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Systemic Lupus Erythematosus Presenting as Acute Lupus Pneumonitis
Lupus eritematoso sistémico manifestado como neumonitis lúpica aguda
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(A) Chest X-ray showing right pleural effusion (asterisk) and pulmonary consolidation in right upper lobe. (B) On the chest X-ray three days later, a right encapsulated pleural effusion (asterisk) was observed. (C) Practical disappearance of encapsulated effusion in the thoracic study two months later, with a thickening of the right costal pleura and a lateral costophrenic sinus. 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(A) Chest X-ray showing right pleural effusion (asterisk) and pulmonary consolidation in right upper lobe. (B) On the chest X-ray three days later, a right encapsulated pleural effusion (asterisk) was observed. (C) Practical disappearance of encapsulated effusion in the thoracic study two months later, with a thickening of the right costal pleura and a lateral costophrenic sinus. (D) Axial section of the computed tomography study at 6 months, at the level of the pulmonary bases with a mediastinum window was normal.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Systemic lupus erythematosus (SLE) is an autoimmune chronic systemic disease involving skin, joints and/or internal organs, such as lungs, brain and heart. SLE affects predominantly women (female to male ratio 10:1).<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">1</span></a> Pulmonary manifestations of SLE can include a wide spectrum of diseases. The most common is lupus pleuritis. Less common is parenchymal involvement, presenting either as acute lupus pneumonitis (ALP) or chronic interstitial lung disease. Possible pulmonary manifestations of SLE are pneumonia, pulmonary embolism, pneumothorax, acute reversible hypoxemia, shrinking lung syndrome and pulmonary haemorrhage.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">2</span></a> It is believed that pulmonary complications are the consequence of the immune complex mediated injury.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 66-year-old woman was presented to the Emergency Department, having severe breath shortness, high temperature and chest pain. She has been suffered from systemic lupus erythematosus for 20 years, and for the last six months was using 10<span class="elsevierStyleHsp" style=""></span>mg Prednisone and Methotrexate 5<span class="elsevierStyleHsp" style=""></span>mg once weekly. At the examination, she was found to have elevated body temperature 38.1<span class="elsevierStyleHsp" style=""></span>°C, tachycardia (120<span class="elsevierStyleHsp" style=""></span>beats/min) and elevated blood pressure (160/90<span class="elsevierStyleHsp" style=""></span>mm Hg). Her appearance was anorexic, diaphoretic, dyspnoeic, slightly disoriented. Her pupils were round equally with good light reaction, and her extraocular muscles were intact. She had neither neck adenopathy, nor jugular venous distention or meningismus. Precordial examination has shown no murmurs, rubs or gallops. Her breath sounds were decreased, with the right side percussion dullness. She had 2+ pitting edema to the midcalf. Examined neurologically, she had mild confusion but was nonfocal otherwise. Routine blood examination revealed normochromic normocytic anemia (haemoglobin: Hgb 11.1<span class="elsevierStyleHsp" style=""></span>gm/dL), white blood cells: WBC count 24<span class="elsevierStyleHsp" style=""></span>600/μL, platelets count 68<span class="elsevierStyleHsp" style=""></span>000/μL and an erythrocyte sedimentation rate (ESR) 120<span class="elsevierStyleHsp" style=""></span>mm in the 1st hour. Serum biochemistry revealed C-reactive protein (CRP) 3<span class="elsevierStyleHsp" style=""></span>mg/dL, whereas unremarkable results were reported from urine analysis and urinoculture. Arterial blood gas analysis showed hypoxemia with respiratory alkalosis. Chest radiograph showed consolidation of the right upper lobe and a mild right-sided pleural effusion on the right side (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A). Sputum culture, blood culture, sputum microscopy for acid fast bacilli, Mantoux test and HIV serology were negative. Treatment of the patient was started with empiric intravenous antibiotics (cefriakson, ciprofloksacin and metronidazole) accompanied with supportive care. On the 3rd day of the therapy, patient had high body