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Background Juvenile dermatomyositis (JDM) is the most frequent idiopathic inflammatory myopathy, characterized by vasculopathy of the skin and/or muscles causing symmetrical proximal weakness and typical skin rashes. In 1975 Bohan and Peter published a classification for dermatomyositis: a certain diagnosis can be obtained with the presence of typical exanthema and at least 3 of the following signs or symptoms: proximal symmetric muscle weakness, elevation of creatine kinase (CK), muscle biopsy and electroneuromyography. Objectives To suggest it is useful to follow less specific muscle enzymes, such as aldolase, in JDM and normal CPK levels. Methods We reviewed 22 patients (8 males and 14 females, median age 8,2 years), with the diagnosis of JDG based on Bohan and Peter criteria. Skin signs preceded muscle manifestations in 7 patients (33%). The diseases was monophasic in 12 (57%) and polyphasic in 9 (43%) patients; one patient was lost at follow up; one patient died of cerebral hemorragia and another patient died of macrophagic activation syndrome. In all patient muscle enzymes were dosed: CK, aspartate amynotraspherase (AST), lactic dehydrogenase (LDH) and aldolase. Results 5/22 (23%, 2 males and 3 females) patients had normal CK: at the beginning 2 of them presented muscle sympthoms (weakness, myalgia, walking difficulty) and cutaneous signs; 3 presented only cutaneous signs. In 3 of 4 patients was observed isolated alteration of aldolase and one of them was affected by a severe hepatic disease with cholestasis (elevated gamma glutamyl transferase). In 1 of them JDM was diagnosed on the basis of cutaneous and muscle signs and symptoms, elevated aldolase ad muscle alteration on magnetic resonance (negative muscle biopsy and electroneuromyography). From literature we know that in 5-10% cases of JDM can be found normal levels of CK. In our cohort 20% of patients with early muscle symptoms has normal CK levels and in 10% normal muscle enzymes. Conclusions The use of aldolase compared with CK in muscle disorders is debated: aldolase can be altered in different diseases, such as erythrocyte diseases or hepatic disorders. CK can be negative because of the presence of inhibitors released by damaged muscle cells, muscle atrophy in advanced stages, concomitant hepatopathy or connective tissue diseases, steroid therapy or elevated acid uric and cystine levels that interfere with enzyme dosage. In suspicious cases of JDM it is useful to combine different enzymes to increase the diagnostic sensibility of this tests, associated with instrumental investigations if one them results negative. References Carter JD, Kanik KS, Vasey FB. Dermatomyositis with Normal Creatine Kinase and Elevated Aldolase Levels. J Rheumatol 2001;28;2366-2367 Gowdie PJ, Allen RC, Kornberg AJ, Akikusa JD. Clinical features and disease course of patients with juvenile dermatomyositis. Int J Rheum Dis. 2013 Oct;16(5):561-7. doi: 10.1111/1756-185X.12107. Epub 2013 Jun 3. Disclosure of Interest None declared

Background PLE has been seldom associated to SLE. Even more rarely, PLE has been described in infancy, as initial and unique clinical presentation of SLE. Methods Clinical case: VM an adopted 10-year-old Vietnamese child, was first admitted to a hospital for sudden generalized oedema at 8 years of age. On admission, hypoalbuminaemia, hypocomplementaemia and a 24-h urinary protein quantification of 610 mg were noticed. For a nephrotic syndrome suspicion, he received daily oral prednisone for 1 month, followed by 4 months of steroid tapering, with complete recovery. One year later, he was again admitted to a hospital for sudden generalized oedema. Laboratory studies showed hypoalbuminaemia, hypocomplementaemia and a 24-h urinary protein quantification of 346 mg. ANAs and dsDNA antibodies were highly positive; serum IgG were elevated too. Because of the suspicion of a nephrotic syndrome relapse, he received a new course of daily oral prednisone for 3 months, followed again by a complete normalization of albumin blood values. After a prednisone free period of 9 months, the patient was admitted to our unit for a third episode of sudden generalized oedema. At clinical examination a conspicuous limbs and periorbital oedema was present. The blood albumin was so low to be undetectable. Again, liver and kidney function were normal, such as 24-h urinary protein. ANAs were positive with speckled appearance, while dsDNA antibodies were absent. Complement was normal. Fecal alpha-1 antitrypsin was of 7.21 mg/g (normal range 0.38-0.5). The Tc-labelled human serum albumin scintigraphy confirmed a severe PLE, mostly deriving from the duodenojejunal area. Gastrointestinal endoscopies showed gastric hypertrophic folds with mucosal nodularity, and lymphoid nodular hyperplasia to the distal ileum. Biopsies revealed a gastric mucosal chronic inflammation with foveolar hyperplasia and microscopic lymphangiectasias. The patient was first started to a nutritional replacement using high-protein diet and medium-chain triglycerides, without a significant improvement of serum albumin. The diagnosis of LUPLE (LUpus Protein Losing Enteropathy) was suspected. Treatment with alternate days i.v. methylprednisolone (20 mg/kg/day) was commenced, followed after one week by daily oral prednisone (1 mg/kg/day) combined to daily oral azathioprine (2 mg/kg/day), with a progressive improvement in albumin blood values. Conclusions PLE is a rare manifestation of autoimmune diseases, including SLE, but the underlying autoimmune disorder is not necessarily apparent at the time of onset of PLE. This case highlights the need of considering PLE as possible etiology of acute hypoalbuminaemia in apparently otherwise healthy children, even without any gastrointestinal symptom. In addition, it is a rare example of PLE as initial and unique clinical presentation of an autoimmune process in childhood. References Al-Mogairen SM. Lupus protein-losing enteropathy (LUPLE): a systematic review. Rheumatol Int 2011;31:995-1001. Awazawa R, Yamamoto Y, Mine Y et al. Systemic lupus erythematosus complicated with protein-losing enteropathy: a case report and review of the published works. J Dermatol 2012;39:454-61. Disclosure of Interest None declared