A Case of Dermatomyositis Causing Cryptogenic Organizing Pneumonia

Abstract

Cryptogenic organizing pneumonia (COP), also known as idiopathic bronchiolitis obliterans organizing pneumonia (BOOP), is a rare inflammatory condition. It often presents as sequelae of existing chronic inflammatory diseases such as rheumatoid arthritis, systemic lupus erythematosus, and various connective tissue conditions. This case describes a 28-year-old African American female who presented with a complex clinical picture involving chronic inflammatory processes and the pulmonary system. The initial evaluation suggested pneumonia to be the underlying cause of respiratory symptoms; however, ultimately, a diagnosis of BOOP with dermatomyositis was made.

A Case Report of Steroid-Resistant Cryptogenic Organizing Pneumonia Managed with Intravenous Immunoglobulins

“…Although mycophenolate mofetil was also started at the same time as IVIg, we believe IVIg to be responsible for his improvement given that the effects of antimetabolites such as mycophenolate mofetil are generally not seen for several weeks. To date, we found only seven published cases that used intravenous immunoglobulins (IVIg) for the treatment of organizing pneumonia, often in the context of underlying selective immunodeficiency or hypogammaglobulinemia and/or nonresponse to steroid [19][20][21][22][23][24][25]. These cases suggest that a nor-mal and functional humoral immune response is not an absolute requirement for the development of COP and also raise the possibility of an association of COP to hypogammaglobulinemia in the setting of repeated infections.…”

Multidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist’s Key Role

“…Organizing Pneumonia (OP) is currently recognized as a nonspecific lung injury pattern that has already been widely described under various names in the past [81][82][83][84]. OP can be secondary (Secondary Organizing Pneumonia, SOP), representing the histopathological manifestation of many clinical conditions such as CTD-ILD, drug reaction, HP, pulmonary infections and aspiration [85][86][87][88][89][90][91][92][93][94][95][96][97][98][99], or it may be not associated with a known cause or clinical condition, and appears to be idiopathic (Cryptogenic Organizing Pneumonia, COP) [27,100,101]. The histopathological hallmark of OP pattern is a patchy airspace filled by plugs of plump fibroblasts-myofibroblasts combined with variable numbers of admixed chronic inflammatory cells and embedded in a pale staining myxoid matrix of immature loose connective tissue with a polypoid shape, known as Masson bodies [22,101,102].…”