temperature of 40<span class="elsevierStyleHsp" style=""></span>°C and the control chest X-ray has revealed the encapsulated pleural effusion on the right side (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). A diagnostic thoracocentesis was performed, yielding 60<span class="elsevierStyleHsp" style=""></span>mL of yellow, slightly cloudy fluid, showing a red blood cell-RBC count of 2.0<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleSup">3</span>/μL and, WBC count of 340/μL (15% leukocytes, 71% neutrophils, and 13% monocytes); pH 7.48; glucose 58<span class="elsevierStyleHsp" style=""></span>mg/dL; total protein 2.46<span class="elsevierStyleHsp" style=""></span>g/dL, lactate dehydrogenase 12<span class="elsevierStyleHsp" style=""></span>545<span class="elsevierStyleHsp" style=""></span>U/L, adenosine deaminase 51.3<span class="elsevierStyleHsp" style=""></span>units/L; Ziehl-Neelsen stain negative; Gram stain and culture negative and Mycobacterium DNA polymerase chain reaction negative, with no malignant cells. Bacterial cultures were negative. Fiber-optic bronchoscopy with bronchoalveolar lavage (BAL) fluid analysis has shown epithelial cells. Since the patient had a continuously high body temperature up to 39<span class="elsevierStyleHsp" style=""></span>°C and round rash emerging over the left elbow, anti-nuclear antibody (ANA) testing was performed and was positive (titer 1:1280, homogeneous pattern), with anti-ds-DNA weakly positive, while perinuclear and cytoplasmic anti-neutrophil cytoplasmic antibody (p-ANCA and c-ANCA, respectively) were negative. Serum C3 and C4 levels were decreased (30<span class="elsevierStyleHsp" style=""></span>mg/dL and 7<span class="elsevierStyleHsp" style=""></span>mg/dL, respectively). According to results, a diagnosis of SLE presenting with acute pneumonitis was made. On the 4th day, the patient was started with 1<span class="elsevierStyleHsp" style=""></span>g intravenous methyl prednisolone once a day for 3 days, followed by tablet hydroxychloroquine 400<span class="elsevierStyleHsp" style=""></span>mg daily and tablet prednisone 1<span class="elsevierStyleHsp" style=""></span>mg/kg daily for 6 weeks with gradually tapering of prednisolone to a maintenance dose of 10<span class="elsevierStyleHsp" style=""></span>mg daily. From two months after, her chest X-ray has shown practical resolution (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>C). At six month later, computed tomography described adhesions of the right side with no other specific abnormalities (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>D).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Acute lupus pneumonitis (ALP) is an uncommon manifestation of lupus, affecting less than 2% of cases. It is often life threatening once ventilator failure sets in, with mortality rate of more than 50%, despite of the treatment.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> The main pathology in ALP could be the acute alveolar capillary unit injury.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">4</span></a> Lupus pneumonitis presents with acute onset of fever, cough, tachypnea and hypoxia. The usual radiological sign of lupus pneumonitis is consolidation in one or more lung areas, typically basal and bilateral, often associated with pleural effusion and pulmonary arterial hypertension.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">5</span></a> Our case was difficult to diagnose at first, since the onset symptoms indicated infection etiology and do the relevant work up. We have excluded infective pneumonia by repeated sputum analyses and single BAL fluid examination; alveolar hemorrhage since there were no hemoptysias and Hemosiderin-laden macrophage was absent in BAL fluid. The mainstay of acute lupus pneumonitis treatment is the systemic corticosteroids usage (prednisone 1–1.5<span class="elsevierStyleHsp" style=""></span>mg/kg/d divided accordingly), although despite high-dose corticosteroid usage, the lupus pneumonitis mortality remains high.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> If there is no response to oral corticosteroids within 72<span class="elsevierStyleHsp" style=""></span>h and the patient has marked tachypnea, hypoxemia or suspected diffuse alveolar hemorrhage, treatment should include intravenous corticosteroid pulse therapy (i.e., 1<span class="elsevierStyleHsp" style=""></span>g methylprednisolone per day for 3 days).