Adult-Onset Still's Disease Misdiagnosed as Acute Fibrinous and Organizing Pneumonia: A Case Report and Literature Review

Adult-onset Stil's disease(AOSD)is a rare condition that lies between autoinflammatory syndrome and autoimmune disease.The main clinical manifestations include fever,chills,rash,joint swelling and pain,peripheral blood leukocytosis,splenomegaly,etc.It is a systemic disease affecting between 1 and 34 people per million.The average age of onset is 35 years old,with a slightly higher prevalence rate in women.Since AOSD lacks early specific symptoms and signs,non-specialist doctors have limited understanding of the disease,and patients are prone to clinical misdiagnosis,mistreatment,and delayed disease progression.This paper reports a patient whose AOSD was misdiagnosed as acute fibrinous and organizing pneumonia.

Two methods of lung biopsy for histological confirmation of acute fibrinous and organizing pneumonia:A case report

BACKGROUND Acute fibrinous and organizing pneumonia(AFOP)is a rare,noninfective lung disease,histologically characterized by a patchy distribution of intra-alveolar fibrin“balls”and organizing pneumonia.The clinical manifestations of AFOP are nonspecific.Diagnosis depends on pathology.Surgical lung biopsy is optimal for tissue sampling to diagnose AFOP.However,many patients have no tolerance to the operation,including mentally and physically.There is still no standard therapy for AFOP and the methods remain controversial.Therefore,further clinical attention and discussion are warranted.CASE SUMMARY A 53-year-old woman presented with fever,cough and dyspnea for 15 d.Antiinfective therapy was ineffective.Chest computed tomography showed bilateral patchy consolidation,especially in the lower lobes.We performed both ultrasound-guided transbronchial lung biopsy and ultrasound-guided percutaneous fine needle puncture at different lung lesion locations.Both samples supported the diagnosis of AFOP.The patient had a good clinical course after treatment with methylprednisolone,and no side effects of steroids.CONCLUSION Percutaneous needle biopsy combined with transbronchial lung biopsies may be a good choice in the absence of surgical biopsy.Methylprednisolone alone is effective in the treatment of idiopathic AFOP.

Clinical and Radiological Profile of Acute Fibrinous and Organizing Pneumonia： A Retrospective Study

Background： Acute fibrinous and organizing pneumonia （AFOP） is a unique pathological entity with intra-alveolar fibrin in the form of ＂fibrin balls＂ and organizing pneumonia. It was divided into rare idiopathic interstitial pneumonia according to the classification notified by American Thoracic Society/European Respiratory Society in 2013. As a rare pathological entity, it is still not well known and recognized by clinicians. We reviewed the clinical features of 20 patients with AFOP diagnosed in a teaching hospital. Methods： The medical records of 20 patients with biopsy-proven diagnosis of AFOP were retrospectively reviewed. The patients＇ symptoms, duration of the disease, comorbidities, clinical laboratory data, pulmonary function testing, radiographic studies, and the response to treatment were extracted and analyzed. Results： Fever was the most common symptom and was manifested in 90% of AFOP patients. For clinical laboratory findings, systematic inflammatory indicators, including C-reactive protein and erythrocyte sedimentation rate, were significantly higher than normal ill AFOP patients. In accordance with this increased indicators, injured liver functions were common in AFOP patients. Inversely, AFOP patients had worse clinical conditions including anemia and hypoalbuminemia. For pulmonary function testing, AFOP patients showed the pattern of restrictive mixed with obstructive ventilation dysfunction. For high-resolution computerized tomography （HRCT） findings, the most common pattern for AFOP patients was lobar consolidation which was very similar to pneumonia. However, unlike pneumonia, AFOP patients responded well to glucocorticoids. Conclusion： Patients with AFOP manifest as acute inflammatory-like clinical laboratory parameters and lobar consolidation on HRCT, but respond well to steroid.