ABSTRACT
Adult onset Still's disease (AOSD) is an autoimmune disease characterized by systemic inflammation and is a rarely reported cause of pulmonary arterial hypertension (PAH). We describe the clinical course of a 40-year-old woman who presented with PAH 19 months after a diagnosis of AOSD. Sildenafil and immunosuppressive therapy with cyclosporine resulted in clinical and hemodynamic improvement with long-term survival 15 years after her initial presentation of AOSD. We review the literature for published cases of PAH due to AOSD and discuss the potential mechanisms relating inflammatory diseases and PAH.
Subject(s)
Benzimidazoles/therapeutic use , Multiple Myeloma/drug therapy , Multiple Myeloma/pathology , Protein Kinase Inhibitors/therapeutic use , Urea/analogs & derivatives , Aurora Kinases/antagonists & inhibitors , Benzimidazoles/administration & dosage , Benzimidazoles/adverse effects , Drug Resistance, Neoplasm , Humans , Protein Kinase Inhibitors/administration & dosage , Protein Kinase Inhibitors/adverse effects , Recurrence , Treatment Failure , Treatment Outcome , Urea/administration & dosage , Urea/adverse effects , Urea/therapeutic useSubject(s)
Appendicitis/diagnosis , Leukemia, Myeloid, Acute/complications , Lymphoma, Large B-Cell, Diffuse/complications , Acute Disease , Adult , Amphotericin B/therapeutic use , Antifungal Agents/therapeutic use , Appendicitis/microbiology , Candidiasis/diagnosis , Candidiasis/drug therapy , Female , Fluconazole/therapeutic use , Humans , Liver Diseases/microbiology , Middle Aged , Splenic Diseases/microbiologyABSTRACT
The esophagus and stomach can be primary sites for Hodgkin lymphoma (HL). The pathognomonic feature of HL is the Reed-Sternberg cell. Because these cells can be rare in HL tumours, biopsies obtained via endoscopy are usually inadequate for establishing a definitive diagnosis. A case of a gastroesophageal junction primary HL is presented that was diagnosed on endoscopic biopsy material with the assistance of the immunohistochemical stains PAX5 and MUM1 which verified the presence of Reed-Sternberg cells. The patient was effectively treated for HL and avoided traditional laparotomy or thoracotomy procedures to obtain the diagnosis. This advanced immunohistochemical approach should be the standard in the diagnosis of primary gastrointestinal HL. An endoscopy-based approach should obviate more invasive, open biopsy procedures for establishing HL diagnosis.
