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American Journal of  Medical 
Science and Innovation (AJMSI) 

A Recent Diagnosis Of  Systemic Lupus Erythematosus In A Male With A History Of  
Recurrent Pneumothorax And Cystic Bronchiectasis: A Case Report

Raja Bakhsh1, Khaled Dairi1, Elaf  Almadabgy1, Hanan Almehmadi1, Bashaeir Alshareeif1, Amani Albiladi1, Alaa Husein1

Lamyaa Gamal1, Turki Alsulimani2 , Fatmah AlShariff3

Volume 3 Issue 2, Year 2024
ISSN: 2836-8509 (Online)

DOI: https://doi.org/10.54536/ajmsi.v3i2.3124
https://journals.e-palli.com/home/index.php/ajmsi

Article Information ABSTRACT

Received: August 18, 2024
Accepted: September 22, 2024
Published: September 26, 2024

Recently published case reports showed that Pneumothorax can be associated with active 
systemic lupus erythematosus (SLE). Herein, we present a smoker man with a positive 
history of  connective tissue disease (CTD) in the family, who was admitted to the hospital 
many times during the previous year for recurrent chronic obstructive pulmonary disease 
(COPD) exacerbations and multiple chest infections. The patient had a history of  recurrent 
spontaneous pneumothorax 20 years prior, which required treatment with pleurodesis. 
Furthermore, a year ago, he received a diagnosis of  SLE. The association of  pneumothorax 
with smoking and COPD is well known. Still, in our patient’s case, he may have been 
predisposed to develop recurrent pneumothorax requiring pleurodesis because of  his 
underlying undiagnosed and untreated active SLE disease.

Keywords
Connective Tissue Disease, 
Chronic Obstructive Pulmonary 
Disease, Pleurodesis Recurrent 
Spontaneous Pneumothorax, 
Smoking, Systemic Lupus 
Erythematosus

1 Department of  Internal Medicine, King Faisal Hospital, Ministry of  Health, Makkah, Saudi Arabia
2 Department of  Radiology, Maternity and Children Hospital, Ministry of  Health, Makkah, Saudi Arabia
3 Ibn Sina College, Jeddah, Saudi Arabia
* Corresponding author’s e-mail: rajaa_bakhsh09@outlook.com

INTRODUCTION
Pneumothorax is a serious condition that can potentially 
lead to severe complications such as respiratory failure, 
cardiovascular collapse, and death if  not recognized and 
treated immediately. Primary spontaneous pneumothorax 
is primarily seen in individuals who are healthy and have 
no prior history of  chest trauma or underlying lung 
conditions. Predominantly, it affects tall and thin males in 
the age range of  20 to 40 years. Secondary spontaneous 
pneumothorax affects individuals who already have lung 
disease, particularly emphysematous lung disorders; 
other causes include lung cancer, interstitial lung disease, 
pneumonia, asthma, cystic disorders, and pulmonary 
tuberculosis (Leys et al., 2020; Nishimoto et al., 2020).  
Spontaneous pneumothorax can be a complication 
that occurs in patients with connective tissue disease-
associated interstitial lung disease (CTD-ILD) and predicts 
a poor prognosis. It is also reported in some studies 
to be a rare pleuropulmonary manifestation of  active 
systemic lupus erythematosus (SLE). Studies associating 
SLE with pneumothorax are limited; therefore, the 
association between the two conditions remains unclear. 
However, all connective tissue disorders may theoretically 
be associated with a decrease in the integrity of  the 
pleural membranes, which could lead to pneumothorax 
(Graves et al., 2022a; Imad et al., 2022; Sharma & Sharma, 
2019). Bronchiectasis shares many clinical features 
with COPD, including inflamed and easily collapsible 
airways, airflow obstruction, and frequent office visits 
and hospitalizations. The diagnosis is established 
clinically based on cough on most days with tenacious 

