Letter to the Editor
Acute fibrinous and organizing pneumonia
Neumonía organizada fibrinoide aguda
Daniela García-Huertas
, Alejandro López-Fernández, Inmaculada De Dios-Chacón
Corresponding author
Unidad de Cuidados Intensivos, Hospital Universitario Virgen de las Nieves, Granada, Spain
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A 70-year-old male patient, admitted to hospital in April 2020 for a 3/4-day history of dyspnoea at rest. In the targeted history-taking he reported dyspnoea on minimal effort during the previous 6 months. The following stand out in his personal history: HBP, obesity, hypercholesterolemia, ex-smoker (ICAT 40 packs/year), leukocytoclastic vasculitis and prostate cancer in 2013.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Given the epidemiological context, the symptoms, and the chest X-ray (bilateral alveolar-interstitial infiltrate), SARS-CoV-2 infection was established as the first diagnostic hypothesis, although the nasopharyngeal swab PCR and the serology were negative.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Despite initiating treatment protocol for Covid-19 (systemic corticosteroids and antibiotic therapy), progression was poor, requiring admission to the ICU and mechanical ventilation (MV). The patient experienced severe distress which prolonged his stay in the ICU for 2 months.</p><p id="par0025" class="elsevierStylePara elsevierViewall">SARS-CoV-2, atypical bacterial serology, HIV, IGRA, bronchial aspiration, bronchoalveolar lavage and echocardiography were negative. Simultaneously, a wide range of antibodies were requested, including rheumatoid factor, ANA, anti-DNA, ANCA, inflammatory myopathy and anti-glomerular basement membrane antibodies, which were also negative. Chest CT scan showed increased 'ground glass' density in most of the lung parenchyma and patchy alveolar consolidation areas.</p><p id="par0030" class="elsevierStylePara elsevierViewall">The study was completed with a surgical biopsy, reported as acute lung parenchymal injury leading to immature fibrosis, without hyaline membranes, with fibrinous exudate and foci of organizing pneumonia suggestive of AFOP.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Given this diagnosis, methylprednisolone boluses (500 mg/24 h for 3 days) followed by 1 mg/kg/24 h were administered, with slow clinical improvement allowing withdrawal of MV.</p><p id="par0040" class="elsevierStylePara elsevierViewall">The patient is transferred to the ward, where cyclophosphamide was added; later replaced by mycophenolate mofetil. In spite of all this, no improvement was seen in 2 CT scan controls and the patient remained with dyspnoea at rest and oxygen therapy, dying 6 months after admission to a hospital for the chronically ill.</p><p id="par0045" class="elsevierStylePara elsevierViewall">This disease was described as a result of the analysis of 17 suggestive cases of ALI, submitted to lung biopsy, which showed organized intra-alveolar fibrin deposition, areas of organizing pneumonia and patchy distribution.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Hyaline membranes, eosinophils, abscesses and granulomas must necessarily be absent for the diagnosis, which distinguishes it from classical interstitial lung diseases.</p><p id="par0050" class="elsevierStylePara elsevierViewall">The usual symptoms are dyspnoea, fever and cough.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Radiologically, there is a diffuse infiltrate and ground-glass image<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and the diagnosis of certainty requires lung biopsy.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There are idiopathic cases and cases secondary to infectious diseases, autoimmune diseases, neoplasms, transplants and toxins.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Acute forms are usually fulminant, while subacute forms have a better prognosis when treatment is established.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The need for MV was identified as the only adverse prognostic factor, but when there is an underlying disease, it is often responsible for the increased mortality.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Our case would correspond to a subacute, idiopathic form, which nevertheless had a fatal outcome that could be attributed to an established lung injury.</p><p id="par0065" class="elsevierStylePara elsevierViewall">AFOP is probably underdiagnosed given its similarity to other lung conditions. Its form of presentation means that it can be interpreted as a pneumonia refractory to antibiotic treatment<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> and its radiological pattern is comparable to that of any interstitial lung disease.</p><p id="par0070" class="elsevierStylePara elsevierViewall">The need for lung biopsy may cause a delay in diagnosis but it is of vital importance in cases such as this, as it may allow the patient to benefit from early treatment with steroids. There are no established recommendations regarding dose and treatment duration, finding variations from low doses to boluses of 250-1000 mg for 3 days.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5</span></a> The regimen is usually prolonged for months or years to avoid recurrences, both in idiopathic cases<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> as well as in those secondary to autoimmune diseases.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Immunosuppressants such as mycophenolate mofetil, azathioprine and cyclophosphamide can be used in both acute and maintenance phases.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">In conclusion, this is a disease with high mortality, which should be considered in the context of ARF with unfavourable progression after ruling out the usual causes, in order to establish an effective treatment as soon as possible.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0085" class="elsevierStylePara elsevierViewall">This article has not received any type of funding.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflict of interests</span><p id="par0090" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Funding" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Conflict of interests" ] 2 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: García-Huertas D, López-Fernández A, De Dios-Chacón I. Neumonía organizada fibrinoide aguda. Med Clin (Barc). 2022;158:147–148.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "M.B. Beasley" 1 => "T.J. Franks" 2 => "J.R. Galvin" 3 => "B. Gochuico" 4 => "W.D. 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