Lung transplantation in rheumatic diseases 3/5 (3)

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Illustration By SSgt. Derrick C. Goode, US Air Force (; exact source) [Public domain], via Wikimedia Commons

The role of the rheumatologist

Lung disease is often a complication by Systemic connective tissue diseases:. Particularly exposed are patients are with Systemic sclerosis and Antisynthetase syndrome. The latter mentioned is a subset of myositis. Some types of lung abnormalities can be very serious. Among potentially severe lung conditions is progressive Interstitial Lung Disease (ILD) og Pulmonary hypertension, but also Bronchiolitis obliterans (BO), Diffuse alveolar bleeding, pleural (lung membrane) - disease. Despite drug treatment, pulmonary disease can develop into pulmonary failure and death. Lung transplant can be life saving treatment for some1. However, applications for transplant are often rejected for this patient group. The causes can be complicating multi-organ manifestations (multiple organs disease), but also unnecessary uncertainty about disease progression and post-transplant management in these diseases.

Lung transplantation in rheumatic diseases

Occurrence of lung manifesations and transplants * in systemic connective tissue disease and rheumatoid arthritis.

        Disease Pulmonary affection2-4 Number of transplanted *      

* Based on data from Organ Procurement and Transplantation Network (OPTN) and Loyola University Medical Center (LUMC), USA in the 1991-2009 period.

The number of lung transplants has increased significantly in recent years. Most people transplant both lungs simultaneously, giving the longest median survival. After one, three and five years of transplant, are respectively 79%, 64% and 55% of the transplanted alive. Among the 14 most frequent transplantation causes, according to the International Society for Heart and Lung Transplantation (ISHLT) registry5is Sarcoidosis number 7 on list and systemic connective tissue disease is number 10. The most common cause of transplant is COPD / emphysema, Followed by pulmonary fibrosis and cystic fibrosis.

When comparing registry data (OPTN and LUMC, Table1) for survival after transplant for Systemic connective tissue diseases: (N = 284) with other groups (COPD n = 4190, Idiopathic pulmonary fibrosis n = 4190), one finds no significant difference in survival. However, there is a highly selected group of patients (most likely to survive) with systemic connective tissue diseases who are transplanted.

Reasons for refusal for transplantation

Oesophagus manifestations (the Gullet) are common, especially in Systemic sclerosis is a problem.

  • Reduced function (dysmotility) and gastro-oesophageal reflux are clearly associated with lung injury in the form of Bronchiolitis obliterans (BO) which again is a common cause of the rejection of the lung transplant
  • In systemic sclerosis, esophagus is affected in the lower 2 / 3 so that the peristalsis and the stomach work poorly. This is detected by X-ray with contrast and by manometry and PH measurements
  • Bronchiolitis obliterans (BO) as a complication often occur 6-24 months after transplantation. After 5 years from transplantation, 50-60% have signs of BO and 30% of transplant-related deaths caused by BO
  • Suspicion of BO should occur if pulmonary function tests show decreasing FEV1 and the patient is experiencing increasing heavy breathing (dyspnea) upon stress
  • Surgical reflux treatment with fundoplication and gastric bypass (RYGB) can reduce the risk and allow transplantation in some cases.

Indications and contraindications for lung transplantation, Guidelines ISHLT6


Progressive interstitial lung disease

  • Lung function measured by FVC and DLCO falls despite immunosuppressive treatment with eg cyclophosphamide (Sendoxan) or mycophenolate (CellCept)
  • Seriously reduced lung function
    • DLCO less than 40% of expected
    • FVC worsening of at least 10% over 6 months
    • Oxygen requirements (often defined for pO2 below 55 mm. Hg at rest or pO2 below 60 mmHg for concomitant pulmonary hypertension)


  • Systemic sclerosis with shorter than 5's medical history (from non-Raynaud's symptoms)
  • No cancer disease last 2 years
  • No HIV or Hepatitis B infection
  • Non-compliance for drugs, abuse or lack of social follow-up
  • Other serious organ complication

Relative contraindications

  • Age over 65 years
  • Critical illness
  • Significantly reduced general condition
  • Obesity
  • Severe osteoporosis
  • Respirator use
  • Infection with multiresistant bacteria

The rheumatologist's tasks during lung transplantation

  • Rheumatologists should help to investigate oesophagus dysmotility and reflux prior to transplant assessment.
  • Rheumatologists should initiate interdisciplinary cooperation with lung doctor, transplant surgeon, gastroenterologist and others
  • Rheumatologists must be able to monitor patient disease activity optimally after a transplant has been performed


  • Systemic connective tissue disease and rheumatoid arthritis can lead to pulmonary end stage
  • Lung transplant is life saving for some, but many applications for treatment are rejected
  • Rheumatologists should know indications for lung transplant treatment in systemic connective tissue disease and play an active role in interdisciplinary transplantation and post-transplant follow-up


1. Sem M. Long-term outcome of lung transplantation in a patient with the anti-synthetase syndrome. Scan J Rheum 2011; 40; 327-328.
2. Takagishi T. Survival and extrapulmonary course of connective tissue diseases after lung transplantation. J Clin Rheum 2012; 18; 283-289.
3. Gunnarsson R. Prevalence and severity of lung disease in mixed connective tissue disease: a nationwide, cross-sectional study. Ann Rheum is 2012; 71; 1966-1972.
4. Palm Ø. Clinical pulmonary involvement in primary Sjogren's syndrome: prevalence, quality of life and mortality - a retrospective study based on registry data. Rheumatology 2013; 52; 173-179.
5. Christie JD. The Registry of the International Society for Heart and Lung Transplantation: 29th Adult lung and heart transplant report-2012. J Heart Lung Transplant 2012; 31; 1073-1086.
6. Orens JB. Internationale richtlijnen voor de selectie van lungtransplantatiekandidaten: 2006 update-een consensusverslag van de Pulmonale Wetenschappelijke Raad van de Internationale Vereniging voor Hart- en Lungtransplantatie. J Heart Lung Transplant 2006; 745-755.

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