (aPTT) was prolonged. Thrombophilia work-up was suggestive of antiphospholipid antibody (APLA) positivity [Lupus anticoagulant positive; Prolonged Russel viper venom time (RVVT), 78.5 (control- 48.3) seconds; anti-cardiolipin (ACL) IgG, 15 U/mL (normal range 0-12.5 GPL U/mL); Beta 2 glycoprotein 1 IgG, 30 U/mL (control 12 U/mL)]. Protein C, Protein S, anti-thrombin levels were unremarkable. Hyperfibrinogenemia (450 mg/dL), hypocomplementemia (C3, 80.8mg/dL and C4 9.2 mg/dL), positive direct coombs test, elevated serum lactate dehydrogenase (600 U/L), high serum ferritin (800 ng/mL) and significant proteinuria (spot urine protein creatinine ratio 1.2) were other positive findings. Peripheral smear ruled out schistocytes. Antinuclear antibody (ANA), Rheumatoid factor and anti-neutrophil cytoplasmic antibodies (ANCA) were negative. A CT angiogram revealed bilateral segmental pulmonary thromboembolism, ground glass changes and diffuse alveolar hemorrhage (DAH) with multiple splenic infarcts. With imaging and laboratory evidence of progressive multiorgan dysfunction, a diagnosis of CAPS triggered by pulmonary tuberculosis was made.

Child was started on parenteral methyl prednisolone followed by oral steroids and anticoagulation with low molecular weight heparin, while continuing ATT. Fever and cough resolved over the next 2 and 4 weeks, respectively. Steroids were tapered over 3 months. Repeat APLA work-up after 12 weeks confirmed APLA positivity. Anticoagulation was continued for 6 months. APLA tested negative at 6 months ruling out primary APS and establishing tuberculosis as the etiology. Repeat neuroimaging at 6 months showed significant resolution of the thrombus.

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