Inflammatory arthritis, such as pseudogout or otherwise referred to as calcium pyrophosphate (CPP) crystal arthritis or calcium pyrophosphate deposition (CPPD) disease, is characterized by the deposition of crystal formation and deposition in large joints. CPPD is known to affect the elderly population and commonly manifests as inflammation of knees, hips, and shoulders. CPPD disease involving the spine has been infrequently encountered in practice and rarely described in the literature. Here, we describe a case of an 80-year-old female with no known history of inflammatory arthritis who presented with left lower extremity weakness and fall, initially thought to have discitis, later confirming CPPD of the spine through biopsy and ultimately resolution of symptoms with anti-inflammatory agents. Although consisting of different clinical presentations, two other case reports have described CPPD of the spine with similar radiographic findings, to this author’s knowledge. With the radiologic similarities, this unique case serves to raise awareness in the medical community and possibly place pseudogout of the spine on the differential list when such cases are encountered. As a result, patients can be initiated on benign anti-inflammatory agents, avoiding invasive testing and unnecessary antibiotic exposure.
Here, we present a 22-year-old female patient with adult-onset Still's disease (AOSD) who was newly diagnosed in the setting of secondary macrophage activation syndrome (MAS), a rare, life-threatening inflammatory disease with 50% mortality due to multi-organ failure. She met the diagnostic criteria of AOSD and MAS, while genetic testing excluded primary causes of MAS. She had high fevers, anemia, thrombocytopenia, splenomegaly, hematophagocytosis, and elevated serum ferritin (37,950 ng/mL) and CD25 levels (11,870 pg/mL), which remained unresponsive to corticosteroids and anakinra. Her serum interferon gamma (IFN-γ) levels were elevated (7 pg/mL). She was markedly responsive to IFN-γ blockade with emapalumab that eliminated her fevers and all MAS-associated laboratory abnormalities. This report provides initial evidence for therapeutic efficacy for IFN-γ blockade in AOSD and secondary MAS.
Drug-induced thrombotic microangiopathy (DITMA) is a secondary cause of thrombotic microangiopathy and a potentially fatal inflammatory disease. DITMA has been attributed to a variety of drugs, particularly chemotherapeutic and immunosuppressive agents. Prompt diagnosis is critical for survival and treatment necessitates withdrawal of the offending drug; however, many cases require further treatment including plasmapheresis, immunosuppression, and anticoagulation. In this article, we report a cutaneous biopsy-proven case of tacrolimus-induced DITMA, which was successfully treated with eculizumab after failing the conventional standard of care.
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