A necessidade do diagnóstico multidisciplinar na síndrome anti-sintetase

Público alvo: técnico.

Ontem mesmo vi paciente com tosse crônica, achados intersticiais na tomografia computadorizada de pulmões, feita há 1 ano atrás, e queixa de fraqueza progressiva, especialmente na musculatura proximal de membros inferiores. Exames inconclusivos, com CPK normal. Claros e múltiplos estertores de bases na ausculta pulmonar. Sem diagnóstico após 6 médicos visitados, nenhum ouviu seus pulmões (mais sobre o abandono do exame físico pelos médicos em um próximo post). A síndrome anti-sintetase foi descrita há vários anos, quadro pulmonar e muscular autoimune do grupo das polimiosites, com a presença de autoanticorpos específicos. Anti-Jo1 é o anticorpo mais conhecido. Publicação de hoje do J Rheumatol (Canadá) dá conta da imperiosa necessidade do diagnóstico multidisciplinar na síndrome anti-sintetase.

A Multidisciplinary Evaluation Helps Identify the Antisynthetase Syndrome in Patients Presenting as Idiopathic Interstitial Pneumonia

Sandra ChartrandJeffrey J. SwigrisLina PeykovaJonathan ChungAryeh Fischer

The Journal of Rheumatologyjrheum.150966

Introduction Interstitial lung disease (ILD) is 1 possible manifestation of the idiopathic inflammatory myopathies (IIM). Occasionally, patients presenting with ILD are mistakenly diagnosed with idiopathic interstitial pneumonia (IIP), but after multidisciplinary evaluation, their ILD is determined to be because of antisynthetase syndrome (SynS) or myositis spectrum of disease.

Methods We used retrospective analytic methods to identify patients with ILD evaluated at the National Jewish Health between February 2008 and August 2014 and believed initially to have IIP but ultimately diagnosed with SynS or myositis spectrum of disease.

Results The cohort included 33 patients; most were white women with a mean age at presentation of 55 years. Their pulmonary physiologic impairment was moderate. In 31 cases, the ILD pattern by thoracic high-resolution computed tomography scan was nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), or a combination of the 2. Surgical lung biopsy was performed in 21 patients; NSIP was the most common pattern. Less than one-third of the cohort had positive antinuclear antibodies. Two-thirds had positive SSA. All patients had either myositis-specific or myositis-associated autoantibody. Most had subtle extrathoracic symptoms or signs of SynS; 12 had an elevated serum creatine phosphokinase, but none had clinical evidence of myositis. None met the Peter and Bohan classification criteria for polymyositis/dermatomyositis.

Conclusion Among patients who present with presumed IIP, a multidisciplinary evaluation that includes the integration of clinical evaluations by rheumatologists and pulmonologists, morphologic (both histopathologic and radiographic) data, and serologic features is helpful in the detection of occult SynS or the myositis spectrum of disease.

 

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