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3
Menzies School of Health Research, Dili, Timor-Leste
4
Leeds Teaching Hospitals NHS Trust, UK
Systemic lupus erythematosus (SLE) is characterized by production of antibodies against various nuclear
antigens with involvement of multiple organs. Pulmonary manifestations of SLE can include a wide spectrum
of diseases such as pleuritis, pneumonia, pulmonary embolism, pneumothorax and pulmonary haemorrhage.
Lupus pneumonitis (LP) has an incidence of 1-8% and may be difficult to distinguish from pulmonary infections.
We report a case of LP mimicking community acquired pneumonia (CAP) and pulmonary tuberculosis (PTB)
admitted to the Internal Medicine ward in Hospital National Guido Valadares, Dili, Timor Leste.
A 43-year-old female school teacher presented with cough and shortness of breath. She also had a history of
fever and malar rash along with significant hair loss. On examination she was febrile and dyspneic, with anaemia,
hair loss, malar rash, tachycardia, tachypnoea, and coarse crepitations on chest auscultation. Sputum for Gene
Xpert MTB/RIF Ultra test, bacterial culture, and fungal stains were negative. She was initially treated with broad-
spectrum antibiotics for CAP, while being investigated for TB. However, as she failed to respond to intravenous
antibiotics, further evaluation was done. Anti-nuclear antibodies (ANA) and ds-DNA were strongly positive.
Urine analysis revealed nephrotic-range proteinuria. High resolution computed tomography (CT) showed
bilateral ground glass changes suggestive of lupus pneumonitis (LP). A diagnosis of SLE with LP was made and
the patient was commenced on corticosteroids (pulsed methylprednisolone for three consecutive days, followed
by oral prednisolone) which led to a dramatic clinical and radiological response.
SLE has a wide range of presentations. Keeping this in mind, even in countries where tuberculosis is endemic,
the differential diagnosis of SLE and LP should be considered. Many challenges exist in the diagnosis and
management of patients with SLE and its complications in resource-limited settings.
autoimmune disease, systemic lupus erythematosus, lupus pneumonitis, community acquired
pneumonia, pulmonary tuberculosis
SLE (systemic lupus erythematosus) is a multisystem autoimmune disorder which has a waxing and waning
course. The clinical manifestations of SLE are variable but include erythematous photosensitive malar rash, oral
ulcers, non-erosive polyarthritis or polyarthralgia, polyserositis, immune-mediated cytopenias, renal, neurologic,
pulmonary and cardiac abnormalities. Pulmonary manifestations of SLE were first described by Osler in 1904
who described a patient of SLE with persistent lower lobe infiltrates [1]. A wide spectrum of pulmonary
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presentations has since been described, including pleuritis, pneumonia, pulmonary embolism, pneumothorax and
pulmonary haemorrhage [2]. Though infections are also a frequent cause of pulmonary infiltrates in patients with
SLE, in many cases pulmonary infiltrates are not related to infection [1]. Lupus pneumonitis (LP) is an unusual
and potentially life-threatening complication of SLE which has an incidence of 1 % to 8 % (3,13) and usually
occurs during SLE flare-ups, with a mortality rate of up to 50%. It is rare during the primary presentation of SLE
and may mimic tuberculosis or other acute infectious pneumonia. LP results from immune complex deposition
in the lung microvasculature with complement activation and inflammatory cell recruitment that then causes
alveolar-capillary injury (12). A high index of clinical suspicion is needed when young adult females present
with unexplained pulmonary infiltrates, especially in tuberculosis endemic countries like Timor Leste where use
of empirical antituberculosis therapy is high. This is a report of LP as a presenting feature of SLE which
mimicked CAP and pulmonary tuberculosis and the diagnostic and therapeutic challenges associated with in a
setting of TB-endemic, resource-limited country (12,14).
A 43-year-old female school teacher was admitted to the Internal Medicine ward at Hospital National Guido
Valadares in Timor Leste with a three-week history of low-grade fever and cough with mucoid expectoration.
She had experienced difficulty in breathing for three days prior to admission. She had a history of intermittent
swelling of hands and feet and associated joint pain, malar rash and hair loss for the past one year but had not
sought medical attention. Systems review revealed history of irregular menstruation but no other features,
including no history of weight loss or exposure to any drugs or toxins. She did give history of contact with a
case of pulmonary tuberculosis in her family. Past medical history was unremarkable and there was no history
of travel in the recent past. She did not use tobacco or drink alcohol.
On examination she was fully conscious and oriented but appeared pale and febrile; oral temperature was 38
degrees Celsius. Her blood pressure was 100/60mmHg. She was tachycardic (pulse rate 106/minute),
tachypnoeic (respiratory rate 32/minute) and in respiratory distress as evidenced by the use of accessory muscles
of respiration. There was presence of grade-2 diffuse alopecia. On respiratory examination, there were diffuse
bilateral coarse crepitations on auscultation of her chest. Cardiac examination revealed a sinus tachycardiac with
a prominent pulmonary component of the second heart sound. There were no other positive findings on physical
examination.
