SP0109 Acute respiratory failure, maculo-papular rash, indurative edema of the extremities and cervical lymphadenopathy in a 6-week-old infant
Authors: C Birolo
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Case report</h3> A previously healthy 40-days-old male infant, from non-consanguineous parents from Morocco, presented in a peripheral hospital with fever for 48 hours, associated with rhinorrhea, mild diarrhea and progressive irritability during the last 24 hours. Initial laboratory studies revealed elevated acute phase reactants (CRP 116 mg/L), mild neutrophilia, elevated liver enzymes (AST 315, ALT 174 U/L), direct hyperbilirubinemia, discoagulopathy. Cerebral spinal fluid analysis and microbiology cultural workup resulted negative. A wide-spectrum antibiotic and antiviral therapy was initiated. On the 6th day of illness he developed diffuse maculo-papular rash, indurative edema of the extremities, right cervical lymphadenopathy and bilateral conjunctival injection. On the basis of a certain clinical diagnosis of Kawasaki disease and the infant was treated with IGIV 2 g/kg. An echocardiography performed prior to the IGIV infusion showed homogeneously dilated coronary arteries (left coronary artery 3.2 mm, right coronary artery 2.2 mm). Twelve hours after the end of IVIG infusion, the child presented a rapidly progressive, severe respiratory failure requiring endotracheal intubation and was transferred to our ICU. On admission (day 7th), physical examination revealed a feverish, critically ill-infant with hepatomegaly (5–6 cm below the right costal margin), diffuse maculo-papular rash, "sock-like" erythema and swelling of the feet, cheilitis, bilateral conjunctival injection and right cervical adenopathy. The urine output was markedly decreased; he rapidly developed hemodynamic instability with hypotension and tachycardia. Complete blood count showed anemia (6.8 g/dl), thrombocytopenia (16.000/mmc), elevated CRP (240 mg/L), hypoalbuminemia (18 g/dl) and hypofibrinogenemia (0.83 g/L); liver enzymes were normal. Intensive ventilatory and hemodynamic support therapy were started, in addition to a massive transfusional regimen. Given the clinical and hematological picture, the diagnosis of MAS was considered and subsequently confirmed by high ferritin level (2197 mcg/L), AST above the normal value (61 U/L) and hypertriglyceridemia (181 mg/dl) [2]. The clinical suspicion was supported by persistent cytopenia despite daily transfusions, low erythrocyte sedimentation rate (3 mm/h) with concomitant rising CRP, elevated IL2-R level (28.320 KU/L) and decreased NK function. The patient was treated with high dose methylprednisolone pulse therapy (25 mg/kg) for 3 consecutive days 12–14), followed by a maintenance of 1 mg/kg/daily. By day 15th, a progressive decrease in inflammatory markers and a concomitant improvement of general conditions was observed, with the possibility to discontinue inotropic support on day 12th and invasive ventilation on day 25th. Since day 17th, a diffuse cutaneous desquamation was noted. The fever settled on day 35th. Echocardiography follow-up revealed an increasing, irregular dilation of left (max 5 mm) and right (max 3.5 mm) coronary arteries, with a progressive left ventricular apex hypocinesis, but a stable ejection fraction (55%). ECG showed persistent repolarization abnormalities. Of note, Adenovirus-PCR was found positive in the bronchoalveolar washing performed on admission. The differential diagnosis included: Kawasaki disease complicated by respiratory distress syndrome and MAS, familiar hemophagocytic lymphohistiocytosis, autoimmune lymphoproliferative syndrome, criopyrinopathies and immunodeficiency. <h3>Comment</h3> Kawasaki disease can be catastrophic in the early infancy, due to atypical presentation and resistance to the conventional therapy. Despite typical in its presentation, this case was complicated by two challenging conditions, respiratory distress syndrome and MAS, which hampered the diagnostic and therapeutic management during the course of the disease and required massive intensive support.These two complication are rarely associated to KD. In this particular case, respiratory failure can probably be explained by a combination of causes: fluid overload, systemic vasculitis and the concomitant and probably triggering Adenovirus infection. <h3>Disclosure of Interest</h3> None declared