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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 1  |  Issue : 2  |  Page : 70-73

Systemic lupus erythematosus with pregnancy and acute respiratory distress


1 Department of Critical Care, Al-Noor Specialist Hospital, Makkah, Saudi Arabia
2 Department of Rheumatology, Al-Noor Specialist Hospital, Makkah, Saudi Arabia

Date of Web Publication7-Nov-2017

Correspondence Address:
Ahmad Banjar
Al-Noor Specialist Hospital, P.O. Box: 13656, Makkah 21955
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sccj.sccj_16_17

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  Abstract 

Systemic lupus erythematosus (SLE) with pregnancy has a high risk of maternal and fetal morbidity and mortality once they get admitted to critical care unit. We report a case of a pregnant women complicated with lupus nephritis and pneumonitis followed by acute respiratory distress leading to cardiopulmonary arrest. During her treatment in Intensive Care Unit, maternal respiratory function was improved after proper management of systemic lupus flare up and acute respiratory distress syndrome with timely induction of pregnancy.

Keywords: Acute respiratory distress syndrome, lupus flare, lupus nephritis, systemic lupus erythematosus in pregnancy


How to cite this article:
Banjar A, Rasheed J, Alkhatib K, Omran N. Systemic lupus erythematosus with pregnancy and acute respiratory distress. Saudi Crit Care J 2017;1:70-3

How to cite this URL:
Banjar A, Rasheed J, Alkhatib K, Omran N. Systemic lupus erythematosus with pregnancy and acute respiratory distress. Saudi Crit Care J [serial online] 2017 [cited 2022 Dec 8];1:70-3. Available from: https://www.sccj-sa.org/text.asp?2017/1/2/70/217808


  Introduction Top


Systemic lupus erythematosus (SLE) is an idiopathic autoimmune condition which has multiorgan involvement. Diagnosis is based on both clinical manifestations and laboratory indices. The disease course can be sporadic and unpredictable but is typically characterized by periods of relapse and remission. This article discusses the maternal complications of SLE and management of the disease during pregnancy. When SLE has been quiescent and when renal disease has been in remission for at least 6 months before the pregnancy, the majority of pregnant lupus patients are able to carry on uneventfully till term and deliver a healthy infant.[1] Although the risk of a lupus flare is not increased in pregnant women when compared to nonpregnant women, lupus flares can occur during pregnancy or immediately following delivery.[2]


  Case Report Top


A 24-year-old woman gravida 2 para 1 had a 3-year history of SLE, diagnosed based on laboratory and clinical criteria including (photosensitivity, arthritis positive antinuclear antibody (ANA), and positive anti-double-stranded DNA (anti-dsDNA). She was in remission for the past 2 years on prednisolone and hydroxychloroquine. She lost follow-up and defaulted from the treatment.

She got pregnant and presented in hospital at 22nd week of gestation with fatigue, shortness of breath, productive cough, lower limbs edema, and high blood pressure. She was admitted at our hospital (a 500 bedded referral hospital in the western region) for further assessment and management. She was tachypnic, required oxygen by face mask, laboratory investigations showed liver function test, prothrombin time, international normalized ratio, and partial thromboplastin time within normal range, renal function (urea 67, creatinine 1.3), high erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), pancytopenia, positive coombs test +3, peripheral blood film: normocytic normochromic anemia, lymphopenia, thrombocytopenia, low level of complement C3/C4, negative anticardiolipin IgG and IgM, normal lupus anticoagulant confirmatory test, urinalysis (active sediment with protein +2 and blood +3, 24 h urine for protein: 7 g).

Chest X-ray (CXR) revealed lung infiltration and because it was difficult to distinguish between lupus pneumonitis and atypical infection, treatment with high-dose prednisolone with empirical injectable antibiotic tazocin (piperacillin and tazobactam) were started. The patient was in respiratory distress on high-flow oxygen by rebreathing mask, therefore shifted to Intensive Care Unit (ICU). Clinically, the patient was conscious oriented on 15 L oxygen restless tachypnic (RR 30/min), SpO2 94%; there was frothy sputum with bilateral coarse crepitation, CXR revealed progressive infiltration bilaterally [Figure 1]; therefore, the patient was intubated and connected to conventional mechanical ventilation (MV).
Figure 1: Chest radiograph at 22-week gestation shows bilateral interstitial infiltration more in the right lung

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The empirical antibiotics upgraded to injectable imipenem, metronidazole, and vancomycin.