sputum production, often one or more exacerbations/
year, and radiographically by the presence of  bronchial 
airway dilatation on chest computed tomographic (CT) 
scans. Bronchiectasis has many causes including Cystic 
fibrosis, mycobacterial infections, Foreign bodies, airway 
compression, human immune deficiency virus (HIV) 
and hypogammaglobulinemia, Primary ciliary dyskinesia 
(PCD), Allergic bronchopulmonary aspergillosis 
(ABPA), Fibrosis of  the lung tissue, Alpha-1 antitrypsin 
deficiency, Autoimmune or inflammatory disorders, like 
rheumatoid arthritis (RA), inflammatory bowel disease 
(IBD), lupus (SLE), and Sjögren’s syndrome. This case 
report presents a Saudi male who developed a left-
sided recurrent spontaneous pneumothorax which was 
treated with chest tube insertion twice then eventually 
pleurodesis. Subsequently, he was diagnosed with 
bilateral bronchiectasis and treated accordingly without 
significant improvement in clinical condition. However, 
20 years later, the patient was diagnosed with SLE due to 
the presence of  musculoskeletal symptoms and positive 
results of  antinuclear antibody and anti-double-stranded 
DNA antibody tests.

Case Presentation
In March 2023, a 48-year-old male from Saudi Arabia, 
who works as a nurse, presented to the emergency 
department at King Faisal Hospital in Makkah City 
complaining of  severe shortness of  breath (SOB) for 
several hours of  a one-day duration with no aggravating 
or relieving factors. His SOB was associated with cough 
and whitish sputum production with no fever, chest pain, 



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or history of  contact with sick patients. The patient, who 
was completely vaccinated against COVID-19, reported 
a history of  repeated hospital admissions during the 
past year including intensive care unit admission due to 
community-acquired pneumonia with respiratory failure 
type 2. Previously he has been prescribed oral short 
courses of  Prednisone (a steroid) in order to decrease 
the inflammation in the airways along with a combination 
of  treatments including moxifloxacin (an antibiotic), 
inhalers such as salbutamol, fluticasone with salmeterol, 
tiotropium, and home BiPAP therapy for respiratory 
assistance but no significant improvement overall with 
the previously mentioned treatments. He reports a 
history of  hookah smoking that had started 30 years ago; 
he had discontinued the activity over the past year. He 
also reported a history of  CTD in the family, including 
a brother with Bechet disease and a history of  left-sided 
recurrent spontaneous pneumothorax 20 years ago which 
was treated by chest tube insertion twice and eventually 
by pleurodesis. Examination revealed tachypnea with a 
respiratory rate of  28 bpm, O2 saturation of  90% on 1 
liter of  oxygen, and venous blood gases showing a pH 
(7.33), PCO2 (74 mmHg), and HCO3 (39 mmol/L). Upon 
chest auscultation, bilateral crepitations were noted, 
clearly accompanied by expiratory wheezes and decreased 
breath sounds specifically over the right lung. Also, the 
patient was noted to be slightly pale with apparent finger 
clubbing in both hands. The chest X-ray shown in (Figure 
1) shows clear hyperinflation in both lungs along with 
consolidation and severe bronchiectasis on the right 
middle lung lobe. The impression of  bronchiectasis 
exacerbation plus pneumonia was made and the patient 
was admitted to the medical ward under the pulmonology 
team for further treatment. Subsequently, a decision was 
made to refer the patient for Spiral computed tomography 
(CT) of  the lungs to rule out pulmonary embolism, but 
the patient refused this procedure due to his fear of  
contrast administration.

He agreed to have high-resolution computed tomography 
(HRCT) of  the lungs shown in (Figure 2) which detected 
bilateral lung bronchiectasis predominantly in the right 
middle lobe and slightly less in the lower and upper lung 
lobes. Bronchiectasis is variable from varicoid to cystic. 
Air fluid levels are present within the cysts, which may 
represent mucus accumulation. Small areas of  scarring 
are observed in the lung apices as well as some trees in 
bud nodularity. The mediastinal area exhibits multiple air 
cysts in the paratracheal and subcarinal areas around the 
main bronchi with no gross lymph node enlargement, no 
pleural effusion or pneumothorax, and no gross chest 
wall abnormality.

