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A variation of vasculopathies in a patient with mild pulmonary artery hypertension
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  1. Takao Kato,
  2. Takamasa Kitajima,
  3. Moriaki Inoko
  1. Department of Cardiovascular Medicine, The Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan
  1. Correspondence to Dr Takao Kato, takao-kato{at}kitano-hp.or.jp

https://doi.org/10.1136/bcr-2013-200068

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    Description

    A 66-year-old woman with a 2-year history of rheumatoid arthritis (RA) that was treated with prednisolone was referred to our hospital due to a slight shortness of breath with normal vasculatures (figure 1A,B) and a small coin lesion (figure 1C,D). An estimated pulmonary artery pressure on echocardiography (figure 1E) of 44 mm Hg that increased to 65 mm Hg after 6 months. The mean pulmonary arterial pressure (PAP) was 37 mm Hg (figure 2) without a shuntvitium, the mean pulmonary capillary wedged pressure was 10 mm Hg, and the calculated pulmonary vascular resistance (PVR) was increased to 376 dyn/(s/cm5). Because perfusion scintigraphy showed no abnormalities without clinical signs of systemic lupus erythematous, mixed connective tissue disease or systemic scleroderma, we diagnosed her as idiopathic pulmonary artery hypertension (IPAH). Administration of bosentan (125 mg) for 1 month improved her symptom and decreased the PAP and PVR to 27 mm Hg and 290 dyn/(s/cm5), respectively. Because the coin lesion increased in size, right upper lobe was resected 2 months later (figure 3A), which revealed a granulomatous lesion with Cryptococcus spp. Although normal pulmonary arteries were also observed, medial hypertrophy, intimal thickening (figure 3B,C), microthrombosis (arrowheads; figure 3B,D), and few plexiform lesions (figure 3E) were observed in the same field, indicating that a variation was observed in a patient with mild PAH,1 as well as severe PAH in autopsy or explantation cases.2

    Figure 1
    Figure 1

    (A and B) Three dimensional reconstraction image of contrast-enhanced CT angiography. Anterior (A). Posterior (B). (C) A chest X-ray at admission. (D) Coin lesion in CT scans. (E) Echocardiography.

    Figure 2
    Figure 2

    Details of right heart catheterisation, laboratory findings, physical activity and respiratory function testing.

    Figure 3
    Figure 3

    (A) Macroscopic examination of resected lung. (B) Medial hypertrophy, intimal thickening and microthrombosis (arrowheads) were observed by H&E stainings. In the same field, normal pulmonary arteries were also observed. (C) Medial hypertrophy by elastic-Van Gieson (EVG) staining. (D) Microthrombosis. (E) A plexiform lesion by EVG staining.

    Since there were few reports3 and clinical signs, we could not conclude whether the RA caused PAH, a very rare case or IPAH was coincidentally observed in a patient with RA.

    Learning points

    • Pathological lesions in the pulmonary artery hypertension (PAH) are thought to belong to a similar spectrum in extension and distribution; however, a variation in the lesions was observed in a patient, even if it is very mild, as previously reported Wagenvoort, 1970.4

    • Medial hypertrophy, intimal thickening, microthrombosis and a few plexiform lesions were observed and normal pulmonary arteries were also observed in the same field.

    • Pulmonary hypertension with rheumatoid arthritis (RA) is rare, therefore we could not conclude whether the RA caused PAH, a very rare case, or idiopathic pulmonary artery hypertension was coincidentally observed in a RA patient.

    References

    1. ↵
      1. Strange G,
      2. Playford D,
      3. Stewart S,
      4. et al
      . Pulmonary hypertension: prevalence and mortality in the Armadale echocardiography cohort. Heart 2012;2013:1805–11.
      OpenUrl
    2. ↵
      1. Yi ES,
      2. Kim H,
      3. Ahn H,
      4. et al
      . Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am J Respir Crit Care Med 2000;2013:1577–86.
      OpenUrl
      1. Udayakumar N,
      2. Venkatesan S,
      3. Rajendiran C
      . Pulmonary hypertension in rheumatoid arthritis–relation with the duration of the disease. Int J Cardiol 2008;127:410–2.
      OpenUrlCrossRefPubMed
      1. Wagenvoort CA
      . The pathology of primary pulmonary hypertension. J Pathol 1970;101:Pi.
      OpenUrlCrossRefPubMed
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    Footnotes

    • Contributors TK wrote the manuscript and RN and MI were supervisors of the manuscript.

    • Competing interests None.

    • Patient consent Obtained.

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