Organizing pneumonia as the first manifestation of anti-synthetase syndrome

Abstract

BackgroundAnti-synthetase syndrome associated interstitial lung disease can occur either simultaneously, before, or after the development of polymyositis/dermatomyositis. Histology of interstitial lung disease can be nonspecific interstitial pneumonia, usual interstitial pneumonia, diffuse alveolar damage, organizing pneumonia. Organizing pneumonia associated anti-synthetase syndrome is a rare finding especially as the first manifestation.Case presentationWe report a 41 year old male patient who presented with organizing pneumonia and 2 years following the onset, developed polymyositis with anti-JO-1 antibody positivity.ConclusionIt is important to screen patients with organizing pneumonia for anti-synthetase syndrome which can be manifested later.

A case of antisynthetase syndrome

“…This case highlights the significance of evaluating serum autoantibodies in patients presenting with subtle autoimmune manifestations not fulfilling classification criteria of an autoimmune disease 1 . Moreover, the unusual presentation of antisynthetase syndrome as OP‐like picture is emphasized 2 …”

Acute hypoxic respiratory failure secondary to antisynthetase syndrome: A case report and review of literature

“…Total 30 cases reported, 17 male patients and 13 female patients. Only 43% of the cases presented with lung symptoms alone [2,4,7,10,[16], [17], [18],[23], [24], [25],29,32], while 57% of the cases presented with lung and muscle symptoms simultaneously [1,5,8,[11], [12], [13], [14], [15],[19], [20], [21], [22],[26], [27], [28],30,31]. We did not review any cases that presented with muscle symptoms alone.…”

“…There are multiple patterns of interstitial lung disease in antisynthetase syndrome. These include nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), diffuse alveolar damage (DAD), organizing pneumonia (OP) and acute interstitial pneumonia (AIP) [5]. NSIP is the most common in antisynthetase syndrome [6].…”

The antisynthetase syndrome

“…6 Disease presentation is heterogenous, with patients presenting with organizing pneumonia, diffuse alveolar hemorrhage, pulmonary capillaritis without diffuse alveolar hemorrhage on biopsy, and even hypoxic respiratory failure leading to intubation. [7][8][9] ASyS pathogenesis remains poorly understood, highlighting the complexity of the disease. It is thought to result from environmental and/or infectious agents leading to pulmonary injury.…”