<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">3</span></a> The corticosteroid improvement was impressive in our case, noticed on the very first day.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In conclusion, acute lupus pneumonitis can be the initial manifestation of SLE. ALS diagnosis is essential, by excluding other causes of lung infiltration, such are infective pneumonia (bacterial, mycobacterial, fungal and viral), organizing pneumonia (OP), alveolar hemorrhage, pulmonary embolism and volume overload state, due to either renal failure or to congestive heart failure.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">6</span></a> Also, it is critically important to differentiate ALP from diffuse alveolar hemorrhage (DAH) which may have similar clinical presentation, laboratory immunology testing (ANA, anti-dsDNA) and radiographic findings, with almost equally grave prognosis. ESR and CRP may be used to support clinical suspicion. Unlike ESR, CRP (or hs-CRP) elevation is only modest in active SLE without infection, while a high hs-CRP level (>5–6<span class="elsevierStyleHsp" style=""></span>mg/dL) is a strong predictor of infection. Also, ESR/CRP ratio ¿15 suggests lupus flare, while ratio ¿2 suggests infection.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">7</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1179 "Ancho" => 1250 "Tamanyo" => 188470 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">66-Year-old woman with systemic lupus erythematosus presenting with acute lupus pneumonitis. (A) Chest X-ray showing right pleural effusion (asterisk) and pulmonary consolidation in right upper lobe. (B) On the chest X-ray three days later, a right encapsulated pleural effusion (asterisk) was observed. (C) Practical disappearance of encapsulated effusion in the thoracic study two months later, with a thickening of the right costal pleura and a lateral costophrenic sinus. (D) Axial section of the computed tomography study at 6 months, at the level of the pulmonary bases with a mediastinum window was normal.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0040" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Respiratory complications in the collagen vascular diseases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "N.W. Todd" 1 => "R.A. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 4 | 3 | 7 |
| 2024 October | 43 | 40 | 83 |
| 2024 September | 60 | 38 | 98 |
| 2024 August | 75 | 53 | 128 |
| 2024 July | 57 | 41 | 98 |
| 2024 June | 84 | 47 | 131 |
| 2024 May | 72 | 41 | 113 |
| 2024 April | 49 | 40 | 89 |
| 2024 March | 53 | 25 | 78 |
| 2024 February | 24 | 24 | 48 |
| 2023 October | 1 | 0 | 1 |
| 2023 March | 16 | 4 | 20 |
| 2023 February | 50 | 25 | 75 |
| 2023 January | 57 | 36 | 93 |
| 2022 December | 65 | 29 | 94 |
| 2022 November | 75 | 34 | 109 |
| 2022 October | 97 | 44 | 141 |
| 2022 September | 102 | 37 | 139 |
| 2022 August | 59 | 54 | 113 |
| 2022 July | 93 | 60 | 153 |
| 2022 June | 114 | 35 | 149 |
| 2022 May | 116 | 50 | 166 |
| 2022 April | 144 | 34 | 178 |
| 2022 March | 161 | 48 | 209 |
| 2022 February | 180 | 41 | 221 |
| 2022 January | 187 | 60 | 247 |
| 2021 December | 209 | 65 | 274 |
| 2021 November | 167 | 52 | 219 |
| 2021 October | 187 | 55 | 242 |
| 2021 September | 142 | 61 | 203 |
| 2021 August | 141 | 35 | 176 |
| 2021 July | 132 | 34 | 166 |
| 2021 June | 211 | 51 | 262 |
| 2021 May | 210 | 45 | 255 |
| 2021 April | 431 | 92 | 523 |
| 2021 March | 357 | 49 | 406 |
| 2021 February | 240 | 27 | 267 |
| 2021 January | 219 | 21 | 240 |
| 2020 December | 278 | 39 | 317 |
| 2020 November | 405 | 39 | 444 |
| 2020 October | 285 | 36 | 321 |
| 2020 September | 354 | 20 | 374 |
| 2020 August | 354 | 32 | 386 |
| 2020 July | 357 | 46 | 403 |
| 2020 June | 415 | 31 | 446 |
| 2020 May | 429 | 31 | 460 |
| 2020 April | 328 | 30 | 358 |
| 2020 March | 197 | 18 | 215 |
| 2020 February | 276 | 39 | 315 |
| 2020 January | 253 | 30 | 283 |
| 2019 December | 256 | 37 | 293 |
| 2019 November | 73 | 21 | 94 |
| 2019 October | 45 | 15 | 60 |
| 2019 September | 40 | 14 | 54 |
| 2019 August | 48 | 27 | 75 |
| 2019 July | 39 | 16 | 55 |
| 2019 June | 43 | 22 | 65 |
| 2019 May | 54 | 12 | 66 |
| 2019 April | 40 | 11 | 51 |
| 2019 March | 52 | 24 | 76 |
| 2019 February | 49 | 23 | 72 |
| 2019 January | 184 | 23 | 207 |
| 2018 December | 54 | 17 | 71 |
| 2018 November | 52 | 26 | 78 |
| 2018 October | 44 | 24 | 68 |
| 2018 September | 3 | 1 | 4 |
| 2018 April | 1 | 1 | 2 |
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