Because the critical condition of the patient, after furnishing all the legal and ethical formalities, a pregnancy induction was decided and made by a multiprofessional committee. Using induced vaginal delivery, in ICU, the pregnancy was induced by obstetric and pediatric consultants. The baby was preterm, viable hence admitted to Neonatal ICU.

Unfortunately, the mother developed cardiac arrest after delivery; cardiopulmonary resuscitation was performed for 15 min then reverted.

Pulmonary emboli suspected and heparin infusion was started. The patient developed hypotension requiring norepinephrine infusion. Therapeutic hypothermia (TH) was applied to keep body temperature between 32°C and 34°C to prevent ischemic brain damage postcardiac arrest. A spiral CT scan chest was negative for pulmonary embolism. Echocardiogram reported normal left ventricle function, ejection fraction = 60%, sever mitral regurgitation and tricuspid regurgitation, pulmonary artery pressure was 63 mmHg. However, there was no significant improvement of respiratory function. Later, bronchoalveolar lavage (BAL) reported bloody smear with acute inflammatory cells, negative for cytological malignancy, and negative for culture. The patient was started on aggressive treatment by for lupus nephritis and lupus pneumonitis with methylprednisolone 1 g IV daily for 5 days, cyclophosphamide 1 g single dose, and 5 sessions of plasmapheresis with daily monitoring by a nephrologist by continuous renal replacement therapy for the acute renal failure.

During the treatment, the patient was desaturated to SpO2 70% on conventional mechanical ventilator using 100% oxygen; CXR revealed acute respiratory distress syndrome (ARDS) picture [Figure 2], therefore connected to high-frequency oscillatory ventilation (HFOV) and showed gradual improvement in oxygenation. Two days later, shifted back to conventional ventilator again and norepinephrine was weaned off.
Figure 2: Chest radiograph shows increased bilateral alveolar infiltrate after delivery

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On day 7th of ICU admission, the patient was conscious, hemodynamically stable and CXR revealed dramatic improvement; hence, she was weaned off the MV and extubated.

The next day, postextubation, she became tachypnic on nasal cannula and desaturated with SpO2 80%, so continuous airway pressure was applied; however, the oxygenation was not improved; therefore, the patient was reintubated and connected to MV for 2 days. The CXR revealed lung congestion. The ultrafiltration of 3 L was done. The patient was improved and extubated uneventfully. She was transferred to an ordinary ward after 14 days of ICU admission.

The patient recovered well and was discharged home in a stable condition. Home medications were prednisolone slow tapering dose, mycophenolate mofetil, hydroxychloroquine, acetylsalicylic acid, angiotensin-converting-enzyme inhibitor and statins.

Renal biopsy was performed on an outpatient basis after improvement of platelet count and showed Grade 3 lupus nephritis.

She was advised for follow-up on regular basis in rheumatology clinic for disease activity assessment.


  Discussion Top


Although lupus pneumonitis is rare, seen in 1%–12% of patients, it is one of the dreadful complications of SLE.[3] A fulminant form lupus pneumonitis may occur during pregnancy.[4] The clinical and radiographic features are not specific. Other causes of alveolar infiltrates such as infectious pneumonia, alveolar hemorrhage, and pulmonary edema should be considered. Because infectious causes cannot be ruled out, empirical broad-spectrum antibiotics should be started immediately and continued until infection is excluded.[5] Infectious causes must always be ruled out in these patients before the diagnosis of acute lupus pneumonitis is entertained. This workup usually requires bronchoscopy with cultures. Biopsies consistent with lupus pneumonitis reveal acute alveolar injury.[6] The main treatment for lupus pneumonitis is systemic corticosteroid.[7] Additional immunosuppressant such as cyclophosphamide should also be considered. In patients refractory to corticosteroids, intravenous immunoglobulin, plasma exchange, or rituximab can be of some help with little evidence.[8]

