A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia

Abstract

Adult-onset Still's disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still's disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still's disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still's disease with acute fibrinous organising pneumonia.Keywords clinical, interstitial lung disease, other rheumatology, respiratory medicine, rheumatology Case presentationOur patient was a Caucasian man who initially presented aged 33 with a seven-week history of gradually worsening fevers, night sweats, lethargy and 6 kg weight loss. He had no significant medical history, was on no regular medications and worked in Information Technology. Admission blood tests showed a total white cell count (WCC) of 2.38 (10 9 / L), neutrophils of 1.5 (10 9 /L), lymphocytes of 0.6 (10 9 /L), haemoglobin of 10.4 (g/dl) and platelets of 209 (10 9 /L) (which subsequently dropped to 135). Liver enzymes showed an alanine aminotransferase (ALT) of 59 (IU/L), bilirubin of 32 (mmol/L) and alkaline phosphatase (ALP) of 57 (IU/L). His c-reactive protein (CRP) was 44 (mg/L) and ferritin elevated at 728 (mg/L). Computerised tomography (CT) of the chest showed multiple bilateral lung nodules and splenomegaly. Due to his fever and imaging appearances (Figure 1), he was treated with broadspectrum antibiotics for possible atypical pneumonia. Multiple investigations including several blood cultures were performed to look for an infective cause of the fever all of which were negative. Autoimmune screen was unremarkable. He continued to deteriorate despite antibiotics developing type-1 respiratory failure requiring non-invasive ventilation. His blood tests also worsened becoming pancytopaenic with worsening liver enzymes (ALT increased to 262 IU/ L) and CRP increasing to 205 (mg/L). Bronchoscopy with transbronchial biopsies and washings were nondiagnostic. Due to a lack of a clear diagnosis, he was intubated and transferred to a tertiary care centre for a surgical lung biopsy which showed acute fibrinous and organising pneumonia, noting a prominent reactive interstitial lymphoid infiltrate ( Figure 2). Following this, he was given intravenous methylprednisolone for five days and started to improve clinically. This was followed by a reducing cou...

Elderly-onset adult Still’s disease

“…Rare cases of bilateral pulmonary nodules have been reported [56,57]. Parenchymal lung involvement was classified into 2 groups: predominant airway involvement (bronchiolitis and bronchitis) and predominant interstitial lung disease (nonspecific interstitial pneumonia, organizing pneumonia, or unclassifiable interstitial pneumonia) [55].…”

COVID‐19 related lung pathology: old patterns in new clothing?

“…21 First reported by Beasley et al in 2002, 22 it is an accepted histological pattern of interstitial pneumonia, but is viewed neither as an idiopathic clinicopathological entity nor a disease-specific histological pattern, as there are a variety of causes (drug, infections) and associations (connective tissue disorders, post-transplantation). [22][23][24][25][26][27][28][29][30][31][32][33] It is also not infrequent to see areas of OP, AFOP and DAD in the same autopsy from a patient dying of acute lung injury, and computerised tomography data describe some patients with COVID-19 who have disproportionate consolidation prior to, or alongside, classic DAD, 34,35 which may reflect a cohort with OP/AFOP as a sequelae of immune dysregulation, as seen in anti-synthetase syndrome. 36 Therefore, the presence of AFOP may reflect the final common pathway of DAD, systemic virally induced immune dysregulation, 37 secondary infection or any combination (Figure 1).…”