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Systemic Lupus Erythematosus (SLE) is a multifaceted, multisystem autoimmune disorder with diverse clinical expressions. While prevalence reports vary widely, pulmonary involvement accounts for significant morbidity and mortality in SLE. This comprehensive review explores the spectrum of pulmonary disease in SLE, including upper airway manifestations (e.g., laryngeal affection), lower airway conditions (e.g., bronchitis, bronchiolitis, bronchiectasis), parenchymal diseases (e.g., interstitial lung disease, acute lupus pneumonitis, diffuse alveolar hemorrhage), pleural diseases (e.g., serositis, shrinking lung syndrome), and vascular diseases (e.g., pulmonary arterial hypertension, pulmonary embolism, acute reversible hypoxemia syndrome). We discuss diagnostic modalities, treatment strategies, and prognosis for each pulmonary manifestation. With diagnostics remaining a challenge and with the absence of standardized treatment guidelines, we emphasize the need for evidence-based guidelines to optimize patient care and improve outcomes in this complex disease. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems and organs, with pulmonary involvement known to be associated with disease prognosis and mortality. Acute lupus pneumonitis (ALP) resembling miliary tuberculosis (TB) is rare. Here, we present a case of ALP mimicking miliary TB and review the literature. A 19-year-old male student was referred to our hospital with fever. Although the patient met the diagnostic criteria for SLE, diffuse miliary nodules in both lungs were observed on a chest computed tomography scan. A series of tests, including pathological testing of bronchoscopy brushes, T-lymphocyte culture + interferon assay (A + B), tuberculin test, detection of mycobacterium TB DNA, and acid-fast bacilli smear in bronchoalveolar lavage fluid, were unable to confirm the presence of TB infection. We considered the patient to have ALP. After beginning ALP therapy, his symptoms disappeared, and the imaging and hematological results returned to normal. Miliary TB and ALP have similar clinical manifestations and imaging changes, which make diagnosis difficult. This case highlights the need to ensure accurate diagnosis and treatment to improve prognosis.

Acute lupus pneumonitis (ALP) is a rare first-presenting manifestation of systemic lupus erythematosus (SLE). The characteristic symptoms are rapid onset of fever, cough (sometimes with hemoptysis), and dyspnea. ALP may progress to acute respiratory distress syndrome (ARDS), and it is a potentially fatal disease unless treated. Coronavirus disease 19 (COVID-19) has overlaps with ALP in terms of clinical presentation, and laboratory and radiological findings. This report describes a case of a young female patient presenting with ARDS during the pandemic of COVID-19. She had pancytopenia, elevated CRP, ferritin, and liver indices resembling macrophage activation syndrome. She also had hepatosplenomegaly, a small spleen infarct, adenopathy, minimal pleural, and pericardial effusion. After excluding COVID-19 by PCR and antibody tests, and other infections by cultures, with the help of antinuclear antibody and anti–double-stranded DNA, SLE and ALP were diagnosed, and she was treated with high-dose steroid and intravenous immunoglobulin. In conclusion, if patients presenting with pneumonia or ARDS have one or more of the findings of arthritis, serositis, rash, oral/nasopharyngeal ulcerations, cytopenias, and renal or neurologic disorder, SLE and ALP should be considered in differential diagnoses. Because of the high mortality rate of ALP reaching up to 50%, early diagnosis and immunosuppressive therapy are of vital importance. [ABSTRACT FROM AUTHOR]

Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO2) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended. [ABSTRACT FROM AUTHOR]

Acute lupus pneumonitis is an uncommon but life threatening condition associated with systemic lupus erythematosus. We report the case of a young female who presented to us with acute hypoxemic respiratory failure secondary to acute lupus pneumonitis as initial presenting manifestation of lupus. She was managed with non-invasive ventilation and pulse steroids, with which she had dramatic improvement.

BackgroundSystemic lupus erythematosus (SLE) is a rheumatological disorder with a heterogeneous clinical presentation and disease course. Case presentationWe report a case concerning a young woman with pleuropneumonia, non-responsive to conventional antibiotic therapy, who was, upon further inquiry and passage of time, diagnosed with SLE. Key pointsBy means of this case, we would like to emphasize the clinical implications and prognostic significance of lymphopenia in patients with SLE. Moreover, we attempt to make the reader aware of some of the protean manifestations of SLE and we would like to raise suspicion of acute lupus pneumonitis by demonstrating a case of a young female with non-resolving pneumonia.

