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CASE LETTER
Year : 2020  |  Volume : 37  |  Issue : 4  |  Page : 346-348  

A case of large unilateral pneumonia as the presenting feature of systemic lupus erythematosus


Department of Pulmonology, Critical Care and Sleep Medicine, Fortis Memorial Research Institute, Gurugram, Haryana, India

Date of Submission26-Oct-2019
Date of Acceptance10-Mar-2020
Date of Web Publication01-Jul-2020

Correspondence Address:
Manoj Kumar Goel
Department of Pulmonology, Critical Care and Sleep Medicine, Fortis Memorial Research Institute, Gurugram, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_491_19

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How to cite this article:
Goel MK, Kumar A, Maitra G. A case of large unilateral pneumonia as the presenting feature of systemic lupus erythematosus. Lung India 2020;37:346-8

How to cite this URL:
Goel MK, Kumar A, Maitra G. A case of large unilateral pneumonia as the presenting feature of systemic lupus erythematosus. Lung India [serial online] 2020 [cited 2020 Aug 6];37:346-8. Available from: http://www.lungindia.com/text.asp?2020/37/4/346/288749



Sir,

Systemic lupus erythematosus (SLE) is a prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues. Pulmonary involvement is seen in 50%–70% of SLE patients and may even be the initial presenting feature in 4%–5% of cases.[1],[2] The pulmonary manifestations in SLE include pleuritis with or without effusion, acute pneumonitis, interstitial lung disease, pulmonary embolism, pulmonary hypertension, large airway disease, shrinking lung syndrome, and diffuse alveolar hemorrhage.[1],[2] Acute lupus pneumonitis (ALP) is an abrupt febrile pneumonic process without an infectious etiology. Only a few cases of ALP have been reported in the literature to date.[1],[3],[4] A couple of studies report that its frequency does not exceed 4% of the cohorts.[5],[6] We report a case of unilateral lupus pneumonia with dense lobar consolidation of the left upper lobe. An extensive review of the literature revealed that such a case has not been reported earlier.

A 62-year- old woman presented with the complaints of cough, progressive breathlessness, and fever in the range of 99.6–100.4°F and left-sided chest pain of a 1-month duration. There was no history of systemic complaints such as joint pains, skin rashes, oral ulcers, alopecia, or symptoms related to any other systemic involvement. She denied any comorbid condition. The patient was treated for a large, left-sided consolidation apparently of an infective etiology with empirical broad-spectrum antibiotics for 15 days in another hospital. However, she had no improvement, and there was a gradual clinical deterioration. On examination, the patient's general condition was weak. She was febrile with a temperature of 100.4°F and had tachypnea with a respiratory rate of 28/min. On examination of the chest, the movements were decreased on the left side with a generalized dull note to percussion. There was bronchial breathing in the left infraclavicular and axillary areas, and a pleural rub was heard in the left infrascapular region. Hematologic investigations revealed an erythrocyte sedimentation rate of 120 mm/1st h, C-reactive protein was 161.5 mg/L (normal <10.0), and procalcitonin was 0.3 ng/mL (normal <0.5). The patient was hypoxic with an SpO2 of 80% and a PaO2 of 50 mmHg on room air. X-ray and contrast-enhanced computed tomography of the chest showed dense consolidation of the left lung [Figure 1] and [Figure 2]. Bronchoscopy revealed mild inflammation of the left bronchial tree and mucoid secretions. Bronchoalveolar lavage (BAL) fluid for bacteria, fungi, and Mycobacteria was negative. Blood culture did not show any bacterial or fungal growth. Her autoimmune profile was as follows: antinuclear antibody (ANA), 1:320 with speckled pattern; anti-dsDNA, 218 IU/ml (normal <60); anti-Sm antibodies; anti-Ro/SSA; and antiphospholipid IgG antibodies, 19.3 U/ml (normal <12.0) were positive. Lupus anticoagulant, anti-La/SS-B, anti-RNP, RA factor, anti-cyclic citrullinated peptide, anti-glomerular basement membrane, cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA), anti-beta-2 glycoprotein, antiphospholipid IgM, and anti-cardiolipin antibodies were negative. Hypocomplementemia was present with C3 of 0.24 g/L (normal 0.9–1.8) and C4 of 0.06 g/L (normal 0.1–0.4). On the basis of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) and 2015 revised criteria, the patient had clinical evidence of pleuritis and positive immunological findings of ANA, anti-dsDNA, anti-Sm, anti-Ro/SSA, antiphospholipid antibodies, and low complement levels. Thus, a definitive diagnosis of acute lupus pneumonia was made. The patient was administered antibiotics clarithromycin and piperacillin-tazobactam. In view of her weak general condition and a large consolidation, methylprednisolone 500 mg for 3 days was given as a pulse therapy followed by azathioprine 100 mg and prednisolone tapered to 7.5 mg daily in the maintenance phase for 2 years. The patient remains asymptomatic with good clinical and radiological recovery without any recurrence till date.
Figure 1: Chest X-ray showing consolidation of the left lung

