BACKGROUND: Apremilast is a phosphodiesterase 4 inhibitor used in psoriasis and psoriatic arthritis Recently, this treatment has received marketing authorization for severe and refractory oral aphthosis in Behçet\'s disease. Idiopathic recurrent aphthous stomatitis (RAS) outside of Behçet\'s disease is often difficult to treat and can affect patients\' quality of life.
METHODS: We report our experience with the use of apremilast in two 49-year-old patients with refractory oral aphthosis not related to Behçet\'s disease or another etiology. In both cases, a combination of apremilast with low-dose prednisone controlled the idiopathic RAS within a few weeks.
CONCLUSIONS: Apremilast, which already has marketing authorization for Behçet\'s disease, also appears to be effective in RAS.

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is a rare and generally self-limiting disease in children.
METHODS: A descriptive cross-sectional study was performed in children under 18 years diagnosed with AIHA from January/1997 to July/2019. Clinical variables were collected and AIHA was classified according to the direct antiglobulin test (DAT) in warm AIHA (IgG+/-C3d) and cold AIHA (C3d). Response to treatment and evolution were analyzed.
RESULTS: 25 patients were included and 72% were males. The median age at diagnosis was 2 years (range 0.4 to 9). Fever (72%), pallor (68%), jaundice (64%), hepatosplenomegaly and coluria (48%) were the most common presenting symptoms. The median hemoglobin at diagnosis was 5.4 g/dl. DAT was positive in 96%, with detection of IgG antibodies in 76%. A single case presented negative DAT. 20% of the patients associated another cytopenia, one of which was subsequently diagnosed with common variable immunodeficiency. Concomitant viral infection was suspected or documented in 32%. Most of the cases were self-limiting and responded to corticosteroid treatment (72%). Those with partial response (24%), mainly those associated with other cytopenias, required other lines of treatment (rituximab, mycophenolate, immunoglobulins). Complications (32%) and relapses (26%) were detected only in warm AIHA.
CONCLUSIONS: Our case series confirms that AIHA is a very rare disease in childhood. Most cases evolve favorably, although up to a quarter of them require second lines of treatment and, in exceptional cases, they need very aggressive treatments. These latter cases generally correspond to patients who present more than one cytopenia in the course of the disease.

BACKGROUND: The clinical benefits of adjunctive corticosteroids for Pneumocystis jirovecii (P. jirovecii) pneumonia in patients not infected with the human immunodeficiency virus (HIV) has not been evaluated by meta-analysis.
METHODS: We conducted a systematic review of published studies describing the effects of adjunctive corticosteroids on outcome in non-HIV P. jirovecii pneumonia patients. Two investigators independently searched the PubMed and Cochrane databases for eligible articles written in English. A meta-analysis was performed using a random-effects model for measuring mortality as the primary outcome, and the need for intubation or mechanical ventilation as the secondary outcome.
RESULTS: Seven observational studies were eligible. In these studies, adjunctive corticosteroids did not affect mortality in non-HIV patients (odds ratio [OR] 1.26; 95% CI 0.60-2.67) and there was no beneficial effect in patients with severe hypoxemia (PaO2<70mmHg) (OR 0.90; 95% CI 0.44-1.83). No significant effect on the secondary outcome was observed (OR 1.34; 95% CI 0.44-4.11).
CONCLUSIONS: Although the studies were observational, meta-analysis showed that adjunctive corticosteroids did not improve the outcome of P. jirovecii pneumonia in non-HIV patients. The results warrant a randomized controlled trial.