Inflammatory pulmonary nodules in Kawasaki disease

Abstract

Symptomatic pulmonary manifestations of Kawasaki disease (KD) are uncommon. However, epidemiologic, radiologic, and histologic studies have indicated that respiratory symptoms and findings occur in KD and suggest that the KD agent may have a respiratory portal of entry. We report on three young infants with KD who developed pulmonary nodules, in addition to coronary artery aneurysms. Two patients had pathologic specimens available, one from biopsy and the other from autopsy. The nodules had predominantly mononuclear cell infiltrates, which were within the lung parenchyma and infiltrating vessel walls. Immunohistochemical studies of the nodules, using antibodies to common leukocyte antigen (LCA) and factor VIII-related antigen, confirmed the inflammatory nature of the lesions and showed capillary proliferation. IgA plasma-cell infiltration was observed in the nodule, consistent with previous KD findings of IgA plasma-cell infiltration in the vessel walls, kidneys, pancreas, and upper respiratory tract. The two patients with nonfatal KD were treated with intravenous immunoglobulin and aspirin, with resolution of the nodules. We propose that when pulmonary involvement occurs in KD, it ranges from subclinical interstitial micronodular infiltrates to larger inflammatory pulmonary nodules. These pulmonary infiltrates and nodules likely reflect the host response to the etiologic agent of KD, and may resolve with the disease process. Recognition of this pulmonary complication of KD may enable cautious observation of such lesions for spontaneous resolution.

Hoarseness as a Presenting Sign in Children With Kawasaki Disease

“…Second, case reports have documented uvulitis, supraglottitis, and pulmonary nodules associated with acute KD. (1, 10, 11) Finally, an oligoclonal IgA immune response suggests entrance of the pathogen through the mucosal surfaces of the oropharynx and upper airway and virus-like inclusion bodies in respiratory epithelial cells may be related to the causative agent. (12, 13) It is also plausible that KD may cause a transient recurrent laryngeal nerve paresis resulting in weakness of one of the vocal folds and associated hoarseness in some patients.…”

“…These include presentation with enlarged anterior cervical lymph nodes that drain the posterior pharynx in 30% of patients, retropharyngeal edema imaged by computed tomography, and occasional reports of pulmonary nodules during the acute phase of the illness (1–4). We noted that many of our patients were hoarse at the time of presentation, although this finding had not been previously reported in the literature.…”

Advances in Kawasaki disease

“…Although the likely infectious trigger of the marked inflammatory immune response remains unknown, the pathologic findings have been well-described and are virtually identical in all patients with the illness, varying only in the severity of the lesions. In addition to arteries and veins [3], the following organ systems typically demonstrate inflammatory lesions: cardiovascular system (myocarditis, pericarditis, and less commonly endocarditis); respiratory system (bronchitis, interstitial pneumonia, and pulmonary nodules); digestive system (stomatitis, sialoduct-adenitis, enteritis, hepatitis, cholangitis, pancreatitis, and pancreatic ductitis); urinary system (focal interstitial nephritis, cystitis, and prostatitis); nervous system (aseptic meningitis and neuritis); and hematopoetic system (lymphadenitis and splenitis) [4,16]. In the 1st month after onset of illness, the inflammatory lesions are quite severe.…”

Medium-size-vessel vasculitis

“…Vascular damage to the major limb arteries, renal and other visceral vessels and the aorta can be demonstrated [129, 130]. Other systemic involvement can occur, including in the gastrointestinal tract [131], the hepatobiliary tract with hydrops of the gall bladder being well recognised [132], the respiratory tract [133], the central nervous system with seizures and meningeal features [134], deafness [135], arthropathy [136] and renal involvement. Renal manifestations include pyuria, proteinuria, tubular disturbances, tubulointerstitial nephritis and renal failure [137–147].…”