Deficiencia de IgA y comorbilidades autoinmunes en la artritis idiopática juvenil / IgA deficiency and autoimmune comorbidities in juvenile idiopathic arthritis

Objetivos: 1) Describir la prevalencia de deficiencia de IgA (DIgA), uveítis, enfermedad celiaca (EC) y alteraciones tiroideas en una cohorte multicéntrica de pacientes diagnosticados de artritis idiopática juvenil (AIJ), y 2) evaluar si los pacientes con AIJ y DIgA presentan otras enfermedades autoinmunes con más frecuencia que los pacientes con niveles normales de IgA. Métodos: Estudio retrospectivo de una cohorte de pacientes con AIJ en seguimiento en unidades de Reumatología pediátrica en 2 hospitales de Madrid (España). Resultados: Se incluyó a 193 pacientes, de los cuales 123 eran mujeres (64%). La edad media al inicio fue 5,6 años (RIC 2,5-9,7) y la mediana de seguimiento 5,1 años (RIC 2,2-8,1). Las 3 categorías ILAR más frecuentes fueron oligoarticular (53%), poliarticular con factor reumatoide negativo (20%) y artritis relacionada con entesitis (10%). Los niveles séricos de IgA estaban disponibles en 172/193 (89%); 25/172 (15%) tenían DIgA, selectiva (< 7mg/dl, n=8) o parcial (7-69mg/dl, n=17). Todos los pacientes tuvieron revisiones oftalmológicas periódicas. Tuvieron uveítis anterior 18 pacientes (9%), 15/18 crónica y 3/18 aguda. Los niveles séricos de antitransglutaminasa IgA (o IgG en pacientes con DIgA) fueron obtenidos en 135/193 (70%); 4 pacientes (3%) fueron diagnosticados de EC por biopsia (n=3) o por criterios clínicos, serológicos o genéticos (n=1); 2 de ellos tenían DIgA (p=0,12; OR=6,4; IC del 95%, 0,9-47,6). Solo 1/153 (0,7%) tuvo hipertirotropinemia con anticuerpos antitiroideos positivos y requirió tratamiento. Conclusión: Los pacientes con AIJ presentan comorbilidades autoinmunes con frecuencia. La DIgA no parece aumentar su prevalencia, con la posible excepción de la EC. (AU)

Objectives: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentre cohort of patients with juvenile idiopathic arthritis (JIA), and (2) to assess whether patients with JIA and IgAD have additional autoimmune disorders more frequently than patients with JIA and normal serum levels of IgA. Methods: Retrospective chart review of a cohort of patients with JIA managed in the paediatric rheumatology units of 2hospitals in Madrid, Spain. Results: This study included 193 patients, 123 (64%) female. The median age at disease onset was 5.6 years (IQR 2.5–9.7) and the median duration of followup was 5.1 years (IQR 2.2–8.1). The 3most common categories of JIA based on the ILAR classification were oligoarticular (53%), poliartritis RF-negative (20%) and enthesitis-related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All patients underwent periodic ophthalmic examinations. Eighteen children (9%) had anterior uveitis, which was chronic in 15 and acute in 3. Serum levels of anti-transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); 2of them had IgAD (OR=6.4; 95% CI, 0.9–47.6; p=.12). Only 1 of these 153 patients (0.7%) had hyperthyrotropinaemia with positive anti-thyroid antibodies and required replacement therapy. Conclusion: Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD. (AU)

IgA deficiency and autoimmune comorbidities in Juvenile Idiopathic Arthritis.

OBJECTIVES: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentric cohort of patients diagnosed with JIA and, (2) to evaluate whether patients with JIA and IgAD present other autoimmune diseases more frequently than patients with normal serum levels of IgA. METHODS: Retrospective chart review of a cohort of patients diagnosed with JIA followed at the paediatric rheumatology units of two hospitals in Madrid, Spain. RESULTS: A total of 193 patients were included. Of them, 123 were females (64%). Median age at disease onset was 5.6 years (IQR 2.5-9.7) and the median time of follow-up was 5.1 years (IQR 2.2-8.1). The three most common ILAR categories were oligoarticular (53%), polyarticular RF negative (20%) and enthesitis related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All the patients had periodic eye exams. Eighteen children (9%) had anterior uveitis, 15/18 chronic and 3/18 acute. Serum anti transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); two of them had IgAD (p=0.12; OR=6.4; 95% CI 0.9-47.6). Only 1/153 (0.7%) patient had hyperthyrotropinemia with positive anti-thyroid antibodies and required replacement therapy. CONCLUSION: Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD.

