Fatal acute fibrinous and organizing pneumonia in a child with juvenile dermatomyositis

Airway involvement in interstitial lung disease

“…The same is also the case in a more recent study [18] describing AFOP in the context of an inflammatory myopathy. In this publication, Prahalad et al [18] present a clinical case of a 14-year-old girl with juvenile dermatomyositis who developed extensive pulmonary parenchymal injury with a patchy pattern, prevailing in both lower lobes.…”

“…In this publication, Prahalad et al [18] present a clinical case of a 14-year-old girl with juvenile dermatomyositis who developed extensive pulmonary parenchymal injury with a patchy pattern, prevailing in both lower lobes. The patient was admitted to hospital but died 2 weeks later, despite various therapeutic measures undertaken, including intravenous administration of immunoglobulin, methylprednisolone, azithromycin, cyclosporine, cyclophosphamide and, finally, mechanical ventilation.…”

Cryptogenic organising pneumonia

“…This pathological pattern has also been reported upon autopsy of patients with severe acute respiratory syndrome [410] and in dermatomyositis [411]. The presence of fibrin in the organising lesions at lung biopsy of patients with COP has been associated with less complete recovery under corticosteroids [412].…”

Distinct histopathology of acute onset or abrupt exacerbation of hypersensitivity pneumonitis

“…AFOP has been described as a pattern of fibrin deposition associated with collagen-vascular diseases (systemic lupus erythematosus, polymyositis, ankylosing spondylitis, fibromyalgia), drug reaction (amiodarone and abacavir), infection (Haemophilus influenzae and Acinetobacter), and lymphoma [19][20][21][22]. AFOP has not been previously described in HSP.…”