Semin Respir Crit Care Med 2012; 33(05): 476-485
DOI: 10.1055/s-0032-1325158
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)

Sanjay Mukhopadhyay
1   Department of Pathology, State University of New York Upstate Medical University, Syracuse, New York
,
Joseph G. Parambil
2   Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
› Author Affiliations
Further Information

Publication History

Publication Date:
21 September 2012 (online)

Abstract

Acute interstitial pneumonia (AIP) is a term used for an idiopathic form of acute lung injury characterized clinically by acute respiratory failure with bilateral lung infiltrates and histologically by diffuse alveolar damage (DAD), a combination of findings previously known as the Hamman-Rich syndrome. This review aims to clarify the diagnostic criteria of AIP, its relationship with DAD and acute respiratory distress syndrome (ARDS), key etiologies that need to be excluded before making the diagnosis, and the salient clinical features. Cases that meet clinical and pathologic criteria for AIP overlap substantially with those that fulfill clinical criteria for ARDS. The main differences between AIP and ARDS are that AIP requires a histologic diagnosis of DAD and exclusion of known etiologies. AIP should also be distinguished from “acute exacerbation of IPF,” a condition in which acute lung injury (usually DAD) supervenes on underlying usual interstitial pneumonia (UIP)/idiopathic pulmonary fibrosis (IPF).