Hemorrhagic bullous IgA vasculitis (Schönlein-Henoch purpura), does it have a worse prognosis?

Haemorrhagic bullous form of IgA vasculitis (IgAV), or Schönlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients (<2%). Blistering eruptions can sometimes be very striking, leading to hospital admissions and administration of high-dose steroids and even immunosuppressants. Review of the literature, however, does not suggest that this clinical form carries a worse prognosis than the other forms of IgAV. In fact, the prognosis of the disease depends on the organic involvement. We present the case of a 5-year-old girl that is very representative. She developed palpable purpura and four days later the skin lesions evolved into blistering lesions. She did not receive any anti-inflammatory nor immunosuppressive treatment and the lesions spontaneously subsided within 14 days. She did not develop any extracutaneous nor systemic involvement.

Vasculitis IgA (púrpura de Schönlein-Henoch) hemorrágico-ampollosa, ¿tiene peor pronóstico? / Hemorrhagic Bullous IgA Vasculitis (Schönlein-Henoch purpura), Does it Have a Worse Prognosis?

La forma hemorrágico-ampollosa de la vasculitis IgA (VIgA) o púrpura de Schönlein-Henoch, es una de las presentaciones menos frecuentes de la enfermedad en pacientes pediátricos (<2%). Cursa con una afectación cutánea muy llamativa que, con frecuencia, motiva ingresos hospitalarios y tratamiento con corticoides a dosis elevadas, incluso con inmunosupresores. Sin embargo, la revisión de la literatura realizada no sugiere que su pronóstico sea distinto al de otras formas de VIgA, lo que sí parece es que depende de la afectación orgánica existente.Se presenta el caso de una niña de 5 años que resulta muy representativo. Fue diagnosticada de VIgA hemorrágico-ampollosa, desarrollando lesiones ampollosas 4 días después de la aparición de las lesiones purpúricas. En ningún momento precisó tratamiento antiinflamatorio ni inmunosupresor, resolviéndose las lesiones 14 días después, sin complicaciones significativas.(AU)

Haemorrhagic bullous form of IgA vasculitis (IgAV), or Schönlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients (<2%). Blistering eruptions can sometimes be very striking, leading to hospital admissions and administration of high-dose steroids and even immunosuppressants. Review of the literature, however, does not suggest that this clinical form carries a worse prognosis than the other forms of IgAV. In fact, the prognosis of the disease depends on the organic involvement.We present the case of a 5-year-old girl that is very representative. She developed palpable purpura and four days later the skin lesions evolved into blistering lesions. She did not receive any anti-inflammatory nor immunosuppressive treatment and the lesions spontaneously subsided within 14 days. She did not develop any extracutaneous nor systemic involvement.(AU)

MALT lymphoma and eosinophilic oesophagitis: incidental finding- review of possible factors influencing the aetiopathogenesis of eosinophilic oesophagitis.

An 11-year-old boy with a history of atopy and allergic rhinitis under treatment with sublingual immunotherapy was referred following several episodes of food impaction. Diagnosis of eosinophilic oesophagitis, chronic gastritis and gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated to Helicobacter pylori were confirmed. Results of the extension study were negative and the lymphoma was resolved with eradicating treatment for H. pylori No improvement was observed in the oesophagitis after the withdrawal of immunotherapy and treatment with high proton pump inhibitor doses or following the omission of several foods to which subclinical sensitisation was confirmed. Oesophagitis was finally resolved by removing cow's milk protein. After 10 years, neither eosinophilic oesophagitis nor MALT lymphoma was observed.Gastric MALT lymphoma associated to H. pylori is a rare disorder in children. Although coexisting H. pylori infection is common in patients with eosinophilic oesophagitis, the association of gastric MALT lymphoma with eosinophilic oesophagitis has not been reported before.

Hemorrhagic Bullous IgA Vasculitis (Schönlein-Henoch purpura), Does it Have a Worse Prognosis? / Vasculitis IgA (púrpura de Schönlein-Henoch) hemorrágico-ampollosa, ¿tiene peor pronóstico?

