|CASE BASED REVIEW
|Year : 2021 | Volume
| Issue : 3 | Page : 333-337
Spontaneous recovery in a case of lupus with severe COVID-19 pneumonia; Do we need to reconsider treatment Protocol?
Pradeepta Sekhar Patro1, Samir Sahu2, Shawaz Iqbal3, Ishwar Chandra Behera3, Priyanka Samal4, Satyasundar Mohapatra5
1 Department of Clinical Immunology and Rheumatology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Medicine, IMS and SUM Hospital, Bhubaneswar, Odisha, India
3 Department of Neurology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
4 Department of Hematolog, IMS and SUM Hospital, Bhubaneswar, Odisha, India
5 Department of Radiology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
|Date of Submission||03-Jun-2020|
|Date of Acceptance||25-Aug-2020|
|Date of Web Publication||21-Sep-2021|
Dr. Pradeepta Sekhar Patro
Department of Clinical Immunology and Rheumatology, IMS and SUM Hospital, Bhubaneswar - 751 003, Odisha
Source of Support: None, Conflict of Interest: None
Individuals with inflammatory rheumatic diseases such as lupus are considered at risk for severe COVID-19 infection due to their immunocompromised state and the use of immunosuppressive treatment. Its impact on lupus is yet to be determined. Herein, we report a case of COVID-19 pneumonia in a 40-year-old female with a history of hypothyroidism and mild pulmonary hypertension who presented with a 4-day history of fever and breathlessness. COVID-19 infection was confirmed by real-time polymerase chain reaction (RT-PCR). On examination, she had a malar rash, short hair, and crepitations on the right side infrascapular and infraaxillary areas. Investigations revealed to have anemia, thrombocytopenia, transaminitis, high ferritin, and low fibrinogen, which were suggestive of cytokine release syndrome. Her antinuclear antibody (ANA) by indirect immunofluorescence on Hep-2 cells was 4+ homogeneous in 1:100 titer dilution and extractable nuclear antigen panel showed antibodies positive for histone, nucleosome, Ro, and smith antigens. The patient was diagnosed with systemic lupus erythematosus (SLE) with severe COVID-19 pneumonia with cytokine release syndrome and managed conservatively without specific antivirals or steroids. Her clinical condition and laboratory parameters improved afterward. On the 12th day, her RT-PCR for COVID-19 was negative and the patient was discharged. This would probably a reference case which showed conservative management can be helpful at times where no definite antiviral therapy has been approved with judicious use of immunosuppressants. Close monitoring of clinical conditions and laboratory parameters are recommended for such autoimmune disorders with COVID-19 infection.
Keywords: Corona, COVID-19 pneumonia, cytokine release syndrome, lupus, systemic lupus erythematosus
|How to cite this article:|
Patro PS, Sahu S, Iqbal S, Behera IC, Samal P, Mohapatra S. Spontaneous recovery in a case of lupus with severe COVID-19 pneumonia; Do we need to reconsider treatment Protocol?. Indian J Rheumatol 2021;16:333-7
|How to cite this URL:|
Patro PS, Sahu S, Iqbal S, Behera IC, Samal P, Mohapatra S. Spontaneous recovery in a case of lupus with severe COVID-19 pneumonia; Do we need to reconsider treatment Protocol?. Indian J Rheumatol [serial online] 2021 [cited 2021 Dec 6];16:333-7. Available from: https://www.indianjrheumatol.com/text.asp?2021/16/3/333/324767
| Introduction|| |
Individuals with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease 2019 (COVID-19) show varied symptoms, such as fever, cough, loss of smell, dyspnea, nausea, and vomiting. The clinical severity of COVID-19 ranges from asymptomatic to acute respiratory distress syndrome (ARDS) and multiple organ dysfunction requiring mechanical ventilation and admission to the intensive care unit.
Patients with rheumatic diseases are more prone to serious infections due to their immunocompromised state resulting either from their underlying immune conditions or due to the use of immunosuppressive medications., There is a definite concern for the management of COVID-19 patients with systemic lupus erythematosus (SLE) among the rheumatologists. COVID-19 Global Rheumatology Alliance registry uncover that among 110 rheumatic disease patients who had COVID-19 infection, 19 had SLE.
