Critical digital ischemia and biliary cholangitis related to graft versus host disease: A case report and systematic literature review.

RATIONALE: Chronic graft versus host disease (cGVHD) is a systemic immune-mediated complication that occurs in approximately half of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HCT), and remains the leading cause of late morbidity and mortality. cGVHD involves a heterogeneous group of organic manifestations, many of which mimic autoimmune diseases such as scleroderma, primary biliary cholangitis, Sjögren syndrome and polymyositis. PATIENT CONCERNS: A 60-years-old female with a history of allo-HCT developed de novo cGVHD 11 months after allo-HCT with isolated liver involvement. The patient presented with jaundice, cytolysis, cholestasis and concomitant acute digital ischemia. Liver biopsy and autoimmunity tests were performed and were found to be compatible with immune-mediated liver damage. Nailfold capillaroscopy revealed microangiopathy, characterized by avascular areas and some enlarged capillaries resembled an early systemic sclerosis pattern. DIAGNOSIS: Biliary cholangitis-like and digital ischemia related to cGVHD. INTERVENTIONS: The patient was treated with high-dose prednisone and ursodeoxycholic acid, and extracorporeal photopheresis. The patient required hospital admission for administration of intravenous prostacyclin due to refractory Raynaud syndrome. OUTCOMES: After 6 to 8 weeks, the patient achieved a good response, with evident clinical improvement and progressive normalization of liver function. LESSONS: cGVHD is a multiorgan pathological condition, and this case emphasizes that a multidisciplinary team, including rheumatologists, should be involved in the follow-up of allo-transplant patients to ensure that the clinical complications are adequately addressed. Early intervention is critical for improving patient' prognosis.In addition, we performed a systemic literature review based on published case articles on hepatic cGVHD and digital ischemia published up to August 2022. To the best of our knowledge, this is the first reported case of such an association.

Ocronosis y osteoporosis: a propósito de un caso / Ochronosis and osteoporosis: a case report

No disponible

Manifestaciones trombóticas en el síndrome SAPHO. Revisión de la literatura / Thrombotic manifestations in SAPHO syndrome. Review of the literature

El síndrome SAPHO (sinovitis, acné, pustulosis, hiperostosis y osteítis) comprende un conjunto de manifestaciones cutáneo-osteoarticulares. Se han descrito algunas complicaciones graves que pueden aparecer durante la evolución de la enfermedad, como la trombosis venosa, principalmente en pacientes que desarrollan afectación inflamatoria grave de la pared torácica anterior. El objetivo de la presente revisión fue analizar los casos descritos en la literatura médica relacionados con la presencia de complicaciones trombóticas en pacientes diagnosticados de síndrome SAPHO e intentar establecer los probables factores de riesgo y su posible mecanismo patogénico. Se analizaron 11 artículos publicados de casos clínicos aislados o series de casos, con un total de 144 pacientes, que describen en total 15 casos de trombosis venosa. Se exponen las características clínicas de estos pacientes, se evalúa si cumplen los criterios de clasificación ASAS para espondiloatritis axial y periférica, y se resalta la necesidad de realizar un diagnóstico y tratamiento precoces (AU)

SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted (AU)

Thrombotic manifestations in SAPHO syndrome. Review of the literature.

SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior-chest-wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, is analyzed the need for early diagnosis and treatment is highlighted.