Severe lupus pneumonitis: A case of life-saving multimodal therapy with rituximab and corticosteroids.

Severe lupus pneumonitis is a rare and life-threatening complication of systemic lupus erythematosus (SLE), characterized by its rapid progression and high mortality rate. This case report describes the clinical trajectory and therapeutic management of a young Aboriginal female with established lupus nephritis who developed severe lupus pneumonitis. Despite her stable renal condition under long-term immunosuppressive treatment, she experienced acute respiratory distress, leading to her admission to the intensive care unit and subsequent mechanical ventilation. The diagnostic process was complicated by the difficulty in obtaining tissue biopsies, necessitating reliance on clinical judgement and radiological evidence to formulate a diagnosis. The patient was treated with pulsed intravenous methylprednisolone followed by rituximab infusions, resulting in significant clinical and radiological improvement. This case highlights the importance of early and intensive immunosuppressive therapy in the management of severe lupus pneumonitis and underscores the utility of a multidisciplinary approach in overcoming diagnostic ambiguities. Furthermore, it contributes to the growing body of evidence supporting the efficacy of rituximab in severe lupus pneumonitis cases, offering insights into potential therapeutic avenues when conventional management strategies are inadequate or unsuitable.

Systemic lupus erythematosus-related acute lung disease.

INTRODUCTION: Systemic lupus erythematosus (SLE) is non-organ specific autoimmune disease with mainly skin, joint, and kidney involvement. SLE-related acute lung disease (ALD) is rare, poorly investigated and can lead to acute respiratory failure. We conducted a retrospective study aiming to describe clinical features, treatments and outcome of SLE-related APD. METHODS: We retrospectively included all patients with SLE and ALD admitted from November 1996 and September 2018 to La Pitié-Salpêtrière Hospital, after exclusion of viral or bacterial lung infection, cardiac failure or any other alternate diagnosis. RESULTS: During the time of the study, 14 patients with 16 episodes were admitted to our center: female 79%, mean age ± SD at admission 24 ± 11 years. ALD was inaugural of the SLE in 70% cases. SLE main organ involvement were: arthritis 93%, skin 79%, serositis 79%, hematological 79%, kidney 64%, neuropsychiatric 36% and cardiac 21%. 11 episodes required ICU admission for a median time of 8 days. Chest CT-scan revealed mostly basal consolidation and ground-glass opacities. When available, bronchoalveolar lavage mostly revealed a neutrophilic alveolitis with alveolar hemorrhage in 67% cases. Symptomatic respiratory treatments were: oxygen 81%, high-flow nasal canula oxygen 27%, non-invasive ventilation 36%, mechanical ventilation 64% and venovenous extracorporeal membrane oxygenation 18%. SLE-specific treatments were: corticosteroids 100%, cyclophosphamide 56% and plasma exchange 25%. All patients but one survived to ICU and hospital discharge. Two patients had a relapse of SLE-related ALD but none had interstitial lung disease during follow-up. CONCLUSION: Systemic lupus erythematosus-related acute respiratory failure is a severe event, mostly occurring at SLE onset, typical harboring a basal consolidation pattern on chest CT-scan and alveolar hemorrhage on BAL pathological examination. Mortality in our cohort is lower than previously reported but these results needs to be confirmed in further larger studies.

Acute lupus pneumonitis resembling miliary tuberculosis: A case-based review.

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.

Systemic lupus erythematosus presenting as non-resolving pneumonia: a case report.

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.

20-Year-old female with fever, cough, and dyspnea: Acute lupus pneumonitis during the pandemic of coronavirus disease 2019 (COVID-19).

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.

Acute onset systemic lupus erythematosus interstitial lung disease: A case report.

A 40-year-old Japanese man was diagnosed with systemic lupus erythematosus. Chest computed tomography showed patchy consolidation in both lungs. A cryobiopsy and bronchoalveolar lavage showed organizing pneumonia, not acute lupus pneumonia or diffuse alveolar hemorrhage. This case demonstrates the usefulness of cryobiopsy for the management of systemic lupus erythematosus interstitial lung disease.

Coevality of Systemic Lupus Erythematosus With Sickle Cell Trait: A Not So Uncommon Entity.

