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The hand of respiratory failure

Published:January 15, 2019DOI:https://doi.org/10.1016/j.ejim.2019.01.003

      Highlights

      • Antisynthetase is an inflammatory myopathy associated with pulmonary manifestations.
      • echanic's hand is one of the most important clinical clues for this disease.
      • Positive anti-RNA synthetase antibodies, most commonly anti-Jo1, is the confirm of the diagnosis.

      1. Case presentation

      73-year-old man presented to our institution with shortness of breath, diffuses myalgia and noticed the significant change of his hands. Physical examination was remarkable for bilateral crackle at lung base and rough, cracked skin at the tips and lateral aspects of the fingers “mechanic's hands” (Fig. 1A ). Complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate and C-reactive protein were unremarkable. Computerized tomography of chest demonstrated small consolidations with peribronchovascular and peripheral subpleural and non-subpleural distribution, some with air bronchogram, and perilobular opacities (Fig. 1B). What is the diagnosis?
      Fig. 1
      Fig. 1A: Mechanic's hands, the cracking and/or fissuring of the lateral and palmar aspects. B: CT Chest demonstrated small consolidations with peribronchovascular and peripheral subpleural and non-subpleural distribution, some with air bronchogram, and perilobular opacities.

      2. Diagnosis

      With high suspicious of Antisynthetase syndrome (AS) due to hand findings, Anti-Jo1 antibody was sent and came back positive. AS was diagnosed base on clinical and serology. Patient was started on prednisone and mycophenolate mofetil with marked clinically improvement.
      AS is an inflammatory myopathy associated with pulmonary manifestations [
      • Jouneau S.
      • Hervier B.
      • Jutant E.M.
      • Decaux O.
      • Kambouchner M.
      • Humbert M.
      • et al.
      ], especially interstitial lung diseases. Positive anti-RNA synthetase antibodies, most commonly anti-Jo1 [
      • Ingegnoli F.
      • Lubatti C.
      • Ingegnoli A.
      • Boracchi P.
      • Zeni S.
      • Meroni P.L.
      Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature.
      ], confirm the diagnosis. The manifestation of extra-thoracic presentations may suggest the diagnosis and “mechanic's hands” is one of the most important clinical clues for this disease [
      • Jouneau S.
      • Hervier B.
      • Jutant E.M.
      • Decaux O.
      • Kambouchner M.
      • Humbert M.
      • et al.
      ]. Prognosis of this rare disease is determined by the extent of pulmonary involvement and progression [
      • Ingegnoli F.
      • Lubatti C.
      • Ingegnoli A.
      • Boracchi P.
      • Zeni S.
      • Meroni P.L.
      Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature.
      ]. Our patient had 6 months follow up without clinical or radiologic signs of progression or exacerbation of disease.

      Funding

      None.

      Disclosure

      None for all authors.

      References

        • Jouneau S.
        • Hervier B.
        • Jutant E.M.
        • Decaux O.
        • Kambouchner M.
        • Humbert M.
        • et al.
        Rev Mal Respir. 2015; 32 (Pulmonary manifestations of antisynthetase syndrome): 618-628
        • Ingegnoli F.
        • Lubatti C.
        • Ingegnoli A.
        • Boracchi P.
        • Zeni S.
        • Meroni P.L.
        Interstitial lung disease outcomes by high-resolution computed tomography (HRCT) in Anti-Jo1 antibody-positive polymyositis patients: a single centre study and review of the literature.
        Autoimmun Rev. 2012; 11: 335-340