Haemorrhagic bullous form of IgA vasculitis (IgAV), or Schönlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients (<2%). Blistering eruptions can sometimes be very striking, leading to hospital admissions and administration of high-dose steroids and even immunosuppressants. Review of the literature, however, does not suggest that this clinical form carries a worse prognosis than the other forms of IgAV. In fact, the prognosis of the disease depends on the organic involvement. We present the case of a 5-year-old girl that is very representative. She developed palpable purpura and four days later the skin lesions evolved into blistering lesions. She did not receive any anti-inflammatory nor immunosuppressive treatment and the lesions spontaneously subsided within 14 days. She did not develop any extracutaneous nor systemic involvement.

Tricotilomanía de nueva aparición durante el tratamiento con fármacos estimulantes. A propósito de dos casos clínicos pediátricos / New-onset trichotillomania during treatment with stimulant drugs. About two pediatric clinical cases

Los fármacos estimulantes se usan, habitualmente, en la población pediátrica para tratar el trastorno por déficit de atención e hiperactividad, y sus efectos secundarios están bien descritos. Sin embargo, la tricotilomanía no aparece como uno de ellos. En la literatura, hay algunos casos publicados de tricotilomanía en relación con la administración de metilfenidato y dextroanfetamina. Se presentan dos casos de tricotilomanía de nueva aparición en niños en seguimiento en nuestro Centro por déficit de atención e hiperactividad y en tratamiento con fármacos psicoestimulantes (metilfenidato y lisdexanfetamina), como probable efecto adverso de estos.

Stimulant drugs are commonly used in pediatric population in the treatment of attention deficit hyperactivity disorder, and their side effects are well described, however trichotillomania does not appear as one of them. In the literature we found some published cases of trichotillomania in relation to methylphenidate and dextroamphetamine. We present two cases of new-onset trichotillomania in children followed up in our center by attention deficit hyperactivity disorder and treated with psychostimulant drugs (methylphenidate and lisdexamfetamine), as a probable adverse effect of this treatment

[New-onset trichotillomania during treatment with stimulant drugs. About two pediatric clinical cases]. / Tricotilomanía de nueva aparición durante el tratamiento con fármacos estimulantes. A propósito de dos casos clínicos pediátricos.

Stimulant drugs are commonly used in pediatric population in the treatment of attention deficit hyperactivity disorder, and their side effects are well described, however trichotillomania does not appear as one of them. In the literature we found some published cases of trichotillomania in relation to methylphenidate and dextroamphetamine. We present two cases of new-onset trichotillomania in children followed up in our center by attention deficit hyperactivity disorder and treated with psychostimulant drugs (methylphenidate and lisdexamfetamine), as a probable adverse effect of this treatment.

Los fármacos estimulantes se usan, habitualmente, en la población pediátrica para tratar el trastorno por déficit de atención e hiperactividad, y sus efectos secundarios están bien descritos. Sin embargo, la tricotilomanía no aparece como uno de ellos. En la literatura, hay algunos casos publicados de tricotilomanía en relación con la administración de metilfenidato y dextroanfetamina. Se presentan dos casos de tricotilomanía de nueva aparición en niños en seguimiento en nuestro Centro por déficit de atención e hiperactividad y en tratamiento con fármacos psicoestimulantes (metilfenidato y lisdexanfetamina), como probable efecto adverso de estos.

Auscultación patológica persistente: no todo son broncoespasmos / Persistent pathological auscultation, not all is brochospasms

Los síntomas respiratorios constituyen un motivo de consulta frecuente en Atención Primaria y de derivación al especialista si se manifiestan crónicamente. Describimos dos casos tratados por asma y neumonía recurrente en los que se planteó el diagnóstico diferencial por una auscultación patológica persistente. El diagnóstico final fue: secuestro pulmonar y bronquiolitis obliterante. Se revisan los aspectos más relevantes de estas patologías graves y poco frecuentes

Respiratory symptoms are the most common reason for primary care visits and, when chronic, a frecuent indication for hospital specialist referrals. We describe two patients treated for asthma and recurrent pneumonia. A differential diagnosis was needed to find out the cause of persistent pathological auscultation. Final diagnosis was: Pulmonary sequestration and Bronchiolitis obliterans. We review the most relevant aspects of this uncommon and severe chronic lung diseases