Correct!
5. All of the above

DAH associated with SLE is rare but with very high mortality. Initial treatment is generally with high dose corticosteroids as our patient received (1). Other cytotoxic drugs such as cyclophosphamide can be added if there is poor response to steroids, as also in our case. She also received 5 days of plasmapheresis, which is another option for difficult cases but has not been demonstrated to improve mortality rates. Other case reports exist with a good response to mycophenolate mofetil, rituximab or even extracorporeal membrane oxygenation (ECMO) (7-9). Concomitant infections are an important co-factor that has been shown to increase mortality (10).

In summary, DAH is a rare but serious complication of SLE. It should be considered in the differential diagnosis in SLE patient presenting with pulmonary complaints and decreasing hemoglobin. Early bronchoscopy should be considered to establish the diagnosis and evaluate for concomitant infection. Immunosuppressive therapy should be started in a timely manner to improve patient outcomes.

References

  1. Traclet J, Lazor R, Cordier JF, Cottin V. Alveolar hemorrhage. Rev Med Interne. 2013;34(4):214-23. [CrossRef] [PubMed]
  2. Meyer KC, Raghu G, Baughman RP, Brown KK, Costabel U, du Bois RM, Drent M, Haslam PL, Kim DS, Nagai S, Rottoli P, Saltini C, Selman M, Strange C, Wood B; American Thoracic Society Committee on BAL in Interstitial Lung Disease. An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med. 2012;185(9):1004-14. [CrossRef] [PubMed]
  3. Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010;137(5):1164-71. [CrossRef] [PubMed]
  4. Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine (Baltimore). 1997;76(3):192-202. [CrossRef] [PubMed]
  5. Virdi RP, Bashir A, Shahzad G, Iqbal J, Mejia JO. Diffuse alveolar hemorrhage: a rare life-threatening condition in systemic lupus erythematosus. Case Rep Pulmonol. 2012;2012:836017. [CrossRef] [PubMed]
  6. Nellessen CM, Pöge U, Brensing KA, Sauerbruch T, Klehr HU, Rabe C. Diffuse alveolar haemorrhage in a systemic lupus erythematosus patient successfully treated with rituximab: a case report. Nephrol Dial Transplant. 2008;23(1):385-6. [CrossRef] [PubMed]
  7. Al Rashidi A, Alajmi M, Hegazi MO. Mycophenolate mofetil as a maintenance therapy for lupus-related diffuse alveolar hemorrhage: a case report. Lupus. 2011;20(14):1551-3. [CrossRef] [PubMed]
  8. Pottier V, Pierrot M, Subra JF, Mercat A, Kouatchet A, Parrot A, Augusto JF. Successful rituximab therapy in a lupus patient with diffuse alveolar haemorrhage. Lupus. 2011;20(6):656-9. [CrossRef] [PubMed]
  9. Patel JJ, Lipchik RJ. Systemic lupus-induced diffuse alveolar hemorrhage treated with extracorporeal membrane oxygenation: a case report and review of the literature. J Intensive Care Med. 2014;29(2):104-9. [CrossRef] [PubMed]
  10. Martinez-Martinez MU, Sturbaum AK, Alcocer-Varela J, et al. Factors associated with mortality and infections in patients with systemic lupus erythematosus with diffuse alveolar hemorrhage. J Rheumatol. 2014;41(8):1656-61. [CrossRef] [PubMed]

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