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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 6  |  Page : 340-342

All that glitters is not gold – “Antineutrophil cytoplasmic antibodies vasculitis presenting with acute respiratory distress syndrome mimic”


1 Department of Pediatrics, SDMH, Jaipur, Rajasthan, India
2 Department of Nephrology, SDMH, Jaipur, Rajasthan, India

Date of Submission27-Jun-2020
Date of Decision22-Jul-2020
Date of Acceptance31-Jul-2020
Date of Web Publication11-Nov-2020

Correspondence Address:
Dr. Ravi Sharma
B-135, Kirti Nagar, Tonk Road, Jaipur - 302 017, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JPCC.JPCC_107_20

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  Abstract 


The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a life-threatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 13-year-old patient admitted to the intensive care unit with respiratory distress and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiation between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.

Keywords: Acute respiratory distress syndrome, antineutrophil cytoplasmic antibodies, diffuse alveolar hemorrhage, vasculitis


How to cite this article:
Sharma R, Bansal RK, Bharadia L, Mathur P. All that glitters is not gold – “Antineutrophil cytoplasmic antibodies vasculitis presenting with acute respiratory distress syndrome mimic”. J Pediatr Crit Care 2020;7:340-2

How to cite this URL:
Sharma R, Bansal RK, Bharadia L, Mathur P. All that glitters is not gold – “Antineutrophil cytoplasmic antibodies vasculitis presenting with acute respiratory distress syndrome mimic”. J Pediatr Crit Care [serial online] 2020 [cited 2020 Nov 30];7:340-2. Available from: http://www.jpcc.org.in/text.asp?2020/7/6/340/300574




  Introduction Top


Vasculitides are disorders of inflammation and necrosis of the blood vessel wall.[1] They can affect multiple organ systems with frequent respiratory and renal involvement. Their clinical manifestations are diverse, often mimicking the symptoms of much more common disorders. Thus, vasculitides remain an important diagnostic challenge to the clinicians, especially in critical care setting.[2]

Early recognition and treatment is important as the presence of advanced disease at diagnosis limits the potential benefit of therapy. We report the case of a 13-year-old female who was admitted in the intensive care unit (ICU) with severe respiratory dysfunction and diffuse alveolar infiltrates in chest imaging and was finally diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. They can occur in association with an underlying disease or exposure or as part of a primary vasculitides, which are entities of unknown cause.[3] Diffuse alveolar hemorrhage, characterized by the widespread extravasation of red blood cells into the pulmonary alveolar spaces, represents the most common clinical manifestation of pulmonary vasculitis.[4] Hemoptysis is regarded as the guiding clinical symptom of the syndrome. Even though the majority of patients experience a variable degree of hemoptysis, approximately one-third of all patients with diffuse alveolar hemorrhage are lacking this symptom.[5],[6] As a result, the diagnosis of pulmonary vasculitis [Figure 1]in the ICU may be delayed or be completely unrecognized. It is, therefore, essential that vasculitis is included in the differential diagnosis.
Figure 1: Kidney biopsy image

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  Case Report Top


A 13-year-old adolescent female presented with a chronic history of easy fatigability, vomiting, and bipedal edema for 6 months. She was investigated, provisionally diagnosed as nutritional anemia in outside hospital. Anemia treated with packed cell transfusions, oral iron & vitamin supplements. Despite the above measures, she continued to have anemia with the persistence of symptoms. Since the last few weeks, she complained of blood in vomitus or sputum. She was admitted to our facility for increasing blood-tinged vomiting and breathing difficulty. She had no history of fever, skin rashes, joint pain, jaundice, ascites, and major bleeding manifestation. Physical examination showed severe pallor, vitals showed normothermic, normotensive with tachycardia, and tachypnea. Chest examination showed subcostal retractions & auscultation revealed bilateral equal air entry with no adventitious sounds. Per abdomen examination showed no organomegaly. For severe anemia she was transfused with packed cell. Later she was shifted to intensive care in view of breathing difficulty. In intensive care, she had tachycardia and tachypnea with subcostal retractions. Chest X-ray showed bilateral heterogeneous opacities. Bronchoalveolar lavage (BAL) and computed tomography of the thorax could not be done in view of respiratory distress S/F ratio at admission <100 and two-dimensional echocardiography was normal.

Clinically, viral pneumonia was suspected, and she was kept on noninvasive ventilation (NIV) for respiratory distress. Blood smear for H1N1 was taken, and she was put on intravenous antibiotics and antiviral (oseltamivir). Relevant investigations were sent which showed very low hemoglobin (Hb) (4.2 g/dl), raised serum creatinine (4.4 mg/dl) with microscopic hematuria, and proteinuria. Pulmonologist and nephrologist opinions were seeked, and they advised workup for vasculitis. Anti nuclear antibody (ANA) profile, complement levels, and c- and p-ANCA were sent. ANA was negative, C3 and C4 were within normal limits, but p-ANCA was positive. C-ANCA was negative. She was started on immunosuppressive treatment; pulse methylprednisolone was given for 3 days. Induction was given with rituximab 375 mg/m2. The patient had dramatic clinical and radiological improvement. In the next 48 h, she was off NIV; renal functions started improving with improvement in appetite. She was then shifted to the general ward where the renal biopsy was performed which showed pauci-immune necrotizing crescentic glomerulonephritis consistent with ANCA vasculitis. The patient was discharged on oral steroids, hydroxychloroquine, and was planned for weekly rituximab for 4 weeks. On follow-up, she was asymptomatic with improvement in Hb and renal function.


