Adult-onset Still's disease complicated by macrophage activation syndrome during pregnancy: a case-based review.

Adult-onset Still's disease is a rare, systemic inflammatory rheumatic disease characterized by recurrent fevers, arthritis, and an evanescent rash. One of the most serious hematologic derangements that can be seen with adult-onset Still's disease is macrophage activation syndrome. Macrophage activation syndrome is characterized by activation of lymphocytes, resulting in a cytokine storm and hemophagocytosis in the bone marrow, along with multi-organ failure. Adult-onset Still's disease with macrophage activation syndrome first presenting during pregnancy is exceptionally rare; here, we report two unique cases of such a presentation and review the pertinent literature. Both of our cases presented critically ill with end-organ failure, and responded to immunosuppression; fetal demise was present in one and an emergency caesarean section with a viable fetus was performed in the other patient. Maternal outcomes were favorable in both cases and both patients did well long-term with systemic therapy. Systemic immunosuppression, particularly anti-IL1 therapy, may be considered as treatment for this rare and life-threatening condition when presenting during pregnancy.

Integrating primary palliative care into hidradenitis suppurativa management.

Hidradenitis suppurativa (HS) is a chronic, often debilitating skin condition that disproportionately impacts women in the United States and other Western nations. Dermatologists should consider incorporating palliative care principles into HS management to optimize care. Primary palliative care principles include utilizing evidence-based frameworks in serious illness communication, acknowledging and addressing physical and psychosocial suffering, recognizing and validating the burden of disease in partners, families, and caregivers, and engaging in collaborative care coordination. Certain patients may also benefit from outpatient, or sometimes inpatient, palliative care specialist collaboration, such as those with refractory HS and superimposed challenging psychosocial dynamics and symptom burden. Through integration of these palliative care domains into HS care, dermatologists can optimize their ability to provide comprehensive and compassionate care for patients suffering with this disease.

Calcium pyrophosphate crystal size and characteristics.

OBJECTIVE: To describe the characteristics of calcium pyrophosphate (CPP) crystal size and morphology under compensated polarized light microscopy (CPLM). Secondarily, to describe CPP crystals seen only with digital enhancement of CPLM images, confirmed with advanced imaging techniques. METHODS: Clinical lab-identified CPP-positive synovial fluid samples were collected from 16 joint aspirates. Four raters used a standardized protocol to describe crystal shape, birefringence strength and color. A crystal expert confirmed CPLM-visualized crystal identification. For crystal measurement, a high-pass linear light filter was used to enhance resolution and line discrimination of digital images. This process identified additional enhanced crystals not seen by raters under CPLM. Single-shot computational polarized light microscopy (SCPLM) provided further confirmation of the enhanced crystals' presence. RESULTS: Of 932 suspected crystals identified by CPLM, 569 met our inclusion criteria, and 293 (51%) were confirmed as CPP crystals. Of 175 unique confirmed crystals, 118 (67%) were rods (median area 3.6 µm2 [range, 1.0-22.9 µm2]), and 57 (33%) were rhomboids (median area 4.8 µm2 [range, 0.9-16.7 µm2]). Crystals visualized only after digital image enhancement were smaller and less birefringent than CPLM-identified crystals. CONCLUSIONS: CPP crystals that are smaller and weakly birefringent are more difficult to identify. There is likely a population of smaller, less birefringent CPP crystals that routinely goes undetected by CPLM. Describing the characteristics of poorly visible crystals may be of use for future development of novel crystal identification methods.

Diagnostic advances in synovial fluid analysis and radiographic identification for crystalline arthritis.

PURPOSE OF REVIEW: The present review addresses diagnostic methods for crystalline arthritis including synovial fluid analysis, ultrasound, and dual energy CT scan (DECT). RECENT FINDINGS: There are new technologies on the horizon to improve the ease, sensitivity, and specificity of synovial fluid analysis. Raman spectroscopy uses the spectral signature that results from a material's unique energy absorption and scatter for crystal identification. Lens-free microscopy directly images synovial fluid aspirate on to a complementary metal-oxide semiconductor chip, providing a high-resolution, wide field of view (∼20 mm) image. Raman spectroscopy and lens-free microscopy may provide additional benefit over compensated polarized light microscopy synovial fluid analysis by quantifying crystal density in synovial fluid samples. Ultrasound and DECT have good sensitivity and specificity for the identification of monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals. However, both have limitations in patients with recent onset gout and low urate burdens. SUMMARY: New technologies promise improved methods for detection of MSU and CPP crystals. At this time, limitations of these technologies do not replace the need for synovial fluid aspiration for confirmation of crystal detection. None of these technologies address the often concomitant indication to rule out infectious arthritis.

Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy.

Adult T-cell leukemia/lymphoma (ATLL) is a rare and aggressive disease caused by human T-cell lymphotropic virus type 1 that predominantly affects Japanese and Caribbean populations. Most studies have focused on Japanese cohorts. We conducted a retrospective analysis of 53 cases of ATLL who presented to our institution between 2003-2014. ATLL in the Caribbean population presents more often as the acute and lymphomatous subtypes, is associated with complex cytogenetics, and has a high rate of CNS involvement. The overall response rate to first-line therapies with anthracycline-based regimens was poor (32%), with a median survival of only 6.9 months. A complete or partial response to first-line regimens was associated with better survival. There was no difference in survival between patients who received chemotherapy alone versus chemotherapy with antiviral agents. Allogeneic transplantation was performed in five patients, two of whom achieved complete remission despite residual or refractory disease. Recipients of allogeneic transplantation had significantly improved overall survival compared to non-transplanted patients. This is the first analysis to describe ATLL pathological features, cytogenetics, and response to standard therapy and transplantation in the Caribbean cohort.