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What does pulmonary–renal syndrome stand for?

Egyptian Journal of Bronchology volume 12, pages 1–13 (2018)Cite this article

Abstract

Pulmonary–renal disorder (PRS) is an emergency situation described by a rapidly progressive course without an early intervention. It is appropriate time to review this disorder, this is may be attributable to frequent patients’ attendance to pulmonologist with both vague pulmonary and/or renal symptoms with disproportionate lack of information concerning consequent care. In addition, the outcome data for PRS still confined to little studies with limited follow-up. An updated working knowledge of PRS including the disease pathogenesis, complications as well as quickly advancing field focused on current new immunomodulatory therapies which offer life-saving options for refractory disease. An often-multi-disciplinary team is required for management. Early rapid identification relies upon a high index of clinical suspicious, carful medical evaluation, accessible laboratory investigations, imaging study, histopathology, with exclusion of differential diagnosis. An accurate diagnosis, exclusion of infection, close monitoring of the patient as well as timely initiation of aggressive therapy are crucial for the patient’s outcome. The mortality rate of PRS, reach up to 25–50 % [1].

References

  1. Griffith M, Brett S. The pulmonary physician in critical care illustrative case 3: pulmonary vasculitis. Thorax 2003; 58:543–546.

    CAS  PubMed  PubMed Central  Google Scholar 

  2. Gallagher H, Kwan J, Jayne RW. Pulmonary-renal syndrome: a 4-year, single center experience. Am J Kidney Dis 2002; 38:42–47.

    Google Scholar 

  3. Papiris SA, Manali ED, Kalomenidis L, Kapotsis GE, Karakatsani A, Roussos C. Bench-to-bedside review: pulmonary-renal syndromes – an update for the intensivist. Critical Care 2007; 11:213.

    PubMed  PubMed Central  Google Scholar 

  4. McCabe C, Jones Q, Nikolopoulou A, Wathen C, Luqmani R. Pulmonaryrenal syndromes: an update for respiratory physicians. Respir Med 2011; 105:1413–1421.

    PubMed  Google Scholar 

  5. Ioachimescu OC, Stoller JK. Diffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med 2008; 75:258–280.

    PubMed  Google Scholar 

  6. Moroni G, Ponticelli C. Rapidly progressive crescentic glomerulo-nephritis: early treatment is a must. Autoimmun Rev 2014; 13:723–729.

    PubMed  Google Scholar 

  7. Kimmel M, Braun N, Alscher MD. Differential diagnosis of the pulmonary–renal syndrome. In: Prabhakar S, editor. An update on glomerulopathies – clinical and treatment aspects. InTech; 2011. pp. 291–299.

  8. Sexena R, Bygren P, Arvastson B, Wieslander J. Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome. J Internal Med 1995; 238:143–152.

    Google Scholar 

  9. Niles JL, Böttinger EP, Saurina GR, Kelly KJ, Pan G, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Internal Med 1996; 156:440–445.

    CAS  Google Scholar 

  10. Falk RJ, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 1988; 318:1651–1657.

    CAS  PubMed  Google Scholar 

  11. Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet 2006; 368:404–418.

    CAS  PubMed  Google Scholar 

  12. LittleMA, Smyth CL, Yadav R,Ambrose L, Cook HT, Sussan Nourshargh S, Pusey CD. Antineutrophil cytoplasm antibodies directed against myeloperoxidase augment leukocyte microvascular interactions in vivo. Blood 2005; 106:2050–2058.

    CAS  PubMed  Google Scholar 

  13. Xiao H, Heeringa P, Liu Z, Huugen D, Hu P, Maeda D, et al. The role of neutrophils in the induction of glomerulonephritis by anti-myelo-peroxidase antibodies. Am J Pathol 2005; 167:39–45.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Booth A, Pusey C, Jayne D. Renal vasculitis – an update in 2004. Nephrol Dial Transplant 2004; 19:1964–1968.

    PubMed  Google Scholar 

  15. Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest 2010; 137:1164–1171.

    PubMed  Google Scholar 

  16. Bacon PA. The spectrum of Wegener’s granulomatosis and disease relapse. N Engl J Med 2005; 352:330–332.

    CAS  PubMed  Google Scholar 

  17. Langford C, Hoffman G. Wegener’s granulomatosis. Thorax 1999; 54:629–637.

    CAS  PubMed  PubMed Central  Google Scholar 

  18. Savige J, Davies D, Falk R, Jennette JC, Wiik A. Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int 2000; 57:846–862.

    CAS  PubMed  Google Scholar 

  19. Schwarz M, Brown K. Small vessel vasculitis of the lung. Thorax 2000; 55:502–510.

    CAS  PubMed  PubMed Central  Google Scholar 

  20. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine 1999; 78:26–37.