Figure 1: Chest X-ray showing hyperinflated chest with 
bilateral bronchiectasis

Figure 2: High-resolution computed tomography 
showing bilateral lung bronchiectasis predominantly in 
the right middle lobe.

The echocardiogram revealed that the left ventricular 
systolic function was within normal limits, with evidence 
of  grade 1 diastolic dysfunction and the ejection fraction 
at 55%. The pulmonary artery systolic pressure (PASP) 
peaked at 50 mmHg. The left atrium was of  normal size 
while the right atrium was a bit more enlarged compared 
to the other. No signs of  atrial septal defect. 
The blood test results were noted down at the time of  
admission at the hospital which is given in Table 1 in 
detail: 

Table 1: Blood test results on admission
WBC: 6.11 x 109/L      (NORMAL)
Lymphocytes: 26.2 %  (NORMAL)
Monocytes: 15.5 %      (HIGH)
Neutrophil: 52.2 %      (NORMAL)
Eosinophils: 5.9 %      (UPPER LIMIT OF 

NORMAL)
Basophils: 0.2 %         (NORMAL)
Red blood cell count:  5.64 
X1012/L  

(HIGH)

Hemoglobin: 10.4 g/dL       (LOW)
MCV: 72.9 fL                       (LOW)
MCH: 19 pg                   (LOW)
DAT:  positive



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Am. J. Med. Sci. Innov. 3(2) 75-79, 2024

reticulocytes count: 0.1641 (HIGH)
Hematocrit: 39.8 %             (LOW)
RDW: 27.4%                        (HIGH)
Platelets: 305  x 109/L
Calcium: 2.43 mmol/L
Phosphate: 1.04 mmol/L
Na: 137 mEq/L
Potassium: 5.4 mmol/L
Total Protein: 81.2 g/L
Albumin: 45 g/L
Creatinine: 0.54 umol/L
BUN: 9 mmol/L
Vitamin D: 74 nmol/L  
CKI: 28.2     U/L     ( LOW)
LDH: 417.8  U/L  (NORMAL)
ALT: 36 U/L
AST: 27 U/L
Total Bilirubin: 0.60 umol/L
Direct Bilirubin: 0.2 umol/L
INR: 1.1 
PT: 14.9 s
PTT: 32.8 s

WBC: White blood cell count; MCV: mean corpuscular 
volume; MCH: Mean corpuscular hemoglobin; RDW:red cell 
distribution width; CKI: Creatine Kinase with Isoenzymes; 
LDH: Lactate Dehydrogenase; AST: aspartate transaminase; 
ALT: alanine aminotransferase; PT: prothrombin time; PTT: 
partial thromboplastin time; BUN: blood urea nitrogen; INR: 
international normalized ratio.

Sputum culture revealed evidence of  Pseudomonas 
aeruginosa growth and appropriate antibiotic treatment 
started. During his hospital stay, he was complaining of  
upper back pain and bilateral shoulder pain that had been 
bothering him over the last year but was responding to 
the previously described short courses of  oral steroids 
(prednisone). Apart from a positive Coombs test and 
anemia, no other rheumatological disease manifestations 
were observed, so an impression of  autoimmune 
hemolytic anemia was made. The rheumatology team 
consulted and a full autoimmune profile was requested 
(see Table 2).
The patient was diagnosed with SLE (based on positive 
ANA testing and anti-DS DNA) plus antiphospholipid 
syndrome with triple positivity of  antiphospholipid 
antibodies. The management plan for the patient 
included oral prednisolone of  50 mg daily for 1 month, 
then tapered by 5 mg every 2 weeks, oral omeprazole 
of  40 mg daily, oral calcium of  600 mg thrice daily, 
oral Vitamin D of  50000 IU once weekly, oral bactrim 
of  960 mg thrice weekly, oral warfarin of  5 mg daily, 
oral hydroxychloroquine of  200 mg twice daily, oral 
mycophenolate mofetil of  1 gram twice daily, rituximab 
of  one cycle every 6 months plus two IVIG doses of  
1 g/kg, Sun-protection SPF of  50%, subcutaneous 
denosumab 60 of  mg every 6 months, fluticasone/
vilanterol of  200 micrograms inhalational once daily, 
tiotropium inhalational of  18 mcg once daily, Ventolin 
inhalation of  2 puff  PRN, long term oxygen therapy, and 
daily home continuous positive airway pressure (C-PAP) 
from 12 midnight to 6 am. Additionally, he was referred 
to a transplant center for lung transplantation assessment. 
After initiation of  the previously mentioned medications, 
the patient reports improvement in his overall symptoms 

