Hamman-Rich Syndrome (Hamman Rich Syndrome)

Acute idiopathic interstitial pneumonitis characterized by diffuse PULMONARY ALVEOLI damage with uniform edematous connective tissue proliferation. It is often associated with extensive fibroblastic distortion of the lung parenchyma and leads to ADULT RESPIRATORY DISTRESS SYNDROME in later stages.

Also Known As:

Networked: 164 relevant articles (12 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Dermatomyositis (Dermatopolymyositis)
2.	Non-Small-Cell Lung Carcinoma (Carcinoma, Non-Small Cell Lung)
3.	Thrombocytopenia (Thrombopenia)
4.	Pericardial Effusion
5.	Idiopathic Pulmonary Fibrosis

Experts

1.	Ishiguro, Takashi: 3 articles (01/2019 - 09/2010)
2.	Takayanagi, Noboru: 3 articles (01/2019 - 09/2010)
3.	Lee, Kyuhong: 2 articles (11/2019 - 01/2018)
4.	Kanauchi, Tetsu: 2 articles (01/2019 - 09/2010)
5.	Shimizu, Yoshihiko: 2 articles (01/2019 - 12/2011)
6.	Hayashi, Tomayoshi: 2 articles (03/2014 - 10/2006)
7.	Kawabata, Yoshinori: 2 articles (12/2011 - 10/2007)
8.	Sugita, Yutaka: 2 articles (12/2011 - 09/2010)
9.	Chung, Man Pyo: 2 articles (11/2011 - 03/2006)
10.	Lee, Kyung Soo: 2 articles (11/2011 - 03/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hamman-Rich Syndrome:

1.	SteroidsIBA 05/01/1986 - "Two main mechanisms of ventilatory impairment seem to occur in children with SLE: firstly, acute interstitial pneumonia, which rapidly improves on treatment with steroids, and, secondly, restriction of lung volume, mainly due to respiratory muscle weakness." 03/01/2009 - "Acute interstitial pneumonitis, so called DAD in patients with PM/DM is a resistant of steroid therapy and a poor prognostic complication. " 07/01/2001 - "[Acute interstitial pneumonia: favorable outcome with steroid therapy]." 05/01/1957 - "Case report: diffuse interstitial pulmonary fibrosis (Hamman-Rich syndrome); diagnosis by lung biopsy; treated with cortico-steroids." 09/01/2016 - "We describe a rare presentation of this condition with regard to the uncharacteristically acute nature of presentation, acute decompensation in clinical condition, development of acute interstitial pneumonitis requiring rescue extracorporeal membrane oxygenation (ECMO) and accompaniment of significant pericardial effusion on presentation, followed by rapid improvement with initiation of steroids. "
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2000 - "A case of probable acute interstitial pneumonia with a dramatic response to pulse corticosteroid administration." 12/01/2011 - "We diagnosed acute interstitial pneumonia and initiated corticosteroid therapy. " 09/01/1994 - "Corticosteroid therapy was effective for acute interstitial pneumonia, but in-effective for thrombocytopenia. " 01/01/2008 - "The stimulation index of the drug lymphocyte stimulation test with shiniseihaito increased after corticosteroid therapy had been tapered and suggested that the mechanism of acute interstitial pneumonia in this case was allergic reaction induced by shiniseihaito. " 10/01/2002 - "He presented with a respiratory distress syndrome due to acute interstitial pneumonitis from which he promptly recovered with corticosteroid therapy."
3.	Gefitinib (Iressa)FDA Link 01/01/2014 - "This fact suggested that the cases of acute interstitial pneumonia were included in the clinical trial before the approval of gefitinib. " 01/01/2006 - "Despite gefitinib being discontinued, high-resolution CT revealed extension of GGO and restructuring of lung parenchyma, suggesting acute interstitial pneumonia. " 01/11/2003 - "Severe acute interstitial pneumonia and gefitinib." 01/11/2003 - "We assessed four patients who had non-small cell lung cancer causing severe acute interstitial pneumonia in association with gefitinib. " 09/01/2005 - "Short interval from the initiation of gefitinib treatment to the onset of interstitial lung disease, acute interstitial pneumonia pattern, and the presence of pre-existing pulmonary fibrosis were associated with poor prognosis. "
4.	Prednisolone (Predate)FDA LinkGeneric 05/01/1997 - "Oral prednisolone treatment was started and the retinopathy was improved, but was complicated by acute interstitial pneumonia. " 07/18/1959 - "Report of a case of diffuse interstitial fibrosis of the lungs (Hamman-Rich syndrome) successfully controlled by prednisolone." 05/01/2020 - "The patient was diagnosed with acute interstitial pneumonitis, and effectively treated with steroid pulse therapy followed by prednisolone. " 01/01/2012 - "Coadministration of cyclosporin a with prednisolone in acute interstitial pneumonia complicating polymyositis/dermatomyositis."
5.	Tyrosine Kinase InhibitorsIBA 03/15/2011 - "Although the EGFR-tyrosine kinase inhibitor gefitinib is effective against advanced non-small cell lung cancer with EGFR mutations, some patients treated with this agent develop severe acute interstitial pneumonia. " 04/01/2013 - "Acute interstitial pneumonia is one of serious side effects of epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) treatment, while it often has significant clinical benefit in cancer patients. " 01/01/2019 - "Although inhibition of epidermal growth factor receptor (EGFR)-mediated cell signaling by the EGFR tyrosine kinase inhibitor gefitinib is highly effective against advanced non-small cell lung cancer, this drug might promote severe acute interstitial pneumonia. "
6.	GlucocorticoidsIBA 04/23/2014 - "On the basis of the preliminary diagnosis of acute interstitial pneumonia, high-dose glucocorticoid was used. " 01/01/2011 - "It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. " 07/01/2020 - " Points• Patients with coronavirus disease 19 (COVID-19) may have features mimicking rheumatic diseases, such as arthralgias, acute interstitial pneumonia, myocarditis, leucopenia, lymphopenia, thrombocytopenia and cytokine storm with features akin to secondary hemophagocytic lymphohistiocytosis.• Although preliminary results may be encouraging, high-quality clinical trials are needed to better understand the role of drugs commonly used in rheumatology like hydroxychloroquine and tocilizumab in COVID-19.• Until further evidence emerges, it may be cautiously recommended to continue glucocorticoids and other disease-modifying antirheumatic drugs (DMARDs) in patients receiving these therapies, with discontinuation of DMARDs during infections as per standard practice."
7.	Infliximab (Remicade)FDA Link 10/01/2013 - "Efficacy of infliximab in the treatment for dermatomyositis with acute interstitial pneumonia: a study of fourteen cases and literature review." 05/12/2017 - "A 53-year-old man with ulcerative colitis (UC) suffered fatal acute interstitial pneumonitis (AIP) post completing an accelerated infliximab induction course. " 05/12/2017 - "A 53-year-old man with dyspnoea, respiratory failure, consistent with infliximab-induced acute interstitial pneumonitis after an accelerated induction dosing schedule." 10/01/2013 - "This retrospective study assessed the efficacy of infliximab therapy in 14 patients with dermatomyositis with acute interstitial pneumonia. "
8.	Cyclosporine (Ciclosporin)FDA LinkGeneric 12/01/2000 - "Successful use of cyclosporin A for the treatment of acute interstitial pneumonitis associated with rheumatoid arthritis." 09/01/2003 - "Efficacy of combination treatment with cyclosporin A and corticosteroids for acute interstitial pneumonitis associated with dermatomyositis." 01/01/2012 - "Coadministration of cyclosporin a with prednisolone in acute interstitial pneumonia complicating polymyositis/dermatomyositis." 01/01/2013 - "Successful combination therapy with corticosteroids, biweekly intravenous pulse cyclophosphamide and cyclosporin A for acute interstitial pneumonia in patients with dermatomyositis : report of three cases."
9.	Sorafenib (BAY 43-9006)FDA Link 04/01/2015 - "However, 19 days after sorafenib treatment was resumed, acute interstitial pneumonia developed. " 01/01/2012 - "Sorafenib-induced acute interstitial pneumonia in patients with advanced hepatocellular carcinoma: report of three cases." 01/01/2012 - "Little is known about acute interstitial pneumonia (AIP) induced by sorafenib therapy in patients with advanced hepatocellular carcinoma (HCC). " 12/01/2010 - "On the 24th day of sorafenib treatment, the patient developed acute interstitial pneumonitis that rapidly improved after the discontinuation of sorafenib and treatment with high-dose steroids. "
10.	ErbB Receptors (EGF Receptor)IBA 04/01/2013 - "Acute interstitial pneumonia is one of serious side effects of epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitor (TKI) treatment, while it often has significant clinical benefit in cancer patients. " 01/01/2019 - "Although inhibition of epidermal growth factor receptor (EGFR)-mediated cell signaling by the EGFR tyrosine kinase inhibitor gefitinib is highly effective against advanced non-small cell lung cancer, this drug might promote severe acute interstitial pneumonia. "