    CAS  PubMed  Google Scholar 

  21. Salama AD, Levy J, Lightstone L, Pusey CD. Goodpasture’s disease. Lancet 2001; 358:917–920.

    CAS  PubMed  Google Scholar 

  22. Phelps RG, Jones V, Turner AN, Rees AJ. Properties of HLA class II molecules divergently associated with Goodpasture’s disease. Int Immunol 2000; 12:1135–1143.

    CAS  PubMed  Google Scholar 

  23. Lamprecht P, Holle J, Wolfgang L. Pulmonary haemorrhage in systemic lupus erythematosus. Lupus 1997; 6:445–448.

    Google Scholar 

  24. Aringer M, Smolen JS. Cytokine expression in lupus kidneys. Lupus 2005; 14:13–18.

    CAS  PubMed  Google Scholar 

  25. Schwarz MI, Fontenot AP. Drug-induced diffuse alveolar haemorrhage syndromes and vasculitis. Clin Chest Med 2004; 25:133–140.

    PubMed  Google Scholar 

  26. Kalra A, Yokogawa N, Raja H, Palaniswamy C, Desai P, Zanotti-Cavazzoni SL, Rajaram SS. Hydralazine-induced pulmonary-renal syndrome: a case report. Am J Ther 2012; 19:e136–e138.

    PubMed  Google Scholar 

  27. Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clin Chest Med 2004; 25:583–592.

    PubMed  Google Scholar 

  28. Costabel U, du Bois RM, Egan JJ, editors. Diffuse parenchymal lung disease. Basel, Switzerlabd: Karger; 2007: pp. 250–263.

  29. Merkel PA, Choi H, Niles JL. Evaluation and treatment of vasculitis in the critically ill patient. Crit Care Clin 2002; 18:321–344.

    PubMed  Google Scholar 

  30. Hudson BG, Tryggvason K, Sundara-moorthy M, Neilson EG. Alport’s syndrome, Good-pasture’s syndrome, and type IV collagen. N Engl J Med 2003; 348:2543–2556.

    CAS  PubMed  Google Scholar 

  31. Lau K, Wyatt R. Glomerulonephritis. Adolesc Med 2005; 16:67–85.

    Google Scholar 

  32. Park MS. Diffuse alveolar hemorrhage. Tuberc Respir Dis (Seoul) 2013; 74:151–162.

    Google Scholar 

  33. Jennette JC, Wilkman AS, Falk RJ. Diagnostic predictive value of ANCA serology [editorial]. Kidney Int 1998; 53:796–798.

    CAS  PubMed  Google Scholar 

  34. Mazidi P, Bajestani S, Khan M, Khair T, Luis L. Goodpasture’s syndrome: a case report and review of the literature. Internet J Intern Med 2008; 7:1–5.

    Google Scholar 

  35. Jara LJ, Vera-Lastra O, Calleja MC. Pulmonary-renal vasculitic disorders: differential diagnosis and management. Curr Rheumatol Rep 2003; 5:107–115.

    PubMed  Google Scholar 

  36. Davies DJ. Small vessel vasculitis. Cardiovasc Pathol 2005; 14:335–346.

    PubMed  Google Scholar 

  37. Papiris SA, Manoussakis MN, Drosos A, Kontogiannis D, Constantopoulos SH, et al. Imaging of thoracic Wegener’s granulomatosis: the computed tomographic appearance. Am J Med 1992; 93:529–536.

    CAS  PubMed  Google Scholar 

  38. Ando Y, Okada F, Matsumoto S, Mori H. Thoracic manifestation of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) related disease. J Comput Assist Tomogr 2004; 28:710–716.

    PubMed  Google Scholar 

  39. Jennings C, King T, Tuder R, Cherniack RM, Schwarz MI. Diffuse alveolar hemorrhage with underlying isolated, pauciimmune pulmonary capillaritis. Am J Respir Crit Care Med 1997; 155:1101–1109.

    CAS  PubMed  Google Scholar 

  40. Brown KK. Pulmonary vasculitis. Proc Am Thorac Soc 2006; 3:48–57.

    CAS  PubMed  PubMed Central  Google Scholar 

  41. Tesar V, Rihova Z, Jancova E, Rysova R, Merta M. European randomized trials: current treatment strategies in ANCA-positive renal vasculitis-lessons from European randomized trials. Nephrol Dial Transplant 2003; 18:v2–v4.

    PubMed  Google Scholar 

  42. Hogan S, Falk RJ, Chin H, Cai J, Jennette CE, Jennette JC, Nachman PH. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small vessel vasculitis. Ann Intern Med 2005; 143:621–631.

    PubMed  Google Scholar 

  43. Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al., European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363:211–220.

    CAS  PubMed  Google Scholar 

  44. British Thoracic Society. Interstitial lung disease guideline; appendix 5, notes on cyclophosphamide therapy in ILD; British Thoracic Society, September 2008 Thorax 2008; 63, supplement V. Available at: http://www.brit-thoracic.org.uk.

