Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2011, 155(4):403-407 | DOI: 10.5507/bp.2011.039

ACUTE INTERSTITIAL PNEUMONIA (HAMMAN-RICH SYNDROME) IN IDIOPATHIC PULMONARY FIBROSIS AND BRONCHOALVEOLAR CARCINOMA: A CASE REPORT

Jiri Plasek^a, Jana Dvorackova^b, Jan Jahoda^c, Kristina Trulikova^d, Radka Mokosova^e, Tomas Danek^f, Vladimir Hrabovsky^a, Arnost Martinek^a

^a Clinic of Internal Medicine, Faculty of Medicine, University of Ostrava and University Hospital Ostrava, Czech Republic

^b Institute of Pathology, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

^c Clinic of Anaesthesiology and Resuscitation, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

^d Institute of Radiology, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

^e Clinic of Tuberculosis and Respiratory Diseases, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

^f Cardiac Surgery Center, Faculty of Medicine, University of Ostrava and University Hospital Ostrava

Aim: Acute interstitial pneumonia is characterized by rapid progressive dyspnoea degenerating into respiratory failure requiring mechanical ventilation. Acute interstitial pneumonia (AIP) and idiopathic pulmonary fibrosis (IPF) are separate clinic/pathological entities although overlap may be present. It is well-known that patients with IPF have increased risk of lung carcinoma; Adenocarcinoma in connection with IPF is less common. Moreover the subtype of adenocarcinoma, diffuse bronchoalveolar carcinoma has not yet been described.

Case report: We report the case of 45 yr old former hockey player with increased bilateral reticular shadowing on chest radiograph, dyspnoea, velcro-like crackles, restrictive respiratory disease and mixed high-resolution computed tomography finding. During brief in-patient treatment the patient developed acute respiratory failure accompanied by multiorgan failure and disseminated coagulopathy. Deterioration of the microcirculation was followed by loss of peripheral vascular resistance, which was irreversible even with normalization of the blood gases achieved by extracorporeal membrane oxygenation. At autopsy, bronchoalveolar carcinoma in usual interstitial pneumonia (UIP) combined with areas of alveolar damage with hyaline membranes was found.

Conclusion: This case alerts clinicians to unusual idiopathic pulmonary fibrosis manifestations and its complications. Close collaboration between clinicians, pathologists and laboratory physicians is highly recommended for early diagnosis and appropriate treatment.

Keywords: Acute Respiratory Failure, Interstitial Lung Disease, Adenocarcinoma

Received: March 15, 2011; Accepted: July 11, 2011; Prepublished online: September 8, 2011; Published: December 1, 2011  Show citation