Therapies and Procedures

1.	Therapeutics 10/01/2007 - "[Case of acute interstitial pneumonia that responded to therapy but relapsed six months later]." 04/01/1959 - "[Acute interstitial pneumonia; clinical aspects and therapy]." 11/01/2011 - "Poor prognoses are expected when there is a short interval from the initiation of target therapy to the onset of ILD, acute interstitial pneumonia pattern of ILD, and preexisting pulmonary fibrosis." 12/01/2006 - "In the more severe forms of IIP, such as idiopathic pulmonary fibrosis and acute interstitial pneumonia, no effective therapies have been identified. " 01/01/2011 - "It is not clear if glucocorticoid therapy is effective in acute interstitial pneumonia. "
2.	Extracorporeal Membrane Oxygenation 12/01/2017 - "Acute interstitial pneumonitis requiring extracorporeal membrane oxygenation and lung transplantation in an adolescent patient." 05/01/2018 - "Extracorporeal Membrane Oxygenation for Refractory Severe Respiratory Failure in Acute Interstitial Pneumonia." 09/01/2016 - "We describe a rare presentation of this condition with regard to the uncharacteristically acute nature of presentation, acute decompensation in clinical condition, development of acute interstitial pneumonitis requiring rescue extracorporeal membrane oxygenation (ECMO) and accompaniment of significant pericardial effusion on presentation, followed by rapid improvement with initiation of steroids. "
3.	Drug Therapy (Chemotherapy) 05/01/1985 - "A good response to chemotherapy was obtained but she died of acute interstitial pneumonitis. " 09/01/2005 - "Acute interstitial pneumonitis during chemotherapy for haematological malignancy." 11/01/2009 - "When diffuse alveolar damage was attributed to tumor treatment with chemotherapy or to drug toxicity, 3 of 16 patients showed multiple organ dysfunction syndrome; 15 of 16 showed interstitial myofibroblast proliferation, 3 of 3 acute interstitial pneumonia patients did not show multiple organ dysfunction syndrome; and 3 of 3 acute interstitial pneumonia showed marked interstitial myofibroblast proliferation. " 10/01/2006 - "The site of the tumor (right side), preoperative radiation or chemotherapy, pneumonectomy, blood transfusion, and intraoperative complication were independent risk factors for the incidence of acute interstitial pneumonia (P=0.001, 0.0484, 0.0012, 0.0002, 0.0003, respectively) in the multivariate analysis. " 09/01/2005 - "Fourteen adult patients with haematological malignancies (eight non-Hodgkin's lymphoma, one multiple myeloma, one chronic lymphocytic leukaemia, two acute lymphoblastic leukaemia and two acute myeloid leukaemia) developed acute interstitial pneumonitis (IP) during the course of chemotherapy. "
4.	Total Parenteral Nutrition 09/01/1982 - "Scanning and transmission electron microscopic studies on two autopsy cases of acute interstitial pneumonitis caused probably by anticancer agents and intravenous hyperalimentation were reported. "
5.	Lung Transplantation 11/01/1996 - "Partial resolution of acute interstitial pneumonia in native lung after single lung transplantation." 12/01/2017 - "Acute interstitial pneumonitis requiring extracorporeal membrane oxygenation and lung transplantation in an adolescent patient." 05/01/2018 - "A 51-year-old woman with dermatomyositis underwent lung transplantation for acute interstitial pneumonitis. " 10/01/2022 - "Acute Interstitial Pneumonia (Hamman-Rich Syndrome) in Lung Transplantation: A Case Series."