A Case of Adult Onset Still's Disease Complicated with Cryptogenic Organizing Pneumonia

Abstract

Only a few pathologic reports exist describing adult onset Still's disease (AOSD) with pulmonary involvement. We report this very rare case of AOSD complicated with cryptogenic organizing pneumonia (COP). A 32-year-old woman was referred with high spiking fever, salmon-pink rash in her arms and legs, and polyarthralgia. The laboratory data showed marked increases in white blood cell count, an erythrocyte sedimentation rate, and C reactive protein, ferritin, and liver dysfunction. All cultures remained negative, as were autoantibodies and rheumatoid factor. The patient was strongly suspected of AOSD according to specific diagnostic criteria. However, chest X ray disclosed an infiltrative shadow accompanied by air bronchogram in the upper lobe of the right lung and therapy with antibiotics was initiated. As the patient did not respond to antibiotics and a remittent fever of over 38 , a flexible bronchoscopy was performed. Organizing pneumonia was diagnosed by transbronchial lung biopsy (TBLB) histology and radiologically, and the lesions were thought to be due to pulmonary involvement of AOSD. Therefore, she was diagnosed with AOSD complicated with COP. Oral treatment with prednisolone (30 mg/day) resulted in rapid disappearance of the infiltrative shadow. Symptoms and markers of inflammation also improved. Clinicians should be aware that COP can be a complication of AOSD.

Parenchymal lung disease in adult onset Still’s disease: an emergent marker of disease severity—characterisation and predictive factors from Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort of patients

“…In this cohort, 12.25% of patients with AOSD were reported to be affected by LD. This result could suggest a higher rate than reported in available literature [16], but it must be pointed out that mainly isolated case reports investigated this issue [14][15][16], thus limiting the validity of this estimation in adults. Clinically, as observed in SJIA, patients with AOSD-related LD showed a striking dissociation between relatively subtle clinical features, including mild tachypnoea, dyspnoea, chronic cough, and the extension of the inflammatory process in lungs based on chest CT.…”

“…Interestingly, recent evidence from the juvenile counterpart of AOSD, the systemic-onset juvenile idiopathic arthritis (SJIA), suggested the emergent high fatality rate of lung disease (LD) [12,13]. Despite this finding, few studies, mainly isolated case reports, analysed LD in AOSD so far [14][15][16], proposing that nearly 5% of patients with AOSD could be affected by LD, with two main patterns, one characterised by an acute respiratory distress syndrome (ARDS), and one with other LDs, including bronchiolitis and nonspecific interstitial lung diseases [16]. On these bases, given the prognostic role of LD in paediatric patients and the inconclusive evidence in adult ones, we aimed to characterise LD in AOSD and to identify associated clinical features and predictive factors.…”

Adult onset Still's disease accompanied by acute respiratory distress syndrome: A case report

“…To the best of our knowledge, the association between lung diseases and AOSD has been reported numerous times (5,15,16), and pleuritis associated with AOSD has been widely reported, ranging between 12 and 53% in various studies (17,18). Pleuritis is usually followed by pleural effusion (12).…”

Adult onset still's disease presenting as recurrent fever with ARDS: A case report

“…While recurrent febrile episodes with arthralgia and rash is the typical presentation, less common AOSD manifestations include pericarditis, valvular abnormalities, thrombotic thrombocytopenic purpura, interstitial nephritis and neurological manifestations like cranial nerve palsies, seizures and aseptic meningoencephalitis. Pulmonary manifestations are infrequent and include pleurisy, cryptogenic organizing pneumonia [4], diffuse alveolar hemorrhage [5] and ARDS. Rare instances of ARDS have been described in literature in relation to AOSD [6][7][8] as well as treatment of AOSD with anakinra [9].…”