Table 2: The autoimmune profile of  the patient, inflammatory markers, complement level, and immunoglobulins level
C4 13.8 mg/dL   (low)
C3 103 mg/dL    (normal)
IgG 1,000 mg/dL (normal)
IgA 318 mg/dL    (normal)
IgM 102 mg/dL    (normal)
ESR 62 mm/hour (high)
CRP 179.2 mg/L   (high)
Anti-jo-1 antibody 4.28    negative
scleroderma Antibody 6.50    negative
Anti-beta 2 glycoprotein abs (IgG) 20.4    positive
Anti-beta 2 glycoprotein abs (IgM) 53.0    positive
Lupus anticoagulant 65.30  positive
Anti-cardiolipin IgM 3.58    negative
Anti-cardiolipin IgG >100   positive
U1RNP antibodies 16.06  negative
SSB/La antibody 10.7    negative



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hypocomplementemia, high levels of  anti-dsDNA, and 
disease activity while lupus nephritis and duration of  SLE 
disease were not (Alamoudi & Attar, 2015). The patient in 
our case report had recurrent left-sided pneumothorax 20 
years back secondary to underlying left lung apical lobe 
emphysematous changes; as written in his previously done 
Video-assisted thoracoscopic surgery (VATS) report; and 
bilateral cystic bronchiectasis, years following that event, 
he was diagnosed with active SLE. It is not clear whether 
remnant apical bullae or the presence of  severe cystic 
bronchiectasis were solely responsible for the patient’s 
recurrent pneumothorax. At that time, the possibility 
of  an underlying CTD was not considered. Although 
smoking and bronchiectasis are all well-known causes 
of  pneumothorax, we are suggesting that the presence 
of  an undiagnosed SLE might have caused the patient 
to be more susceptible to recurrent pneumothorax and 
contributed to the formation of  a more severe form of  
his cystic bronchiectasis with further worsening of  the 
outcome for the affected organ, which is the lung tissue 
in this case.

CONCLUSION
Pulmonary system involvement in SLE is much wider 
than what is thought to be and can present in any shape 
with varying degrees of  severity. The current 2019 
European Alliance of  Associations for Rheumatology 
(EULAR)/American College of  Rheumatology (ACR) 
classification criteria for systemic lupus erythematosus 
include only pleural effusion, pericardial effusion, and 
acute pericarditis, therefore, a high level of  suspicion is 
warranted when diagnosing unclassical SLE-related lung 
disease. Furthermore, it is crucial to screen for, diagnose, 
monitor, and treat all pulmonary symptoms in patients 
diagnosed with SLE, whether SLE is the cause or not, to 
prevent further deterioration and progression. We have 
to keep in mind that measures for SLE disease activity 
like SLEDAI and BILAG do not encompass the full 
spectrum of  lung disorders encountered in SLE patients. 
Additional research is warranted to ascertain the optimal 
management strategies for pulmonary involvement in 
SLE. It is noteworthy that individuals with SLE who 
contract acute respiratory infections may experience a 
higher risk of  developing structural lung problems such 
as pneumothorax and cystic changes. Physicians should 
be aware of  this possibility and treat all patients with 
autoimmune diseases who present with active infections 
promptly to prevent further tissue damage.

