Acute pneumonitis in a patient with adult-onset disease after toclizumab treatment with good response to anakinra. / Neumonitis aguda en un paciente con enfermedad de Still del adulto tras tratamiento con tocilizumab con buena respuesta a anakinra.

Pulmonary involvement in the form of acute pneumonitis in adult-onset Still's disease (AOSD) is an uncommon manifestation, with few cases reported in the literature. We report the case of a 61-year-old male with 3 years of AOSD evolution, treated with methotrexate (MTX) and half-dose corticosteroids, which debuted with symptoms of fever, dyspnea and dry cough after 3 weeks of receiving the first dose of tocilizumab (TCZ). In the follow-up study showed leukocytosis with left shift, elevated serum ferritin and C-reactive protein standard. The chest CT scan showed ground-glass pattern predominantly in central and upper lobes and the BAL shows an increase in the percentage of lymphocyte with normal subpopulations and negative cultures. MTX and TCM were suspended, prednisone was increased to 30mg/day and within a week Anakinra 100mg/day SC was iniciated, noting in a few days a progressive clinical, analytical and radiological improvement.

Bone health, vitamin D and lupus.

The prevalence of vitamin D deficiency and insufficiency among patients with systemic lupus erythematosus is high. This is likely due to photoprotection measures in addition to intrinsic factors of the disease. Low levels of vitamin D increase the risk of low bone mineral density and fracture. Vitamin D deficiency could also have undesirable effects on patients' immune response, enhancing mechanisms of loss of tolerance and autoimmunity. Vitamin D levels should be periodically monitored and patients should be treated with the objective of reaching vitamin D levels higher than 30-40 ng/ml.

Dermatomyositis and myastenia gravis: An uncommon association with therapeutic implications.

The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed.

Neumonitis aguda en un paciente con enfermedad de Still del adulto tras tratamiento con tocilizumab con buena respuesta a anakinra / Acute pneumonitis in a patient with adult-onset disease after toclizumab treatment with good response to anakinra

La afectación pulmonar en forma de neumonitis aguda en la enfermedad de Still del adulto (ESA) es una manifestación infrecuente, existiendo pocos casos descritos en la literatura. Presentamos el caso de un varón de 61 años con ESA de 3 años de evolución, en tratamiento con metotrexato (MTX) y corticoides a dosis medias, que comenzó con cuadro de fiebre, disnea y tos seca a las 3 semanas de recibir la primera dosis de tocilizumab (TCZ). En la analítica destacaban leucocitosis con desviación izquierda, ferritina sérica muy elevada y proteína C reactiva normal. La TAC de tórax mostró un patrón en vidrio deslustrado de predominio central y lóbulos superiores, y el BAL, un incremento del porcentaje de linfocitos, con subpoblaciones normales y cultivos negativos. Se suspendieron el MTX y el TCZ, se aumentó la prednisona a 30mg/día y al cabo de una semana se inició anakinra 100mg/día por vía subcutánea, observando en pocos días una mejoría progresiva espectacular clínica, analítica y radiológica (AU)

Pulmonary involvement in the form of acute pneumonitis in adult-onset Still's disease (AOSD) is an uncommon manifestation, with few cases reported in the literature. We report the case of a 61-year-old male with 3 years of AOSD evolution, treated with methotrexate (MTX) and half-dose corticosteroids, which debuted with symptoms of fever, dyspnea and dry cough after 3 weeks of receiving the first dose of tocilizumab (TCZ). In the follow-up study showed leukocytosis with left shift, elevated serum ferritin and C-reactive protein standard. The chest CT scan showed ground-glass pattern predominantly in central and upper lobes and the BAL shows an increase in the percentage of lymphocyte with normal subpopulations and negative cultures. MTX and TCM were suspended, prednisone was increased to 30mg/day and within a week Anakinra 100mg/day SC was iniciated, noting in a few days a progressive clinical, analytical and radiological improvement (AU)

Salud ósea, vitamina D y lupus / Bone health, vitamin D and lupus

Los pacientes con lupus eritematoso sistémico presentan una prevalencia elevada de deficiencia e insuficiencia de vitamina D. Esto se debe probablemente a las medidas de fotoprotección y a factores intrínsecos de la enfermedad. Los niveles bajos de vitamina D aumentan el riesgo de presentar una densidad mineral ósea reducida y de fractura. El déficit de vitamina D podría también tener efectos no deseados sobre la respuesta inmune de los pacientes, potenciando mecanismos de pérdida de tolerancia y autoinmunidad. Los niveles de vitamina D deberían ser monitorizados periódicamente y los pacientes deberían ser tratados con el objetivo de alcanzar unos niveles de vitamina D superiores a 30-40 ng/ml (AU)

The prevalence of vitamin D deficiency and insufficiency among patients with systemic lupus erythematosus is high. This is likely due to photoprotection measures in addition to intrinsic factors of the disease. Low levels of vitamin D increase the risk of low bone mineral density and fracture. Vitamin D deficiency could also have undesirable effects on patients’ immune response, enhancing mechanisms of loss of tolerance and autoimmunity. Vitamin D levels should be periodically monitored and patients should be treated with the objective of reaching vitamin D levels higher than 30-40 ng/ml (AU)

Dermatomiositis y miastenia gravis: una asociación infrecuente con implicaciones terapéuticas / Dermatomyositis and myastenia gravis: an uncommon association with therapeutic implications

La asociación de dermatomiositis y miastenia gravis (MG) es infrecuente, habiéndose comunicado hasta la actualidad únicamente 26 casos. Se presenta el caso de un varón de 69 años diagnosticado de MG 2 años atrás, en tratamiento con piridostigmina, que inicia cuadro agudo de debilidad muscular proximal, artralgias en hombros y elevación de creatincinasa (CK); así como aparición de eritema facial generalizado y pápulas de Gottron. En el estudio de laboratorio se evidenció positividad de anticuerpos antinucleares y anti-Mi2. Ulteriores determinaciones de CK mostraron niveles por encima de 1.000 U/l. Se discute el manejo clínico de este paciente y las implicaciones terapéuticas que plantea la coexistencia de ambas entidades (AU)

The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed (AU)