Many lupus patients are immunocompromised; the primary alternative that needs to be excluded is infection. Noninvasive respiratory sampling (tracheobronchial aspiration or BAL) can obtain lower respiratory sample for microscopic analysis and microbiologic culture. Throughout the disease course, lupus activity must be monitored by a combination of clinical history, physical examination, organ-specific functional tests, and serologic studies. Anti-dsDNA titers, complement levels, and ESR are common indicators combined with disease activity indices to stratify patients at risk for flares.[9] It has been shown by multiple studies that high-resolution computed tomography scan (HRCT) is a sensitive means of detecting interstitial lung disease. It can be correlated with BAL and lung biopsy. Since the extent of morphological changes in HRCT correlates with the severity of impairment of the diffusing capacity, it allows a rough estimation of the degree of lung impairment. Serial HRCT serves as a good means for monitoring the disease progress, but its role in pinpointing the diagnosis is limited.[3] We excluded infective pneumonia in our patient by repeated sputum and single BAL fluid examination, alveolar hemorrhage by the absence of hemoptysis, and hemosiderin-laden macrophage in BAL fluid pulmonary embolism by spiral CT and the syndrome of acute reversible hypoxemia by the presence of abnormal CXR.

Because strongly positive ANA and anti-dsDNA associated with high ESR and CRP lupus and negative pathogen findings on BAL, lupus pneumonitis considered. Therefore, we applied intensive steroid treatment with plasmapheresis and cyclophosphamide to our patient, and prominent clinical response confirmed our diagnosis.

The prevalence of ARDS is 4%–15% in patients with lupus.[10] If it develops the mortality rate can reach up to 70%.[10] ARDS-related mortality contributes to 30% of all lupus deaths. The most frequent cause of ARDS is sepsis; other causes include lupus pneumonitis and diffuse alveolar hemorrhage. In lupus patients, ARDS tends to occur at a younger age and is more progressive than ARDS in non-SLE patients.[8],[10] Treatment of ARDS depends on the severity of the syndrome and underlying causing diseases.

For the treatment of pregnant women with ARDS, previous studies have suggested the best option after 28 weeks gestation is expeditious delivery,[11] yet the delivery method is controversial. We decided to deliver the fetus at 22-week gestation by induction vaginally.

MV strategy is the most important factors in improvement of this syndrome. If conventional MV is not effective, then HFOV is a rescue maneuver.

Previous randomized trials of the use of HFOV in adults with ARDS have suggested that this strategy results in improvements in oxygenation and survival, but the trials were limited by small sample sizes and outdated ventilation strategies for the control group.[12],[13] Consequently, despite the frequent use of HFOV in patients who do not have an adequate response to conventional MV and the increased use of HFOV earlier in the course of the disease, this approach remains an unproven therapy for adults with ARDS.[14],[15] Two large randomized controlled trials, OSCILLATE and OSCAR, failed to show benefit from this mode of ventilation.[16],[17] However, our patient connected to HFOV for 2 days and oxygenation was dramatically improved and was put back to conventional MV till extubation.

Ischemic brain damage commonly occurs in patients postcardiac arrest. Studies have shown that lowering brain temperature even by a few degrees decreases ischemic damage. In many studies, TH improves neurological outcomes. TH should be initiated as soon as possible after the return of spontaneous circulation with a target temperature of 32°C–34°C. There is a 20% increase in mortality for every hour of delay in the initiation of TH.[18] In this reported case, the patient responded well for 24 h to this kind of therapeutic modality.


  Conclusion Top


In this critically ill patient described above with lupus pneumonitis, ARDS, and pregnancy, a clinically significant improvement can be achieved with ICU admission and multiprofessional support team with armamentarium of rescue HFOV, induced TH, timed delivery of the pregnancy, and plasma exchange.

Conventional high-dose steroid and cytotoxic drug therapy suggest that it can be an important component of treatment in patients of SLE with acute life-threatening complications.

