Clinical diagnosis
Case 94
【Progress】She was given blood transfusion, acute hemodialysis and steroid pulse treatment. These treatments brought about transient improvements of her symptoms. However, she passed away approximately one month later.
【Discussion】
Acute respiratory distress syndrome (ARDS) is a condition that alveolar spaces are diffusely occupied by protein fluid, whole blood, foreign body or others. Cardiogenic pulmonary edema is excluded from ARDS. Etiology of ARDS is categorized into pulmonary and extra-pulmonary. As pulmonary origins, pneumonia, pulmonary injury and inhalation of food and fluids are listed (1 - 6). As extra-pulmonary origins, sepsis, massive blood transfusion and severe systemic injury are listed (1 - 6). Then, the exact cause can be difficult to differentiate by radiological findings alone. Of these causes, sepsis is most often, followed by trauma and food aspiration (1 - 6). As pathology-etiology, alveolar damages are usually found. Leukocytes release protein-lytic enzyme and active oxygen which injure alveoli and enhance permeability of capillaries, inducing non-cardiogenic edema. In our case, ARDS is caused by one of the extrapulmonary origins, antineutrophilic cytoplasmic antibody (ANCA) associated pneumonia which induces for neutrophils to release these chemo-substances, inducing permeability of vessels and acinar bleeding.
According to Berlin definition of ARDS (6), the onset is; acute with over 1 week or less; chest radiograph or CT reveals bilateral opacities consistent with pulmonary edema; symptoms and signs were not fully explained by cardiac failure and fluid overload: PF ratio < 300mmHg with a minimum of 5 to 20 cm H2O PEEP. PF ratio was calculated as a formula: oxygen partial pressure (PO2 mmHg) / fraction of inspiratory oxygen (Fi, decimal point). In a healthy condition, PF ratio is approximately 480 (100 / 0.21). The severity degree of ARDS is based on PF ratio; mild, PF 200 to 300; moderate, PF 100 to 200: severe, less than 100 (6). Mortality rate was up to 50% (6) and increased accompanied with ARDS severity (Table 1). Mild ARDS is called acute lung injury (ALI) as another name (1 – 5). Our patient was transported probably at the day of the onset, chest CT showed bilateral consolidation in the whole lung except left upper lobe, and PF ratio was 71.8 (66.5 / 0.926) under 5 cmH2O PEEP implying to meet a severe stage of ARDS. Unfortunately, our patient passed away approximately one month later after careful treatment.
Pulmonary renal syndrome features diffuse alveolar damages and necrotizing glomerular nephritis (7 -11). The three most known causes of pulmonary renal syndrome are ANCA positive vasculitis, anti-glomerular membrane disease (Goodpasture disease) and systemic lupus erythematosus (SLE). Three major ANCA positive vasculitis diseases are microscopic polyangiitis, granulomatosis with polyangiitis (Wegener) and eosinophilic granulomatosis with polyangiitis (previously known as Churg-Strauss syndrome)(7 - 11). In our case, para-sinusitis was not found and laboratory test revealed no evidence of eosinophilia, meeting microscopic polyangiitis rather than granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis.
【Summary】
We present an eighty three-year-old female suffering from sudden dyspnea and renal disorder. Chest CT showed bilateral consolidation in the whole lung except left upper lobe and laboratory test revealed creatinin : 3.31mg/dL, PO2 66.5 mm Hg and fraction of inspiratory oxygen (FiO2) 0.926, PF ratio 71.8 indicating pulmonary renal syndrome associated with severe ARDS. Further it revealed MPO-ANCA > 274 U/mL (< 3.5), indicating ANCA positive polyangiitis. It is borne in mind that the definition of ARDS which is PF ratio is less than 300, and ARDS is caused by leukocytes release protein-lytic enzyme and active oxygen which injure alveoli and enhance permeability of capillaries, inducing non-cardiogenic edema. ARDS occurs after sepsis, food inhalation, trauma and so on. Pulmonary renal syndrome includes ANCA positive polyangiitis, anti-glomerular membrane disease and SLE. As ANCA positive polyangiitis, microscopic polyangiitis, granuloma with polyangiitis and eosinophilic granuloma with polyangiitis. Our case was not contradictory to meet the diagnostic criteria of microscopic polyangiitis.
【References】
1.Wheeler AP, Bernard GR. "Acute lung injury and the acute respiratory distress syndrome: a clinical review." Lancet. 2007 May 5;369(9572):1553-64. Review. PMID 17482987
2.Bernard GR. 'Acute respiratory distress syndrome: a historical perspective'. Am J Respir Crit Care Med. 2005 Oct 1;172(7):798-806. Epub 2005 Jul 14. PMID 16020801
3.Netzer G, Shah CV, Iwashyna TJ, Lanken PN, Finkel B, Fuchs B, Guo W, Christie JD. "Association of RBC transfusion with mortality in patients with acute lung injury." Chest. 2007 Oct;132(4):1116-23. Epub 2007 May 15. PMID 17505028
4.Rubenfeld, GD, Caldwell, E, Peabody, E, et al "Incidence and outcomes of acute lung injury." NEJM. 2005;353,1685-1693
5.Gordon D. Rubenfeld, et al. "Epidemiology and Outcomes of Acute Lung Injury." Chest. 2007; 131:554-562
6.Ferguson ND, Fan E, Camporota L et-al. The Berlin definition of ARDS: an expanded rationale, justification, and supplementary material. Intensive Care Med. 2012;38 (10): 1573-82. doi:10.1007/s00134-012-2682-1 - Pubmed citation
7.McCabe C, et al. Pulmonary-renal syndromes: an update for respiratory physicians. Respiratory medicine 2011; 105: 1413-1421.
8.ANCA associated vasculitis-Diagnostic and therapeutic guideline 2014 (in Japanese) minds4.jcqhc.or.jp/minds/ANCA/anca.pdf
9.Jennette JC, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65:1–11.
10.Ando M, et al. Incidence of myeloperoxidase anti-neutrophil cytoplasmic antibody positivity and microscopic polyangitis in the course of idiopathic pulmonary fibrosis. Respir Med. 2013;107:608-615. doi: 10.1016/j.rmed.2013.01.006. Epub 2013 Feb 19.
11.Tanaka T, et al. Interstitial pneumonia associated with MPO-ANCA: clinicopathological features of nine patients. Respir Med. 2012;106:1765-1770. doi: 10.1016/j.rmed.2012.08.024. Epub 2012 Sep 17.
2018.2.28