However, there have been few reports on the management of COVID-19 in SLE patients. Herein, we report a case of severe COVID-19 pneumonia in a lupus patient with a cytokine release syndrome. The judicious use of immunosuppression is extremely important in these patients as it can lead to reduced viral clearance with poor outcomes.
| Case Report|| |
A 40-year-old female with a history of fever and breathlessness for the past 4 days presented to a hospital elsewhere and was diagnosed with COVID-19 infection with real-time polymerase chain reaction (RT-PCR) method following, which she was referred to our hospital. She had a history of contact with a COVID-19 positive case in her family. She had also a history of intermittent low-grade fever, joint pain, hair loss, easy fatiguability, and breathlessness of Grade II for the past 6 months for which she was evaluated elsewhere and diagnosed to have hypothyroidism and mild pulmonary hypertension. She was on thyroxine supplementation, sildenafil, and intermittent diuretics. She was also on alternative medications on and off for her fever and joint pain.
At the time of admission, she had a temperature of 102oF, heart rate of 110 beats/min, respiratory rate of 32/min, and SpO2 of 88% on room air for which she was categorized as severe COVID-19 infection. On examination, she had short hair, malar rash, healed oral ulcer over the hard palate, no pharyngeal congestion [Figure 1], and crepitations present over the right infrascapular and infraaxillary areas along with loud pulmonary component of second heart sound heard. There was no pedal edema or hepatosplenomegaly. The baseline laboratory parameters [Table 1] revealed thrombocytopenia, altered transaminases, high serum ferritin, and elevated lactate dehydrogenase (LDH) with low fibrinogen. Her reticulocyte count was normal, and direct Coomb's test was negative, which rule out ongoing hemolysis. Based on these parameters on the day of admission, her H score was 126. X-ray chest [Figure 2]a showed opacity in the right lower zone with blunting of the right costophrenic angle typical for COVID-19 pneumonia. Two-dimensional echocardiography revealed global hypokinesia with an ejection fraction of 45% suggestive of myocarditis and mild pulmonary hypertension (mean pulmonary artery pressure 35 mm Hg) without any dilation of right atrium and ventricle. The patient was given supplemental oxygen, paracetamol, piperacillin-tazobactam, azithromycin, tadalafil, and diuretics. On the next day of admission, there was a dropping of all the three lineages of blood parameters, i.e., hemoglobin, leukocyte, and thrombocyte counts, and her H score was increased to 150, which suggested a possible macrophase activation syndrome. The erythrocyte sedimentation rate was also decreased to 41 mm/h from 63 mm/h. As she was clinically stable, no immunosuppressants were administered. She was kept under strict monitoring of both clinical conditions and laboratory parameters. She was managed conservatively without any antivirals. Hydroxychloroquine was not given as there was the prolongation of QTc in the electrocardiogram (ECG). Her laboratory parameters, such as platelet count, liver function tests, ferritin, and LDH, were improved subsequently [Figure 3]a and [Fiogure 3]b. After 12 days of hospitalization, her repeat RT-PCR for COVID-19 came negative. Her C-reactive protein (CRP) came to the normal level, and repeat chest X-ray [Figure 2]b showed partial clearing of right lower zone opacity, and pleural effusion was ruled out by ultrasonography of the thorax, which revealed only peripheral subpleural opacity and no pleural space collection. For evaluation of the underlying condition, her antinuclear antibody by indirect immunofluorescence on Hep-2 cells was 4 + homogenous in 1:100 titer dilution, and extractable nuclear antigen panel was done, which revealed antibody positive to histone, nucleosome, Ro, and smith antigens, establishing the diagnosis of lupus. For measurement of lupus disease activity, urinary parameters, anti-double strand DNA, and complement levels such as C3/C4 were done, which were normal. Lupus anticoagulant and anti-phospholipid antibodies were within the normal limits. The patient was discharged with oral hydroxychloroquine 200 mg once a day following QTc interval normalization on ECG along with low dose steroids.
|Table 1: Laboratory results of the patient on the day of hospitalization|
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|Figure 1: Hyperpigmented Lesion over the malar area ,nose sparing the nasolabial area on supplemental oxygen|
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|Figure 2: Chest X-ray imaging finding. (a) Peripheral subpleural opacity with blunting of right costophrenic angle on the day of admission. (b) Follow up X-ray shows partial clearing of right lower zone opacity (ultrasonography correlation showed patchy lower lobe consolidation without any pleural space collection)|
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|Figure 3: Progression of laboratory parameters after hospitalisation (a) Hemoglobin (Hb), Total leukocyte counts (TLC), platelet, absolute lymphocyte count (ALC). (b) Aspartate aminotransferase(AST), alanine aminotransferase (ALT), lactate dehydrogenase (LDH), Serum ferritin, D-dimer|
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| Discussion|| |
This case illustrates the presentation, clinical course, management, and follow-up of a case of lupus with COVID-19 infection and impending macrophage activation syndrome. This case highlights the conservative approach with careful monitoring might be effective in selected patients.
