Treatment of hypertrophic osteoarthropathy with zoledronic acid: case report and review of the literature.

OBJECTIVES: Zoledronic acid (ZA) is rarely used to manage hypertrophic osteoarthropathy (HOA). We report our experience with ZA treatment of a patient with HOA and sarcoidosis who had also undergone lung transplant. We also conducted a literature review of the usefulness of bisphosphonates in HOA. METHODS: We performed a PubMed literature search using keywords HOA, periostitis, bisphosphonate, ZA, sarcoidosis, and lung transplant. A PRISMA flow diagram is presented to depict the data collection process, and a case is reported. RESULTS: A 62-year-old woman with bilateral lung transplant as a result of severe pulmonary sarcoidosis developed severe limb pain and inflammatory polyarthritis. HOA was diagnosed in the presence of periostitis with the symptoms. Failure of the refractory bone and joint pain to respond to low doses of prednisone, tramadol, or even pamidronate infusion prompted a trial of a single dose of intravenous ZA. Surprisingly, the pain completely resolved without recurrence. A total of 12 cases of HOA treated with bisphosphonates were retrieved from the literature and reviewed. CONCLUSIONS: Bisphosphonates are generally effective therapy for HOA-related pain. ZA may be even more efficacious and longer lasting than pamidronate for management of the bone and joint pain associated with HOA irrespective of the underlying disorders.

Macrophage activation syndrome: why and what should a gastroenterologist know.

We recently treated a patient with adult-onset Still's disease who developed macrophage activation syndrome (MAS) secondary to disseminated histoplasmosis while being treated with adalimumab. The gastroenterology service was consulted early, before diagnosis, as the patient presented with elevated liver enzymes and disseminated intravascular coagulation. MAS is an exaggerated immune response that can develop as a primary condition or secondary to infections, drugs and various diseases, resulting in liver dysfunction, encephalopathy, pancytopenia and disseminated intravascular coagulation. The development of MAS has also been reported in patients with inflammatory bowel disease and post-liver transplantation and has been triggered by medications used by gastroenterologists, particularly sulfasalazine and anti-tumor necrosis factor biologic modifiers. Therefore, we present a review on etiology, pathogenesis, clinical and laboratory features, and treatment of MAS with a focus on gastrointestinal aspects and presentations. MAS is a life threatening condition with a high mortality rate if untreated. Therefore it is important to recognize this condition early. As these patients may occasionally present to gastroenterologists we hope this review will increase awareness of this rare, but serious syndrome.