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Figure 2: (a) Contrast.enhanced computed tomography chest – mediastinal window showing dense consolidation of the left lung with air bronchogram sign. There is no mediastinal lymphadenopathy. (b) Lung window showing large pneumonia of the whole left lung with dense consolidation of the entire left upper lobe

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The true frequency of pulmonary complications in SLE is difficult to establish as many cases are due to infections.[7] ALP may present with nonspecific unilateral or bilateral focal or diffuse alveolar infiltrates,[5] ground-glass opacities, or patchy areas of consolidation.[8] The clinical features, such as fever, cough, dyspnea, pleuritic chest pain, hemoptysis, pleural rub, and basilar crepts, are nonspecific, and the picture may mimic an infective pathology. Hence, ALP is diagnosed by exclusion of other causes of pneumonitis and the presence of diagnostic criteria of SLE, as was done in this case who also tested positive for anti-Ro/SSA. There is a correlation between anti-Ro/SSA antibodies and ALP. A study showed that patients with SLE who had pulmonary complications had 81% positivity for anti-Ro/SSa antibodies, whereas patients without pulmonary involvement had a positivity of 38%.[9] This high frequency of the association between anti-Ro/SSA antibodies and ALP suggests its possible role in the pathogenesis of the disease.[10] It is important to note that pleuritis with or without pleural effusion is the only pleuropulmonary criterion which has been included in the SLICC criteria for the diagnosis of SLE.

The treatment of ALP is not clearly defined and is usually based on case reports and anecdotal experience.[2] Empiric broad-spectrum antibiotics are started immediately and continued until the infection is excluded. Corticosteroids are the mainstay of treatment. Intravenous pulse steroids up to a dose of 1 g methylprednisolone daily for 3 days is advised in hypoxic patients with poor general condition,[7] as was required in this case. Additional immunosuppressive agents such as azathioprine, methotrexate, and cyclophosphamide should also be considered. In patients refractory to corticosteroids, intravenous immunoglobulin, plasma exchange, or rituximab have also been used only with a little evidence.[11],[12]

The prognosis of ALP is poor, with an estimated mortality rate of 50%–90% even with treatment.[5] The presence of eosinophilia or neutrophilia in BAL carries a worse prognosis than lymphocytosis.[8]

This case is unique as the patient presented with large pneumonia of the left lung with dense consolidation of the entire left upper lobe. In this case, lupus pneumonia was the presenting feature of SLE without any other organ or systemic involvement. ALP is a special diagnostic challenge and in view of the associated high morbidity and mortality requires prompt and aggressive management. Early initiation of immunosuppressive treatment in patients with ALP is lifesaving and may be associated with complete recovery.

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.
Aggarwal HK, Jain D, Mittal A, Rao A, Yadav RK, Jain P. Systemic lupus erythematosus presenting as fulminant lupus pneumonitis: A rare case report. Reumatismo 2016;68:48-52.  Back to cited text no. 1
    
2.
Hannah JR, D'Cruz DP. Pulmonary complications of systemic lupus erythematosus. Semin Respir Crit Care Med 2019;40:227-34.  Back to cited text no. 2
    
3.
Şişmanlar Eyüboǧlu T, Aslan AT, Özdemir Y, Gezgin Yıldırım D, Buyan N, Boyunaǧa Ö. Isolated acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus in an 8-year-old girl. Auto Immun Highlights 2018;9:4.  Back to cited text no. 3
    
4.
Lazovic B, Zlatkovic-Svenda M, Jasarovic D, Stevanovic D. Systemic lupus erythematosus presenting as acute lupus pneumonitis. Arch Bronconeumol 2018;54:222-3.  Back to cited text no. 4
    
5.
Orens JB, Martinez FJ, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Rheum Dis Clin North Am 1994;20:159-93.  Back to cited text no. 5
    
6.
Crestani B. The respiratory system in connective tissue disorders. Allergy 2005;60:715-34.  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.
Matthay RA, Schwarz MI, Petty TL, Stanford RE, Gupta RC, Sahn SA, et al. Pulmonary manifestations of systemic lupus erythematosus: Review of twelve cases of acute lupus pneumonitis. Medicine (Baltimore) 1975;54:397-409.  Back to cited text no. 8
    
9.
Boulware DW, Hedgpeth MT. Lupus pneumonitis and anti-SSA (Ro) antibodies. J Rheumatol 1989;16:479-81.  Back to cited text no. 9
    
10.
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. 10
    
11.
Lim SW, Gillis D, Smith W, Hissaria P, Greville H, Peh CA. Rituximab use in systemic lupus erythematosus pneumonitis and a review of current reports. Intern Med J 2006;36:260-2.  Back to cited text no. 11
    
12.
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. 12
    


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  [Figure 1], [Figure 2]



 

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