  45. Hu W, Liu C, Xie H, Chen H, Liu Z, Li L. Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant 2008; 23: 1307–1312.

    CAS  PubMed  Google Scholar 

  46. Levy JB, Turner AN, Rees AJ, Pusey CD. Long term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann InternMed 2001; 134:1033–1042.

    CAS  Google Scholar 

  47. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, et al.Arandomized trial ofmaintenance therapy for vasculitis associatedwith antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003; 349:36–44.

  48. White ES, Lynch JP. Pharmacological therapy for Wegener’s granulomatosis. Drugs 2006; 66:1209–1228.

    CAS  PubMed  Google Scholar 

  49. Booth A, Harper L, Hammad T, Bacon P, Griffith M, Levy J, et al. Prospective study of TNF alpha blockade with infliximab in antineutrophil cytoplasmic antibody-assocaited systemic vasculitis. J Am Soc Nephrol 2004; 15:717–721.

    CAS  PubMed  Google Scholar 

  50. Lamprecht P, Voswinkel J, Lilienthal T, Nolle B, Heller M, Gross WL, Gause A. Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 2002; 41:1303–1307.

    CAS  Google Scholar 

  51. Wegener’s Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med 2005; 352:351–361.

    Google Scholar 

  52. Feldman M, Pusey CD. Is there a role for TNF-alpha in antineutrophil cytoplasmic antibody-associated vasculitis? Lessons fron other chronic inflammatory diseases. J Am Soc Nephrol 2006; 17:1243–1252.

    Google Scholar 

  53. Stasi R, Stipa E, Del Poeta GD, Amadori S, Newland AC, Provan D. Long term observation of patients with anti-neutrophil cytoplasmic antibodyassociated vasculitis treated with rituximab. Rheumatology (Oxford) 2006; 45:1432–1436.

    CAS  Google Scholar 

  54. Antoniu SA. Rituximab for refractory Wegener’s granulomatosis. Exp Opin Invest Drugs 2006; 15:1115–1117.

    CAS  Google Scholar 

  55. Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ. Mycophenolate mofetil for maintenance therapy of Wegener’s granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol 1999; 10:1965–1971.

    CAS  PubMed  Google Scholar 

  56. Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission aintenance in the treatment of Wegener’s granulomatosis. Arthritis Rheum 2004; 51:278–283.

    CAS  PubMed  Google Scholar 

  57. Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract 2006; 102:c100–c107.

    CAS  PubMed  Google Scholar 

  58. Metzler C, Fink C, Lamprecht P, Gross WL, Reinhold-Keller E. Maintenance of remission with leflunomide in Wegener’s granulomatosis. Rheumatology (Oxford) 2004; 43:315–320.

    CAS  Google Scholar 

  59. Schmitt WH, Hagen EC, Neumann I, Nowack R, Flores-Suarez LF, van der Woude FJ, European Vasculitis Study Group. Treatment of refractory Wegener’s granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int 2004; 65:1440–1448.

    CAS  PubMed  Google Scholar 

  60. Candan S, Pirat A, Varol A, Torgay A, Zeyneloglou P, Arslan G. Respiratory problems in renal transplant recipients admitted to intensive care during long-term follow-up. Transplant Proc 2006; 38:1354–1356.

    CAS  PubMed  Google Scholar 

  61. Appel GB, Contreras G, Dooley MA, Ginzler EM, Isenberg D, Jayne D, et al. Aspreva Lupus Management Study Group. Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis. J Am Soc Nephrol 2009; 20:1103–1112.

    CAS  PubMed  PubMed Central  Google Scholar 

  62. Smith KG, Jones RB, Burns SM, Jayne DR. Long term comparison of rituximab treatment for refractory systemic lupus erythematosus and vasculitis: remission, relapse, and re-treatment. Arthritis Rheum 2006; 54:2970–2982.

    CAS  PubMed  Google Scholar 

  63. Hanly JG. Antiphospholipid syndrome: an overview. CMAJ 2003; 168: 1675–1682.

    PubMed  PubMed Central  Google Scholar 

  64. Fontana S, Kremer Hovinga JA, Lammle B, Mansouri Taleghani B. Treatment of thrombotic thrombocytopenic purpura. Vox Sang 2006; 90:245–254.

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Departments of Chest Diseases, Faculty of Medicine for Girls, Al-Azhar University, Al-Zahraa University Hospital, Al-Abbassia, Cairo, 02-26854947- 11517, Egypt

    Taghreed S. Farag MD

  2. Departments of Pathology, Faculty of Medicine for Girls, Al-Azhar University, Cairo, Egypt

    Abeer S. Farag

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  1. Taghreed S. Farag MD
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  2. Abeer S. Farag
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Correspondence to Taghreed S. Farag MD.

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Farag, T.S., Farag, A.S. What does pulmonary–renal syndrome stand for?. Egypt J Bronchol 12, 1–13 (2018). https://doi.org/10.4103/ejb.ejb_6_17

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