title: Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature.
abstract: OBJECTIVE
  To describe the spectrum of pulmonary involvement in immunoglobulin A nephropathy (IgAN).
  
  
  METHODS
  We describe two patients with pulmonary renal syndrome related to IgAN and a systematic review of previously reported cases of IgAN and lung involvement.
  
  
  RESULTS
  We identified 23 reports of IgAN-related pulmonary disease, including 19 reports of alveolar hemorrhage and two cases of organizing pneumonia. Dyspnea (84%), hemoptysis (74%), cough (53%) and fever (47%) were common presenting complaints. Simultaneous involvement of kidneys and lung was the most common presentation (42%) but alveolar hemorrhage occurred independent of renal disease in one-fifth (21%). Azotemia was seen in 55.5% at presentation. Mesangio-proliferative glomerulonephritis was the most common biopsy finding and crescentic glomerulonephritis was seen in 27.7%. Among patients undergoing lung biopsy, capillaritis was seen in 72.7%; 37.5% of these had IgA deposits. Steroids with cyclophosphamide, followed by maintenance with methotrexate or azathioprine was used in 44%. Mechanical ventilation, dialysis and plasmapheresis were other adjunctive therapies used. IgAN-related alveolar hemorrhage was associated with a mortality of 26.3% and significant morbidity, with 52.7% having end-stage kidney disease despite immunosuppression. Organizing pneumonia with pulmonary IgA deposition is a well-described association of IgAN.
  
  
  CONCLUSIONS
  These findings are similar to our previous observations of Henoch-Schonlein purpura (HSP)-related alveolar hemorrhage, highlighting the similarities of these related syndromes. Multicentric studies of IgAN and HSP-related pulmonary renal syndrome with a standard protocol are needed to define their similarities and differences, optimum suppression and its role in preventing renal progression in this setting.
authors: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
authors: Srinivas
authors: http://orcid.org/0000-0001-5723-4375
authors: S
authors: Rajagopala
authors: Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
authors: Sreejith
authors: S
authors: Parameswaran
authors: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
authors: Jail Singh
authors: JS
authors: Ajmera
authors: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
authors: Rajesh Nachiappa
authors: RN
authors: Ganesh
authors: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India.
authors: Anudeep
authors: A
authors: Katrevula
mesh_terms: D000328:Adult; D001706:Biopsy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006470:Hemorrhage / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000276:immunology / Q000473:pathology; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D006435:Renal Dialysis; D012121:Respiration, Artificial; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: IgA nephropathy; cryptogenic organizing pneumonia; diffuse alveolar hemorrhage; pulmonary renal syndrome