The coexistence of systemic lupus erythematosus (SLE) with sickle cell trait is quite sparingly reported in literature. Here, we narrate the case of a 17-year-old girl from Eastern India with sickle cell trait who presented with acute lupus pneumonitis. The challenges to the final diagnosis of SLE with sickle cell trait were because of the often lesser degree of clinical suspicion at the outset. In this report, we discuss this not so uncommon combination of conditions and review related literature. This girl, who was a known case of sickle cell trait, presented with fever, cough, shortness of breath with subsequent rashes, oral ulceration, high erythrocyte sedimentation rate (ESR) and proteinuria. After ruling out infective causes, she was found to be antinuclear antibody (ANA) positive and with stage 4 lupus nephritis. Emphasis should be given to the presence of autoimmune conditions in patients with sickle hemoglobinopathies, including sickle cell trait wherein atypical or systemic involvement may occur. Such association holds more importance as sickle hemoglobinopathies is one of the major hemoglobinopathies reported in this part of the country.

Lupus and the Lungs: The Assessment and Management of Pulmonary Manifestations of Systemic Lupus Erythematosus.

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.

Pulmonary manifestations in systemic lupus erythematosus: pleural involvement, acute pneumonitis, chronic interstitial lung disease and diffuse alveolar hemorrhage. / Manifestaciones pulmonares en lupus eritematoso sistémico: afección pleural, neumonitis aguda, enfermedad intersticial crónica y hemorragia alveolar difusa.

Systemic lupus erythematosus is the diffuse autoimmune connective tissue disease that most frequently involves pulmonary involvement, affecting 20% of 90% of the patients. The percentage varies depending on the defining criteria (symptoms, pulmonary tests or histopathological studies). At least once during the disease course, 50% of those affected have pleural and/or pulmonary manifestations, which are associated with higher morbidity and mortality. Pulmonary involvement has no correlation with lupus activity biomarkers, and it is necessary to rule out infectious processes in the initial approach. Bacterial infection is most frequently the cause of lung involvement in lupus and is one of the most important causes of death. Pulmonary involvement is considered to be primary when it is associated with disease activity, and secondary when other causes participate. Drugs have been reported to be associated with pulmonary damage, including interstitial disease. The incidence of malignant lung diseases is increased in systemic lupus erythematosus. Treatment depends on the type and severity of pulmonary involvement.

Isolated acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus in an 8-year-old girl.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which has broad pleuropulmonary manifestations. One of the rare and mortal complications is acute lupus pneumonitis, which is reported very rarely, especially in childhood. Herein, we report an 8-year-old girl with isolated acute lupus pneumonitis as the initial presentation that required a lung biopsy for diagnosis. Although she had improvement with the administration of steroids, steroid treatment was reduced due to the drug's side effects resulting in the addition of azathioprine and mycophenolate mofetil to the treatment regimen. After the new regimen failed to result in clinical improvement, hydroxychloroquine treatment was started and a significant improvement was observed. Acute lupus pneumonitis is an uncommon manifestation of SLE and diagnosis may be difficult in patients without other organ involvement.

Lupus pneumonitis presenting with high titre of anti-Ro antibody.

Lupus pneumonitis carries high mortality and is a rare manifestation of systemic lupus erythematosus (SLE). However, it is difficult to diagnose and is often mistaken as pneumonia, alveolar haemorrhage, or organizing pneumonia. Previous studies demonstrated that serum anti-Ro antibodies are elevated more frequently in SLE patients with pneumonitis than in those without. We report a 21-year-old female who was newly diagnosed as having SLE with nephritis and who suddenly developed right lung opacity and rapidly progressed to severe hypoxaemia despite the use of broad-spectrum antibiotics. The serum titre of anti-Ro antibody was greater than 240 U/mL. She underwent lung biopsy and lupus pneumonitis was confirmed by the pathological findings. Subsequently, she showed a favourable response to plasma exchange, steroid pulse therapy, and mycophenolate mofetil (MMF) treatment. For SLE patients with pulmonary infiltrates, high degree of clinical suspicion of lupus pneumonitis is required and measurement of serum anti-Ro antibody may help to make the diagnosis.

Acute lupus pneumonitis followed by intestinal pseudo-obstruction in systemic lupus erythematosus: A case report.

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.

Lupus pneumonitis as the initial presentation of systemic lupus erythematosus: case series from a single institution.

Objective The aim of this study was to examine the clinical features, treatment and outcome of systemic lupus erythematosus (SLE) patients in our centre who presented with lupus pneumonitis as the initial manifestation. Methods We performed a retrospective review of all patients who presented with lupus pneumonitis during the initial SLE manifestation from March 2006 to March 2015. Results There were a total of five patients in our study who presented with fever and cough as the main clinical features. All patients had pulmonary infiltrates on chest radiographs. High-resolution computed tomography, which was performed in two patients, showed ground glass opacities with patchy consolidations bilaterally. All patients received high-dose steroids, 80% received intravenous cyclophosphamide and 60% received intravenous immunoglobulin. Two patients died from severe lupus pneumonitis within 2 weeks of admission despite treatment with ventilation, steroids, cyclophosphamide and intravenous immunoglobulin. Conclusions Acute lupus pneumonitis is an uncommon presentation of SLE. Mortality in this case series is 40%.