Initial laboratory investigation results and ordered ANA and ds-DNA testing from Private clinic after
strong suspicion on SLE with LP.
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results of radiological studies on admission and follow up
Initial investigation and radiology results are shown in Tables 1 and 2. Bloods at presentation showed anaemia,
thrombocytopenia, elevated ESR and CRP and hypoalbuminaemia as well as elevated urea and serum creatinine.
Coagulation profile was normal. Chest X-ray on admission showed patchy bilateral air space opacification,
worse on right side involving all zones of the lung without any cranio-caudal gradient and cardiomegaly with
prominent pulmonary conus (see Figure 1). Urinalysis showed gross proteinuria with occasional pus cells.
Sputum for bacteriological culture was sterile; sputum for Gene-Xpert MTB/RIF Ultra assay was repeatedly
negative. Sputum microscopy and fungal stains did not reveal presence of fungi. Serological markers for HBV,
HCV, HIV and TPHA were non-reactive.
Abdominal ultrasonography revealed mild splenomegaly. Echocardiography showed pulmonary arterial
hypertension with a normal left ventricular function.
The patient was initially managed with empirical broad-spectrum antibiotics in line with local guidelines
(intravenous ceftriaxone 2 grams daily with azithromycin 500 milligrams oral daily) and high flow oxygen
(6L/minute) through face mask for suspected community acquired pneumonia. However, the patient’s overall
clinical condition failed to improve over the next 72 hours. On grounds of prolonged duration of her illness,
history of contact with tuberculosis and radiographic findings of multiple pulmonary opacities, the patient was
empirically put on anti-tubercular therapy, despite which her clinical condition did not improve over the
following five days.
Further investigations were therefore ordered and tested at a private facility which showed positive (high, 115.9
Au/mL) antinuclear antibody (ANA) and anti-ds-DNA (high, 435.4 IU/mL) tests, following which a diagnosis
of SLE was made. Rheumatoid factor was negative. Quantitative analysis of urine for protein showed nephrotic-
range proteinuria (3.5grams/24 hours). High resolution CT thorax of the patient showed bilateral ground glass
changes (right more than left) with relative sparing of the subpleural regions suggestive of LP (see Figure 3).
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Based on these findings and her failure to respond to empirical IV and oral antibiotics and anti-tuberculosis
treatment, we made a revised diagnosis of SLE with LP. Anti-tuberculosis treatment was stopped and intravenous
pulsed methylprednisolone (1,000 mg/day) for three days was given, followed by oral prednisolone 40mg/day
and oral Azathioprine 50mg/day. There was gradual improvement of the symptoms with disappearance of clinical
signs. There was also marked resolution of radiographic findings in the follow up chest X- rays and CT scan
thorax at the follow up (see Figures 2 and 4). The patient was discharged on steroids and Azathioprine at
maintenance doses and continues to do well on follow up at the outpatient department (OPD); she has been able
to return to work.
This case report describes a severe case of LP in a patient with no pre-existing diagnosis of SLE in a setting with
high TB endemicity and limited diagnostic resources and demonstrates the importance of thorough clinical
assessment and re-evaluation of diagnosis when response to treatment is not as expected.
LP is relatively rare, but is a serious complication of SLE. It necessitates prompt and effective intervention to
prevent its potentially lethal outcomes (13). The diagnosis of LP presents significant challenges due to its
nonspecific clinical manifestations that often mimic other pulmonary disorders and the fact it is not a common
manifestation during the primary presentation of SLE. It generally presents with acute onset of fever, cough,
tachypnea and hypoxia. The usual radiological features of LP are consolidation in one or more areas, usually
basal and bilateral, and often associated with pleural effusion and pulmonary arterial hypertension [1]. The
underlying histology in cases of LP are those of diffuse alveolar damage, bronchiolitis obliterans organizing
pneumonia, non-specific interstitial pneumonia, or a combination of these [4]. The mortality of lupus
pneumonitis is around 50%, with respiratory failure being the primary cause of death [5]. The diagnosis of LP is
primarily by exclusion of other causes of lung infiltration such as infective pneumonia (bacterial, mycobacterial,
fungal and viral), organizing pneumonia, alveolar hemorrhage, pulmonary embolism among others.
In our case the primary suspicion was indeed of an infective origin, especially CAP and/or TB, on account of the
chronic temporal pattern of the symptoms and signs with a positive history of contact with tuberculosis.
However, when repeated sputum analysis ruled out a possible infective aetiology the index of suspicion shifted
towards other possible causes and finally the diagnosis of LP was established by the clinical manifestations,
HRCT findings, anti-nuclear antibody panel and dramatic response to corticosteroids.