REFERENCES
Alamoudi, O. S., & Attar, S. M. (2015). Pulmonary 

manifestations in systemic lupus erythematosus: 

ANA by IF Titer = 1:80, with Nuclear Coarse Speckled pattern
anti-(DS)-DNA antibody 41.1   positive

ANA: antinuclear antibody testing; IF: immunofluorescence; C3 and C4: complement proteins; DAT: direct antiglobulin test; DS: 
double-stranded; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; IgA: immunoglobulin A; IgG: immunoglobulin G; and 
IgM: immunoglobulin M; CRP: C-reactive protein.

and a reduction of  repeated hospital admissions along 
with enhanced exertional dyspnea and an increased 
walking distance. The patient continued to follow up 
at the clinic with the pulmonology and rheumatology 
teams and currently staying in Riaydh city where he is 
undergoing assessments to establish his eligibility for the 
lung transplant.

DISCUSSION
Respiratory system involvement is common among SLE 
patients, it is estimated that up to 50% of  patients will have 
lung involvement during the course of  their disease which 
can involve the chest wall, pleura, airways, lung parenchyma, 
and pulmonary vasculature (Di Bartolomeo et al., 2021). 
Sometimes it can mimic other aetiologies and be treated 
unproperly. In order of  frequency, it includes pleuritis 
(40%–60%) pleural effusion (50%), acute pneumonitis 
(1%–12%), interstitial lung disease (3%–9%), shrinking 
lung syndrome (1%–6%), pulmonary hypertension 
(4%), diffuse alveolar hemorrhage, and thromboembolic 
disease (Amarnani et al., 2021). Pneumothorax is rare 
to be encountered, yet some reports have mentioned 
a group of  patients with synchronous pneumothorax 
and active SLE disease (Graves et al., 2022b). Regarding 
bronchiectasis, it has been associated with rheumatoid 
arthritis (RA) and Sjögren’s disease (SjD) more frequently 
than with SLE. Arthropathy and sicca features are usually 
advanced when bronchiectasis becomes apparent, yet 
in some cases, bronchiectasis occurs even before the 
rheumatic disease is diagnosed. The mechanism that 
lies behind the appearance of  bronchiectasis in patients 
with autoimmune diseases is not well established but 
probable causes include the presence of  abnormal cystic 
fibrosis gene (CFTR) allele in patients with RA, recurrent 
aspiration and traction in patients with interstitial lung 
disease, chronic inflammation with a weakened immune 
system, and multiple recurrent infections. All these 
mechanisms can contribute to the formation or worsening 
of  a previously formed dilated airway. Bronchiectasis 
has also been noted in association with other systemic 
diseases, such as inflammatory bowel disease (IBD) and 
yellow nail syndrome (Jakharia et al., 2022; Suarez-Cuartin 
et al., 2016). A large Swedish population-based register 
study of  lung disease among SLE patients showed that 
bronchiectasis occurs at a higher rate in patients with 
SLE compared to the general population (Forbess et 
al., 2019). Another 10-year retrospective hospital-based 
study conducted at KingAbdulaziz University Hospital 
(KAUH) in Saudi Arabia by Prof. S. Attar and Prof. O. 
Alamoudi, involved 184 SLE patients (61 with pulmonary 
involvement and 52 with HRCT abnormalities), showed 
a significant association between abnormal HRCT and 



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association with disease activity. Respirology, 20(3), 
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Amarnani, R., Yeoh, S.-A., Denneny, E. K., & Wincup, 
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Di Bartolomeo, S., Alunno, A., & Carubbi, F. (2021). 
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erythematosus. Pharmaceuticals, 14(3), 276. 

Forbess, L. J., Rossides, M., Weisman, M. H., & Simard, 
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Graves, N., Flint, J., Sagdeo, A., Askari, A., Ball, P., & 
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Imad, Z., Abdalla, Y. A., Hamza, S. B., Abubakr, M. 
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