Case Presentation:

A 21 year old man presented with severe shortness of breath and hypoxia. He had history of Anti-GBM disease (Goodpasture’s syndrome), living related donor renal transplant, failed renal transplant due to medication non-adherence with subsequent acute rejection. He underwent removal of his transplanted kidney and was taken off all immunosuppression one month prior to this presentation.
Chest X ray showed bilateral pleural effusions with left side significantly worse than right. Left sided thoracentesis was performed and a chest tube was placed with drainage of 2 Liters of exudative pleural fluid. Computed tomography of the chest confirmed presence of loculated effusions bilaterally.
Laboratory work up showed lymphopenia, ANA of 1:640, nucleolar pattern and positive anti-Histone antibody. Anti-dsDNA, Anti-Sm, RNP, SSA, and SSB, ANCA and Anti-GBM antibodies were all negative. Lupus anticoagulant was positive. Anti-cardiolipin and Anti-beta2-glycoprotein antibodies were negative. C3 and C4 levels were normal. Original renal biopsy and transplant pathologies showed no evidence of an immune complex process suggestive of lupus nephritis.
Patient met the ACR criteria for Systemic Lupus Erythematosus and was started on pulse dose steroids and hydroxychloroquine. This resulted in significant improvement in hypoxia and dyspnea. He was discharged and has stayed in remission of pulmonary symptoms with mycophenolate mofetil, prednisone and hydroxychloroquine.

Discussion:  

This patient’s presentation with respiratory symptoms after withdrawal of immunosuppression for transplant was suspicious for pulmonary manifestation of recurrent Anti-GBM disease. However, Anti-GBM antibody was negative. High ANA titres with Anti-histone positivity and clinical symptoms led to the diagnosis of SLE. The absence of Anti-dsDNA and Anti-Sm antibodies was also peculiar. Improvement of symptoms with steroids and disease modifying agents validated the diagnosis of SLE. 

Conclusions:

 The association of SLE in a patient previously diagnosed with and treated for Anti GBM disease is rare. There have been no reported cases of concomitancy and none to our knowledge, of SLE developing in a post renal transplant patient with Anti-GBM disease. This case also highlights that patients with one autoimmune disease are at an increased risk of being diagnosed with other autoimmune diseases.