Managing SLE by acute lupus pneumonitis in resource-limited countries is particularly challenging due to
multiple clinical, logistical, and systemic factors. Diagnostic challenges, limited access to advanced imaging
such HRCT is the gold standard to detect ALP, but many hospitals may only have X-ray capabilities, and early
subtle lung changes may be missed leading to delayed diagnosis. ALP may mimic pneumonia or TB which are
prevalent in resource-limited settings, and misdiagnosis often leads to inappropriate antibiotic therapy instead of
immunosuppression. Timor Leste faces a high TB burden; WHO, in 2022 global TB report stated Democratic
republic of Timor-Leste ranking 6
th
with incidence rate of 498 cases per 100,000 population, making it the highest
in the SEA region.
Another important diagnostic challenge in resource-limited settings is limited laboratory testing; autoantibody
panels (ANA, anti-dsDNA) and complement levels may not be readily available. Bronchoscopy and
bronchoalveolar lavage for ruling out infection are often unavailable. Such as in our case, complement levels,
bronchoscopy and bronchoalveolar lavage were not done because they are not available in our setting; tests for
ANA and dsDNA are only available in the private setting, not in all government hospitals.
Meanwhile, limited or lack of availability of immunosuppressive drugs, high-dose IV corticosteroids and
biologics/targeted therapies such as Belimumab and Rituximab in resource-limited settings make the
management of SLE very challenging. Still another challenge is the decision to commence immunosuppression
therapy in areas with high of TB or other endemic infection (e.g. strongyloides), along with the increased risk of
opportunistic infection to the patient, with limited capacity for infection monitoring and prophylaxis, limitation
of supportive care such as Intensive care unit (ICU) with oxygen therapy, monitoring and rapid response to
respiratory failure as compare to high resource setting in developed countries (10,11,12,13,14)
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When connective tissue disorders have a presentation which mimic an infective process the diagnostic challenges
for the clinician become manifold. The possibilities of co-existence of a connective tissue disorder with an
infection, or an infection which trigger an auto-immune response further complicate the diagnostic process.
While on the one hand, patients with SLE can be complicated with superimposed infections, on the other hand,
others may present with immune mediated processes which very closely resemble an infectious process, as was
found in our case. Pulmonary infections in patients with SLE are also quite common. A previous study reported
that pulmonary infections are seen in 20% of cases with SLE [6]. As discussed in our case, it may be difficult to
differentiate pulmonary infection from LP, especially in countries like Timor Leste which are highly endemic
for TB and there being limited resources for carrying out sophisticated immunological tests to diagnose or
exclude connective tissue diseases. In our hospital there is not available test for dsDNA and ANA for connective
tissue disorders (SLE), in cases where there is a strong suspicion, it is important to recommend the patient to be
tested through a private clinic, such as in this case. In such cases, it sometimes becomes imperative to administer
broad spectrum antibiotics and also to consider a trial of anti-tuberculous therapy prior to establishing a definitive
diagnosis. However, other signs of active lupus like rash, arthralgias, malar rash, alopecia, elevated ESR and
CRP with high urinary protein and casts may help in differentiating LP from pulmonary infections. The other
possibilities that come in the differential diagnosis of lupus pneumonitis include are Goodpasture’s syndrome
and granulomatosis with polyangiitis (GPA), because all three share overlapping clinical, radiological and
pathological features. In our case these entities could not be excluded as there was no availability of testing for
ANCA and anti-GBM autoantibodies (8,9,10).
Because acute LP is a rare entity there is no definite consensus on the optimal modality of treatment [7]. Patients
are generally treated with high dose corticosteroids. In cases which respond poorly to corticosteroids
(methylprednisolone/prednisolone), immunosuppression (cyclophosphamide, azathioprine, methotrexate and
mycophenolate mofetil (MMF) and biologics/ targeted therapy (Belimumab or Rituximab) are used.
Hydroxychloroquine (HCQ) has also been used to some effect [11,12,13,14].
Connective tissue disorders have a wide range of presentation. Our case shows a rare presentation of SLE
presenting as acute LP which was initially considered as community acquired pneumonia and pulmonary
tuberculosis. Even in tuberculosis endemic country like Timor-Leste where clinical findings and chest X-rays
are sometimes taken as sufficient evidence for initiation of anti-tuberculous therapy in the absence of an
alternative diagnosis, the differential diagnosis of SLE, especially LP should be borne in mind and appropriate
investigations should be carried out so that the possibility of LP is not missed in these patients. In resource-
limited settings, there are range of challenges in managing SLE and LP, including delays in diagnosis, lack of
immunosuppressive drugs, high infection risk, and limited ICU support make ALP management extremely
difficult, contributing to high morbidity and mortality as compared to high resource countries.
None declared.
All procedures performed in studies involving human participants were in accordance with the ethical standards
of the institutional and/or National institute of Public Health Timor-Leste (INSPTL). Informed consent was
obtained from all individual participants included in the study. The authors declare that appropriate written
informed consent was obtained for the publication of this manuscript and accompanying images.
The data that support the findings of this study are available from the corresponding author upon reasonable
request from Radiology department and medical record hospital national Guido Valadares.
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