Neumonitis lúpica con derrame pleural refractario: Pneumonitis lupica with pleural effusion refractory

RESUMEN: El Lupus Eritematoso Sistémico suele afectar el aparato respiratorio, en un 70% de los casos, siendo la pleuritis la manifestación más frecuente, con o sin derrame pleural. Es en general unilateral, autolimitado y con buena respuesta al tratamiento. Presentamos el caso de una pacientes que debuta con lupus eritematoso sistémico con extenso compromiso respiratorio que interpretamos como una neumonitis lúpica asociado a derrame pleural bilateral refractario al tratamiento corticoideo e inmunosupresor intenso.

ABSTRACT Systemic Lupus Erythematosus usually affects the respiratory system, 70% of cases, pleurisy being the most common manifestation, with or without pleural effusion. It is unilateral, self-limited and generally good response to treatment. We present the case of patients with Systemic Lupus Erythematosus debut with extensive respiratory compromise interpret as a lupus pneumonitis associated with bilateral pleural effusion refractory to corticosteroid and immunosuppressive therapy intense.

New-onset systemic lupus erythematosus in a long-term hemodialysis patient with acute pleuritis and pneumonitis.

A 61-year-old woman, with a 25-year history of maintenance hemodialysis due to end-stage renal disease of unknown causes, was admitted because of systemic joint pain and inflammatory response of unknown etiology that persisted for 1 month. Laboratory data on admission revealed leukocytopenia, lymphocytopenia, high serum C-reactive protein, and positivity for antinuclear antibody (ANA) and anti-double strand DNA. After admission, she progressively developed cough and dyspnea. A chest radiograph revealed bilateral ground glass opacity and pleural effusion. A thoracentesis revealed lupus erythematosus cells, suggesting lupus pleuritis. A chest computed tomography showed a pattern of diffuse alveolar damage compatible with acute lupus pneumonitis. She fulfilled the American Rheumatism Association diagnostic criteria for systemic lupus erythematosus (SLE). Methylprednisolone pulse therapy followed by oral prednisone treatment improved the clinical symptoms and laboratory abnormalities. ANA was negative 25 years earlier when she first started hemodialysis and she had neither clinical nor serological abnormalities related to SLE during the last 25 years. Further, she had neither received drugs that can cause drug-induced SLE, nor had a history of ultraviolet ray exposure, pregnancy, blood transfusion, trauma and smoking. This report suggests that new-onset SLE can develop in patients undergoing long-term dialysis. Hence, when we encounter dialysis patients with arthralgia and/or respiratory disorders, we should consider the possibility of new-onset SLE.

A fatal case of seronegative, late-onset systemic lupus erythematosus presenting with motor sensory axonal polyneuropathy.

Systemic lupus erythematosus is a multisystemic, autoimmune, inflammatory disorder predominantly affecting young females. Its onset may be abrupt or insidious, presenting with a broad range of clinical and immunological features. We report an unusual case of elderly-onset systemic lupus erythematosus in a woman initially diagnosed with discoid lupus, and subsequently admitted to hospital due to a progressive psycho-motor deficit. Electrophysiological measurements suggested a diagnosis of acute motor sensory axonal neuropathy. Unusual clinical features and negative serology led to diagnostic uncertainty. This case report offers information on the course of the disease through the entire chain of the health care delivery (from primary to tertiary). Despite the efforts of the hospital staff, it was not possible to save the life of the woman.

Non-invasive ventilation in patients with acute lupus pneumonitis: A case report and review of literature.

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.

Bilateral acute lupus pneumonitis in a case of rhupus syndrome.

Rhupus syndrome, the overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), is an extremely uncommon condition. Organ damages found due to SLE are usually mild in rhupus. Lupus pneumonitis in rhupus syndrome has not been reported worldwide. We are reporting a 23-year-old female with bilateral symmetric erosive arthritis, oral ulcer, alopecia, polyserositis, anemia, leucopenia, positive RA-factor, anti nuclear antibody (ANA) and anti ds-DNA. She presented with acute onset dyspnea, high fever, chest pain, tachycardia, tachypnea, hypoxia and respiratory alkalosis. High resolution computed tomography (HRCT)-thorax showed bilateral, basal consolidation with air bronchogram. Repeated sputum and single broncho alveolar lavage (BAL) fluid examination revealed no organism or Hemosiderin-laden macrophage. The diagnosis of rhupus was confirmed by combined manifestations of RA and SLE, and the diagnosis of acute lupus pneumonitis was established by clinico-radiological picture and by excluding other possibilities.