Acknowledgment

We would like to acknowledge rheumatology team led by Dr. Abdulsalam Noorwali and Dr. Hossam El Deeb and from Department of Medicine, Dr. Farah Salih and Sharif Mahmoud for their assistance in the management of this case. We specially thank Dr. Ali Raza for reviewing the manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kwok LW, Tam LS, Zhu T, Leung YY, Li E. Predictors of maternal and fetal outcomes in pregnancies of patients with systemic lupus erythematosus. Lupus 2011;20:829-36.  Back to cited text no. 1
    
2.
Lockshin MD, Salmon J, Erkan D. Pregnancy and rheumatic disease. In: Creasy RK, Resnik RR, Iams JD, Lockwood CJ, Moore TR, editors. Creasy, and Resnik's Maternal-Fetal Medicine. 6th ed. Philadelphia: Elsevier; 2008. p. 1079-88.  Back to cited text no. 2
    
3.
Cheema GS, Quismorio FP Jr. Interstitial lung disease in systemic lupus erythematosus. Curr Opin Pulm Med 2000;6:424-9.  Back to cited text no. 3
    
4.
Comer M, D'Cruz D, Thompson I, Erskine K, Dacre J. Pneumonitis in a lupus twin pregnancy: A case report. Lupus 1996;5:146-8.  Back to cited text no. 4
    
5.
Todd DJ, Costenbader KH. Dyspnoea in a young woman with active systemic lupus erythematosus. Lupus 2009;18:777-84.  Back to cited text no. 5
    
6.
Boulware DW, Hedgpeth MT. Lupus pneumonitis and anti-SSA(Ro) antibodies. J Rheumatol 1989;16:479-81.  Back to cited text no. 6
    
7.
Kamen DL, Strange C. Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med 2010;31:479-88.  Back to cited text no. 7
    
8.
Pego-Reigosa JM, Medeiros DA, Isenberg DA. Respiratory manifestations of systemic lupus erythematosus: Old and new concepts. Best Pract Res Clin Rheumatol 2009;23:469-80.  Back to cited text no. 8
    
9.
Bombardier C, Gladman DD, Urowitz MB, Caron D, Chang CH. Derivation of the SLEDAI. A disease activity index for lupus patients. The committee on prognosis studies in SLE. Arthritis Rheum 1992;35:630-40.  Back to cited text no. 9
    
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Kim WU, Kim SI, Yoo WH, Park JH, Min JK, Kim SC, et al. Adult respiratory distress syndrome in systemic lupus erythematosus: Causes and prognostic factors: A single center, retrospective study. Lupus 1999;8:552-7.  Back to cited text no. 10
    
11.
Catanzarite V, Willms D, Wong D, Landers C, Cousins L, Schrimmer D, et al. Acute respiratory distress syndrome in pregnancy and the puerperium: Causes, courses, and outcomes. Obstet Gynecol 2001;97:760-4.  Back to cited text no. 11
    
12.
Derdak S, Mehta S, Stewart TE, Smith T, Rogers M, Buchman TG, et al. High-frequency oscillatory ventilation for acute respiratory distress syndrome in adults: A randomized, controlled trial. Am J Respir Crit Care Med 2002;166:801-8.  Back to cited text no. 12
    
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Sud S, Sud M, Friedrich JO, Meade MO, Ferguson ND, Wunsch H, et al. High frequency oscillation in patients with acute lung injury and acute respiratory distress syndrome (ARDS): Systematic review and meta-analysis. BMJ 2010;340:c2327.  Back to cited text no. 13
    
14.
Fort P, Farmer C, Westerman J, Johannigman J, Beninati W, Dolan S, et al. High-frequency oscillatory ventilation for adult respiratory distress syndrome – A pilot study. Crit Care Med 1997;25:937-47.  Back to cited text no. 14
    
15.
Adhikari NK, Bashir A, Lamontagne F, Mehta S, Ferguson ND, Zhou Q, et al. High-frequency oscillation in adults: A utilization review. Crit Care Med 2011;39:2631-44.  Back to cited text no. 15
    
16.
Ferguson ND, Cook DJ, Guyatt GH, Mehta S, Hand L, Austin P, et al. High-frequency oscillation in early acute respiratory distress syndrome. N Engl J Med 2013;368:795-805.  Back to cited text no. 16
    
17.
Young D, Lamb SE, Shah S, MacKenzie I, Tunnicliffe W, Lall R, et al. High-frequency oscillation for acute respiratory distress syndrome. N Engl J Med 2013;368:806-13.  Back to cited text no. 17
    
18.
Mooney MR, Unger BT, Boland LL, Burke MN, Kebed KY, Graham KJ, et al. Therapeutic hypothermia after out-of-hospital cardiac arrest: Evaluation of a regional system to increase access to cooling. Circulation 2011;124:206-14.  Back to cited text no. 18
    


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