Systemic lupus erythematosus (SLE) is an autoimmune disease with a multisystem involvement. Pleuro-pulmonary manifestations are present in almost half of the patients during the disease course and may be the presenting symptoms in 4-5% of patients with SLE. Acute lupus pneumonitis (ALP) is an uncommon manifestation reported to occur in up to 4% of lupus cohorts, in 50% of whom it may be the presenting symptom. Usually, ALP occurs during a generalized lupus flare and occurs in association with multisystem involvement. The clinical presentation is nonspecific with fever, dyspnea, pleuritic chest pain, and cough with minimal sputum production. Histological features of ALP are nonspecific. The differential diagnosis of ALP is broad and includes infection, alveolar hemorrhage, pulmonary embolism, etc. The therapy for ALP is high dose systemic corticosteroids and other immunosuppressive drugs. The aim of this paper is to describe an unusual presentation of ALP characterized by unilateral pulmonary involvement and review literature. [ABSTRACT FROM AUTHOR]

Acute lupus pneumonitis is an uncommon but life threatening condition associated with systemic lupus erythematosus. We report the case of a young female who presented to us with acute hypoxemic respiratory failure secondary to acute lupus pneumonitis as initial presenting manifestation of lupus. She was managed with non-invasive ventilation and pulse steroids, with which she had dramatic improvement. [ABSTRACT FROM AUTHOR]

Pulmonary manifestations of systemic lupus erythematosus (SLE) are wide-ranging and debilitating in nature. Previous studies suggest that anywhere between 20 and 90% of patients with SLE will be troubled by some form of respiratory involvement throughout the course of their disease. This can include disorders of the lung parenchyma (such as interstitial lung disease and acute pneumonitis), pleura (resulting in pleurisy and pleural effusion), and pulmonary vasculature [including pulmonary arterial hypertension (PAH), pulmonary embolic disease, and pulmonary vasculitis], whilst shrinking lung syndrome is a rare complication of the disease. Furthermore, the risks of respiratory infection (which often mimic acute pulmonary manifestations of SLE) are increased by the immunosuppressive treatment that is routinely used in the management of lupus. Although these conditions commonly present with a combination of dyspnea, cough and chest pain, it is important to consider that some patients may be asymptomatic with the only suggestion of the respiratory disorder being found incidentally on thoracic imaging or pulmonary function tests. Treatment decisions are often based upon evidence from case reports or small cases series given the paucity of clinical trial data specifically focused on pulmonary manifestations of SLE. Many therapeutic options are often initiated based on studies in severe manifestations of SLE affecting other organ systems or from experience drawn from the use of these therapeutics in the pulmonary manifestations of other systemic autoimmune rheumatic diseases. In this review, we describe the key features of the pulmonary manifestations of SLE and approaches to investigation and management in clinical practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Amarnani, Yeoh, Denneny and Wincup.)

Acute lupus pneumonitis is a rare initial presentation of systemic lupus erythematosus (SLE). We report a 19-year-old female presenting with fever and recurrent hemoptysis with radiological evidence of parenchymal lung involvement with mild pleural effusion. Subsequent development of malar and discoid rash with anti-nuclear antibodies (ANA) and anti-dsDNA positivity clinched the diagnosis. Her clinical signs and symptoms resolved with a course of intravenous pulse methyl-prednisolone along with radiological resolution [ABSTRACT FROM AUTHOR]

Intestinal pseudo-obstruction (IpsO) and acute lupus pneumonitis (ALP) are uncommon severe complications of systemic lupus erythematosus (SLE). The present study reports the case of a 26-year-old female who presented with abdominal pain, nausea and vomiting as initial symptoms. Computed tomography (CT) scanning revealed the jejunal wall was thickened and streaky, mimicking the presentation of intestinal obstruction. Following emergency surgery, the patient's general condition was aggravated, with evident limb erythematous rashes. A series of laboratory examinations revealed SLE, and combined with patient's medical history IpsO was diagnosed, with a disease Activity Index score of 10. During the therapeutic period, high fever, dyspnea and oxygen saturation (SaO 2 ) reductions were detected, and CT scans indicated lung infiltration, excluding other causes through a comprehensive infectious work-up and a bronchoalveolar lavage examination. ALP was confirmed and treated with high-dose methylprednisolone and gamma globulin supplement. The patient responded well and was discharged in 2 weeks. In the one-year tapering period and after stopping corticosteroids, the patient recovered well with no relapse detected. In conclusion, the manifestation of IpsO in SLE is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention. ALP may be the consequence of emergency surgery, and immediate high-dose glucocorticoid therapy is recommended.