Article Text

Download PDFPDF
Rare case of Sjögren’s syndrome antibody (SSA-Ro52kDA)-associated interstitial lung disease and myositis with pipe stem capillaries
  1. Anitha Ramu1,
  2. Navneet Kaur2 and
  3. Ruchi Jain2
  1. 1Department of Internal Medicine, Jacobi Medical Center, Bronx, New York, USA
  2. 2Department of Rheumatology, Montefiore Medical Center, Bronx, New York, USA
  1. Correspondence to Dr Anitha Ramu; anitha_ramu{at}live.com

Abstract

Necrotising myopathy with pipestem capillaries is a distinct form of inflammatory myopathy exhibiting only sparse inflammation on biopsy, with clinical presentation and histopathological profile entirely different from dermatomyositis, polymyositis or inclusion body myositis. A 51-year-old non-diabetic man presents with progressively worsening shortness of breath and myalgias with only mild proximal muscle weakness and elevated serum creatine kinase. Autoimmune workup, ordered after ruling out infectious and cardiac aetiologies, returned positive for Sjögren’s syndrome antibody (SSA/Ro-52). Lung imaging and biopsy were suggestive of cryptogenic organising pneumonia and muscle biopsy showed myositis with pipestem capillaries and abnormal deposition of membrane attack complex with only sparse inflammation. The patient received high-dose steroids, mycophenolate mofetil, intravenous immunoglobulin and rituximab with improvement in muscle symptoms. However, his pulmonary findings progressed, requiring evaluation for a lung transplant. This case emphasises the need for further research to better understand this disease entity and improve mortality and morbidity in these patients.

  • interstitial lung disease
  • musculoskeletal syndromes
  • sjogren's syndrome
  • connective tissue disease
  • capillary

Statistics from Altmetric.com

Footnotes

  • Contributors AR was actively involved in writing and editing the case report. NK and RJ were actively involved in treating the patient and contributed to the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.