  Discussion Top


Systemic vasculitides are characterized by histological evidence of blood vessel inflammation. They can occur in association with an underlying disease or exposure or as part of a primary vasculitides, which are entities of unknown cause.[3] ANCA-associated vasculitis is necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels and is characterized by the presence of circulating ANCA specific for myeloperoxidase or PR3-ANCA.[7] The clinical presentation of vasculitides is highly variable extending from self-limited illness to critical or even life-threatening complications, including diffuse alveolar hemorrhage and glomerulonephritis. Diffuse alveolar hemorrhage, characterized by the widespread extravasations of red blood cells into the pulmonary alveolar spaces, represents the most common clinical manifestation of pulmonary vasculitis.[4] In a series of 37 patients with the syndrome requiring ICU support, diffuse alveolar hemorrhage was an unexpected diagnosis in most patients, and the diagnosis had not been considered before BAL analysis.[8] It is, therefore, essential that vasculitis is included in the differential diagnosis of unexplained, persistent pulmonary infiltrates.

Since the introduction of combination immunosuppressive therapy, consisting of corticosteroids, cyclophosphamide, and newer agents like rituximab, the prognosis of patients with vasculitis has been improved. While potentially lifesaving, the aforementioned treatments are not without significant risk. Notably, the prognosis of vasculitis patients in the intensive care setting remains poor.[2],[9],[10] For example, for patients admitted to the ICU with suspected pulmonary vasculitis, a mortality of 25%–50% has been reported.[11] One of the main reasons for this finding might be the enhanced susceptibility to infections when under treatment in the ICU with high doses of immunosuppressive agents. Risk factors that have been associated with an unfavorable outcome of critical patients with connective tissue disease are higher APACHE II scores at the ICU admission, longer stay in the ICU (>10 days),[12] and the presence of respiratory failure.[13] Fortunately, all of the aforementioned factors were not present in our patient which lead to favorable outcome in our patient.


  Conclusion Top


Alveolar hemorrhage should be considered in the differential diagnosis of acute respiratory distress syndrome. Early diagnosis and prompt initiation of treatment is vital to prevent irreversible critical organ injury.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lapraik C, Watts R, Bacon P, Carruthers D, Chakravarty K, D'Cruz D, et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford) 2007;46:1615-6.  Back to cited text no. 1
    
2.
Wilfong EM, Seo P. Vasculitis in the intensive care unit. Best Pract Res Clin Rheumatol 2013;27:95-106.  Back to cited text no. 2
    
3.
Langford CA. Vasculitis. J Allergy Clin Immunol 2010;125:S216-25.  Back to cited text no. 3
    
4.
Krause ML, Cartin-Ceba R, Specks U, Peikert T. Update on diffuse alveolar hemorrhage and pulmonary vasculitis. Immunol Allergy Clin North Am 2012;32:587-600.  Back to cited text no. 4
    
5.
de Prost N, Parrot A, Picard C, Ancel PY, Mayaud C, Fartoukh M, et al. Diffuse alveolar haemorrhage: Factors associated with in-hospital and long-term mortality. Eur Respir J 2010;35:1303-11.  Back to cited text no. 5
    
6.
Diaz J, Calamia KT, Lee AS. Pulmonary vasculitis in the intensive care unit. J Intensive Care Med 2011;26:88-104.  Back to cited text no. 6
    
7.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65:1-1.  Back to cited text no. 7
    
8.
Rabe C, Appenrodt B, Hoff C, Ewig S, Klehr HU, Sauerbruch T, et al. Severe respiratory failure due to diffuse alveolar hemorrhage: Clinical characteristics and outcome of intensive care. J Crit Care 2010;25:230-5.  Back to cited text no. 8
    
9.
Cruz BA, Ramanoelina J, Mahr A, Cohen P, Mouthon L, Cohen Y, et al. Prognosis and outcome of 26 patients with systemic necrotizing vasculitis admitted to the intensive care unit. Rheumatology (Oxford) 2003;42:1183-8.  Back to cited text no. 9
    
10.
Semple D, Keogh J, Forni L, Venn R. Clinical review: Vasculitis on the intensive care unit-part 1: Diagnosis. Crit Care 2005;9:92-7.  Back to cited text no. 10
    
11.
Griffith M, Brett S. The pulmonary physician in critical care illustrative case 3: Pulmonary vasculitis. Thorax 2003;58:543-6.  Back to cited text no. 11
    
12.
Burkhardt O, Köhnlein T, Wrenger E, Lux A, Neumann KH, Welte T. Predicting outcome and survival in patients with Wegener's granulomatosis treated on the intensive care unit. Scand J Rheumatol 2007;36:119-24.  Back to cited text no. 12
    
13.
Lee J, Yim JJ, Yang SC, Yoo CG, Kim YW, Han SK, et al. Outcome of patients with connective tissue disease requiring intensive care for respiratory failure. Rheumatol Int 2012;32:3353-8.  Back to cited text no. 13
    


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