 
  • References

  • 1 Bernard GR, Artigas A, Brigham KL , et al. The American-European Consensus Conference on ARDS: definitions, mechanisms, relevant outcomes, and clinical trial coordination. Am J Respir Crit Care Med 1994; 149 (3 Pt 1) 818-824
  • 2 Patel SR, Karmpaliotis D, Ayas NT , et al. The role of open-lung biopsy in ARDS. Chest 2004; 125 (1) 197-202
  • 3 Schwarz MI, Albert RK. “Imitators” of the ARDS: implications for diagnosis and treatment. Chest 2004; 125 (4) 1530-1535
  • 4 Ware LB, Matthay MA. The acute respiratory distress syndrome. N Engl J Med 2000; 342 (18) 1334-1349
  • 5 de Hemptinne Q, Remmelink M, Brimioulle S, Salmon I, Vincent JL. ARDS: a clinicopathological confrontation. Chest 2009; 135 (4) 944-949
  • 6 Katzenstein AL, Bloor CM, Leibow AA. Diffuse alveolar damage—the role of oxygen, shock, and related factors: a review. Am J Pathol 1976; 85 (1) 209-228
  • 7 Kaarteenaho R, Kinnula VL. Diffuse alveolar damage: a common phenomenon in progressive interstitial lung disorders. Pulm Med 2011; 2011: 531302 . EPub 2010 Nov 2
  • 8 Hamman L, Rich AR. Fulminating diffuse interstitial fibrosis of the lungs. Trans Am Clin Climatol Assoc 1935; 51: 154-163
  • 9 Olson J, Colby TV, Elliott CG. Hamman-Rich syndrome revisited. Mayo Clin Proc 1990; 65 (12) 1538-1548
  • 10 Askin FB. Acute interstitial pneumonia: histopathologic patterns of acute lung injury and the Hamman-Rich syndrome revisited. Radiology 1993; 188 (3) 620-621
  • 11 Bouros D, Nicholson AC, Polychronopoulos V, du Bois RM. Acute interstitial pneumonia. Eur Respir J 2000; 15 (2) 412-418
  • 12 Katzenstein AL, Myers JL, Mazur MT. Acute interstitial pneumonia: a clinicopathologic, ultrastructural, and cell kinetic study. Am J Surg Pathol 1986; 10 (4) 256-267
  • 13 Katzenstein A-LA, Mukhopadhyay S, Myers JL. Diagnosis of usual interstitial pneumonia and distinction from other fibrosing interstitial lung diseases. Hum Pathol 2008; 39 (9) 1275-1294
  • 14 American Thoracic Society; European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165 (2) 277-304
  • 15 Vourlekis JS. Acute interstitial pneumonia. Clin Chest Med 2004; 25 (4) 739-747 , vii
  • 16 Vourlekis JS, Brown KK, Schwarz MI. Acute interstitial pneumonitis: current understanding regarding diagnosis, pathogenesis, and natural history. Semin Respir Crit Care Med 2001; 22 (4) 399-408
  • 17 Inoue A, Saijo Y, Maemondo M , et al. Severe acute interstitial pneumonia and gefitinib. Lancet 2003; 361 (9352) 137-139
  • 18 Chang SC, Chang CY, Chen CY, Yu CJ. Successful erlotinib rechallenge after gefitinib-induced acute interstitial pneumonia. J Thorac Oncol 2010; 5 (7) 1105-1106
  • 19 Kamata Y, Nara H, Kamimura T , et al. Rheumatoid arthritis complicated with acute interstitial pneumonia induced by leflunomide as an adverse reaction. Intern Med 2004; 43 (12) 1201-1204
  • 20 Primack SL, Hartman TE, Ikezoe J, Akira M, Sakatani M, Müller NL. Acute interstitial pneumonia: radiographic and CT findings in nine patients. Radiology 1993; 188 (3) 817-820
  • 21 Ichikado K, Johkoh T, Ikezoe J , et al. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AJR Am J Roentgenol 1997; 168 (2) 333-338
  • 22 Johkoh T, Müller NL, Taniguchi H , et al. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211 (3) 859-863
  • 23 Vourlekis JS, Brown KK, Cool CD , et al. Acute interstitial pneumonitis: case series and review of the literature. Medicine (Baltimore) 2000; 79 (6) 369-378
  • 24 Quefatieh A, Stone CH, DiGiovine B, Toews GB, Hyzy RC. Low hospital mortality in patients with acute interstitial pneumonia. Chest 2003; 124 (2) 554-559
  • 25 Rice AJ, Wells AU, Bouros D , et al. Terminal diffuse alveolar damage in relation to interstitial pneumonias: an autopsy study. Am J Clin Pathol 2003; 119 (5) 709-714
  • 26 Bonaccorsi A, Cancellieri A, Chilosi M , et al. Acute interstitial pneumonia: report of a series. Eur Respir J 2003; 21 (1) 187-191
  • 27 Suh GY, Kang EH, Chung MP , et al. Early intervention can improve clinical outcome of acute interstitial pneumonia. Chest 2006; 129 (3) 753-761
  • 28 Parambil JG, Myers JL, Aubry MC, Ryu JH. Causes and prognosis of diffuse alveolar damage diagnosed on surgical lung biopsy. Chest 2007; 132 (1) 50-57
  • 29 Avnon LS, Pikovsky O, Sion-Vardy N, Almog Y. Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations. Anesth Analg 2009; 108 (1) 232-237
  • 30 Tomiyama N, Müller NL, Johkoh T , et al. Acute respiratory distress syndrome and acute interstitial pneumonia: comparison of thin-section CT findings. J Comput Assist Tomogr 2001; 25 (1) 28-33
  • 31 Askin FB, Katzenstein AL. Pneumocystis infection masquerading as diffuse alveolar damage: a potential source of diagnostic error. Chest 1981; 79 (4) 420-422
  • 32 Travis WD, Pittaluga S, Lipschik GY , et al. Atypical pathologic manifestations of Pneumocystis carinii pneumonia in the acquired immune deficiency syndrome. Review of 123 lung biopsies from 76 patients with emphasis on cysts, vascular invasion, vasculitis, and granulomas. Am J Surg Pathol 1990; 14 (7) 615-625
  • 33 Guarner J, Paddock CD, Shieh WJ , et al. Histopathologic and immunohistochemical features of fatal influenza virus infection in children during the 2003-2004 season. Clin Infect Dis 2006; 43 (2) 132-140
  • 34 Noble RL, Lillington GA, Kempson RL. Fatal diffuse influenzal pneumonia: premortem diagnosis by lung biopsy. Chest 1973; 63 (4) 644-646
  • 35 Mukhopadhyay S, Philip AT, Stoppacher R. Pathologic findings in novel influenza A (H1N1) virus (“Swine Flu”) infection: contrasting clinical manifestations and lung pathology in two fatal cases. Am J Clin Pathol 2010; 133 (3) 380-387