Lupus patients are at more risk for the severe form of COVID-19 infection due to dysregulation of their immune response, long-term use of immunosuppressive drugs, and high prevalence of chronic kidney disease., In a study by Mathian et al., among seventeen lupus patients hospitalized for COVID-19 infection, 11 (65%) had a respiratory failure, whereas 5 (29%) had ARDS. In the above study except for a single patient with active tenosynovitis at the onset of COVID-19 infection, none of the other lupus patients showed clinical signs of lupus flare during the disease course. The current case at the time of presentation had respiratory insufficiency requiring supplemental oxygen, myocarditis, malar rash, bicytopenia, transaminitis, low fibrinogen, and elevated ferritin, suggesting macrophage activation syndrome. H score of this index case being 150 gives the probability of progressing to macrophage activation syndrome as 25%–40% even though the laboratory features suggest the same. There was unilateral peripheral subpleural opacity on chest X-ray through unilateral involvement is less common in comparison to bilateral one. Peripheral and lower zone distribution were the most common presentations. Unilateral involvement can be seen in 30.8% of COVID-19 cases.
Several studies have reported that severe COVID-19 patients have immune dysregulation, decreased lymphocyte count, thrombocytopenia, and increased serum ferritin, which are consistent with this case. There are similarities in the laboratory parameters due to either severe COVID-19 infection or active lupus such as anemia, leukopenia, lymphopenia, thrombocytopenia, high LDH, elevated serum ferritin, and prolonged activated partial thromboplastin time. In this scenario, high CRP value might be helpful to differentiate active lupus versus COVID-19 infection as its more common in the latter. Even though high CRP in lupus occurs in the presence of serositis, arthritis and bacterial infections, the presence of normal complements, and anti dsDNA in the index case with high CRP is more favorable for COVID 19 infection than underlying active lupus.
COVID-19 treatment in lupus patients is challenging, as the immunosuppressive agents could aggravate the viral infection. For rheumatic patients admitted to hospitals with severe infections, temporary discontinuation of most immunosuppressants is recommended to develop protective immunity and eliminate the pathogens. On the other hand, discontinuing the immunosuppressants might aggravate the lupus disease activity. The effect of steroid use in COVID-19 is still unknown. Steroid might worsen the infections by reducing the immune response, but the inflammatory cascade of pneumonia could be blocked by the administration of systemic steroid treatment. Steroid use was associated with delayed coronavirus RNA clearance of both SARS and the Middle East respiratory syndrome. In a study, seven hospitalized lupus patients with COVID-19 infection, three had severe hypoxemia who were treated with immunosuppressants such as steroid and tocilizumab after which one patient improved clinically and two remained critically ill despite improving inflammatory markers. The real-life benefit of these modalities still needs to be explored and mandate careful use of these drugs. COVID-19 patients presenting with continuous fever, respiratory failure, and multiorgan dysfunction with elevated serum IL-6 level can lead to secondary macrophage activation syndrome, where a therapeutic trial of tocilizumab can be useful. As there were no bleeding manifestations despite thrombocytopenia, she was not initiated on corticosteroids. Calculating the H-score can be helpful to predict the probability of progressing to develop secondary macrophage activation syndrome in such scenarios. Hence, close monitoring of the clinical condition and laboratory parameters were done. In this case, hydroxychloroquine was not given as there was QTc prolongation in the ECG. After 4 days of admission with conservative management, her clinical conditions, platelet counts improved, as well as other inflammatory parameters normalized. Her X-ray chest also revealed the resolution of the chest shadows. She was discharged with HCQ 200 mg/day and low-dose steroids for controlling lupus disease activity.
| Conclusion|| |
We reported the clinical manifestations and disease course of severe COVID-19 pneumonia in a lupus patient with impending macrophage activation syndrome who improved without specific antivirals or immunosuppression. This case highlights that conservative management can be instituted in selected patients until a definite antiviral or another effective modality are available. Close monitoring of clinical conditions, laboratory parameters, and calculating H-score are important strategies in autoimmune disorders with COVID-19 infection. The treatment decisions should not be made pre-emptive and the use of immunosuppressants in COVID-19 infection should be used cautiously. More clinical data are needed to further optimize the treatment regimen for severe COVID-19 infection in lupus patients.
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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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