  • 36 Kim EA, Lee KS, Primack SL , et al. Viral pneumonias in adults: radiologic and pathologic findings. Radiographics 2002; 22 (Spec No) S137-S149
  • 37 Barberà JA, Martín-Campos JM, Ribalta T , et al. Undetected viral infection in diffuse alveolar damage associated with bone marrow transplantation. Eur Respir J 1996; 9 (6) 1195-1200
  • 38 Nash G, Fligiel S. Pathologic features of the lung in the acquired immune deficiency syndrome (AIDS): an autopsy study of seventeen homosexual males. Am J Clin Pathol 1984; 81 (1) 6-12
  • 39 Andrade ZR, Garippo AL, Saldiva PH, Capelozzi VL. Immunohistochemical and in situ detection of cytomegalovirus in lung autopsies of children immunocompromised by secondary interstitial pneumonia. Pathol Res Pract 2004; 200 (1) 25-32
  • 40 Siminovich M, Murtagh P. Acute lower respiratory tract infections by adenovirus in children: histopathologic findings in 18 fatal cases. Pediatr Dev Pathol 2011; 14 (3) 214-217
  • 41 Ebbert JO, Limper AH. Respiratory syncytial virus pneumonitis in immunocompromised adults: clinical features and outcome. Respiration 2005; 72 (3) 263-269
  • 42 Garrido L, Mata-Essayag S, Hartung de Capriles C, Eugenia Landaeta M, Pacheco I, Fuentes Z. Pulmonary histoplasmosis: unusual histopathologic findings. Pathol Res Pract 2006; 202 (5) 373-378
  • 43 Parambil JG, Myers JL, Lindell RM, Matteson EL, Ryu JH. Interstitial lung disease in primary Sjögren syndrome. Chest 2006; 130 (5) 1489-1495
  • 44 Parambil JG, Myers JL, Ryu JH. Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases. Chest 2006; 130 (2) 553-558
  • 45 Tsuchiya Y, Takayanagi N, Sugiura H , et al. Lung diseases directly associated with rheumatoid arthritis and their relationship to outcome. Eur Respir J 2011; 37 (6) 1411-1417
  • 46 Douglas WW, Tazelaar HD, Hartman TE , et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001; 164 (7) 1182-1185
  • 47 Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV. Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 1990; 141 (3) 727-733
  • 48 Muir TE, Tazelaar HD, Colby TV, Myers JL. Organizing diffuse alveolar damage associated with progressive systemic sclerosis. Mayo Clin Proc 1997; 72 (7) 639-642
  • 49 Yousem SA, Gibson K, Kaminski N, Oddis CV, Ascherman DP. The pulmonary histopathologic manifestations of the anti-Jo-1 tRNA synthetase syndrome. Mod Pathol 2010; 23 (6) 874-880
  • 50 Camus P, Kudoh S, Ebina M. Interstitial lung disease associated with drug therapy. Br J Cancer 2004; 91 (Suppl. 02) S18-S23
  • 51 Rosenow III EC, Myers JL, Swensen SJ, Pisani RJ. Drug-induced pulmonary disease. An update. Chest 1992; 102 (1) 239-250
  • 52 Myers JL, Limper AH, Swensen SJ. Drug-induced lung disease: a pragmatic classification incorporating HRCT appearances. Semin Respir Crit Care Med 2003; 24 (4) 445-454
  • 53 Myers JL, Kennedy JI, Plumb VJ. Amiodarone lung: pathologic findings in clinically toxic patients. Hum Pathol 1987; 18 (4) 349-354
  • 54 Camus P, Fanton A, Bonniaud P, Camus C, Foucher P. Interstitial lung disease induced by drugs and radiation. Respiration 2004; 71 (4) 301-326
  • 55 Yousem SA, Faber C. Histopathology of aspiration pneumonia not associated with food or other particulate matter: a clinicopathologic study of 10 cases diagnosed on biopsy. Am J Surg Pathol 2011; 35 (3) 426-431
  • 56 Nash G, Blennerhassett JB, Pontoppidan H. Pulmonary lesions associated with oxygen therapy and artifical ventilation. N Engl J Med 1967; 276 (7) 368-374
  • 57 Afessa B, Peters SG. Noninfectious pneumonitis after blood and marrow transplant. Curr Opin Oncol 2008; 20 (2) 227-233
  • 58 Fischer A, West SG, Swigris JJ, Brown KK, du Bois RM. Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest 2010; 138 (2) 251-256
  • 59 El-Zammar O, Rosenbaum P, Katzenstein AL. Proliferative activity in fibrosing lung diseases: a comparative study of Ki-67 immunoreactivity in diffuse alveolar damage, bronchiolitis obliterans-organizing pneumonia, and usual interstitial pneumonia. Hum Pathol 2009; 40 (8) 1182-1188
  • 60 Pratt PC, Roggli VL. Acute interstitial pneumonia [letter]. Am J Surg Pathol 1987; 11 (2) 156-157
  • 61 Hansell DM. Acute interstitial pneumonia: clues from the white stuff. Am J Respir Crit Care Med 2002; 165 (11) 1465-1466
  • 62 Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases. Chest 1993; 103 (6) 1808-1812
  • 63 Hyzy R, Huang S, Myers J, Flaherty K, Martinez F. Acute exacerbation of idiopathic pulmonary fibrosis. Chest 2007; 132 (5) 1652-1658
  • 64 Collard HR, Moore BB, Flaherty KR , et al; Idiopathic Pulmonary Fibrosis Clinical Research Network Investigators. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007; 176 (7) 636-643
  • 65 Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 2005; 128 (5) 3310-3315
  • 66 Raghu G, Collard HR, Egan JJ , et al; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183 (6) 788-824
  • 67 Meduri GU, Headley AS, Golden E , et al. Effect of prolonged methylprednisolone therapy in unresolving acute respiratory distress syndrome: a randomized controlled trial. JAMA 1998; 280 (2) 159-165
  • 68 Suter PM. Lung Inflammation in ARDS—friend or foe?. N Engl J Med 2006; 354 (16) 1739-1742
  • 69 Steinberg KP, Hudson LD, Goodman RB , et al; National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS) Clinical Trials Network. Efficacy and safety of corticosteroids for persistent acute respiratory distress syndrome. N Engl J Med 2006; 354 (16) 1671-1684
  • 70 Peter JV, John P, Graham PL, Moran JL, George IA, Bersten A. Corticosteroids in the prevention and treatment of acute respiratory distress syndrome (ARDS) in adults: meta-analysis. BMJ 2008; 336 (7651) 1006-1009