Disease characteristics, pathogenesis, and treatment controversies of axial psoriatic arthritis.

Axial psoriatic arthritis (axPsA) has considerable overlap with axial spondyloarthritis (axSpA) but has some unique features that sometimes preclude classification into axSpA. It has some clinical and radiographic differences compared to axSpA. Imaging typically shows asymmetric syndesmophytes, mainly in the cervical spine, with less frequent sacroiliitis. It more commonly presents later in life and is associated with less severe inflammatory back pain than axSpA. The interleukin (IL) IL-23/IL-17 axis is central to the pathogenesis of both diseases. However, the response to therapies targeting these cytokines has been different. IL-23 inhibitors are ineffective in axSpA but may be effective in psoriatic arthritis (PsA). Recent post hoc analyses of clinical trial data with IL-23 inhibitors in PsA have raised the possibility of their efficacy in axPsA and need evaluation in future clinical trials. Moreover, there is a need for classification criteria for axPsA and better tools to assess therapeutic response.

Red Cell Distribution Width and Absolute Lymphocyte Count Associate With Biomarkers of Inflammation and Subsequent Mortality in Rheumatoid Arthritis.

OBJECTIVE: Morbidity and mortality in rheumatoid arthritis (RA) is partly mitigated by maintaining immune and hematologic homeostasis. Identification of those at risk is challenging. Red cell distribution width (RDW) and absolute lymphocyte count (ALC) associate with cardiovascular disease (CVD) and mortality in the general population, and with disease activity in RA. How these variables relate to inflammation and mortality in RA was investigated. METHODS: In a retrospective single Veterans Affairs (VA) Rheumatology Clinic cohort of 327 patients with RA treated with methotrexate (MTX)+/- a tumor necrosis factor (TNF) inhibitor (TNFi), we evaluated RDW and ALC before and during therapy and in relation to subsequent mortality. Findings were validated in a national VA cohort (n = 13,914). In a subset of patients and controls, we evaluated inflammatory markers. RESULTS: In the local cohort, high RDW and low ALC prior to MTX treatment was associated with subsequent mortality over 10 years (both P < 0.001). The highest mortality was observed in those with both high RDW and low ALC. This remained after adjusting for age and comorbidities and was validated in the national RA cohort. In the immunology cohort, soluble and cellular inflammatory markers were higher in patients with RA than in controls. ALC correlated with age, plasma TNF receptor II, natural killer HLA-DR mean fluorescence intensity, and CD4CM/CD8CM HLA-DR/CD38%, whereas RDW associated with age and ALC. MTX initiation was followed by an increase in RDW and a decrease in ALC. TNFi therapy added to MTX resulted in an increase in ALC. CONCLUSION: RDW and ALC before disease-modifying antirheumatic drug therapy are associated with biomarkers of monocyte/macrophage inflammation and subsequent mortality. The mechanistic linkage between TNF signaling and lymphopenia found here warrants further investigation.

Cardiac Tamponade as Initial Presentation of Systemic Lupus Erythematosus in Third-Trimester Pregnancy.

BACKGROUND Systemic lupus erythematosus (SLE) is a common autoimmune disorder in women of childbearing age. It can present during pregnancy and may lead to poor maternal and fetal outcomes with a higher risk of preterm birth and pre-eclampsia. Women are at a higher risk of lupus flares during pregnancy, especially if undiagnosed or disease is poorly controlled. Cardiac tamponade is a rare complication of SLE and can be fatal. CASE REPORT A 21-year-old primigravida African-American female with a history of asthma presented with progressive pleuritic left shoulder pain. She had a recent history of sore throat, facial rash, and depressed mood after sun exposure. Work-up was strongly positive for antinuclear antigen, anti-smith, anti-smith/ribonucleoprotein, anti-chromatin, anti-SSA, anti-SSB, anti-dsDNA, and low C3. Echocardiogram showed hemodynamically stable cardiac tamponade. Patient also had proteinuria and hypertension attributed to pre-eclampsia. However, a renal biopsy confirmed lupus nephritis. The patient was treated with pericardiocentesis, prednisone, azathioprine, and hydroxychloroquine. There was significant clinical improvement with resolution of cardiac tamponade and improvement in renal function. CONCLUSIONS Cardiac tamponade is a rare and life-threatening manifestation of SLE. Prompt work-up and treatment with immunosuppressants and pericardiocentesis is needed to improve maternal and fetal outcomes. SLE patients are at a higher likelihood of exacerbations of the disease during pregnancy. It also important to rule out lupus nephritis in an SLE patient with pre-eclampsia. This report has shown the importance of accurate diagnosis of SLE in pregnancy and the appropriate management to ensure the best outcomes for the mother and fetus.

A Rare and Fatal Case of Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis.

BACKGROUND Sarcoidosis is a systemic inflammatory disorder characterized by a classic pathologic feature of non-caseating granulomas involving any organ system. Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic cytokine surge characterized by dysregulation of the macrophage response, which can be rapidly fatal. Recognition of HLH has been increasing over the past decade. HLH can present with features of sepsis that can make the diagnosis challenging and requires high clinical suspicion. CASE REPORT We report a case of a 48-year-old African American male with a past medical history of sarcoidosis infiltrating the lymph nodes, liver, and bone marrow with initial presentation of abdominal pain, nausea, vomiting, and weight loss of 100 pounds over 8 months. Sepsis was suspected, but the patient clinically deteriorated with vancomycin and cefepime. Fevers, bone marrow biopsy, anemia, thrombocytopenia, elevated ferritin, and elevated soluble receptor interleukin 2 confirmed HLH. The patient was treated with etoposide and dexamethasone with poor response and died from cardiac arrest. CONCLUSIONS Sarcoidosis associated with HLH is an extremely rare phenomenon with only 10 cases reported in the literature. Early clinical suspicion can be challenging as this condition is a sepsis-mimicker. To reduce mortality, prompt initiation of therapy is a key determinant in patients who are clinically deteriorating despite treatment for sepsis.

Lupus-Induced Vasculitis and Multiple Organ Dysfunction Syndrome as the First Presentation of Systemic Lupus Erythematosus (SLE) in Pregnancy.

BACKGROUND Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production leading to inflammation in multiple organs; it commonly affects young women in their child-bearing years. Clinical manifestations are diverse and range from mild arthritis to diffuse alveolar hemorrhage (DAH). DAH is a rare and devastating complication of SLE that carries a mortality rate of up to 50%, despite aggressive therapy. CASE REPORT A 21-year-old primigravida at 16 weeks gestation presents with a productive cough, rash, sore throat, and high-grade fever. Chest x-ray suggested multifocal pneumonia. Patient deteriorated despite antibiotics and intravenous (IV) fluids. She developed worsening anemia, leukopenia, and thrombocytopenia. Autoimmune workup was positive for Coombs, antinuclear antibody, anti-smith antibody, and hypocomplementemia. Skin biopsy was consistent with SLE. SLE vasculitis was suspected. She required mechanical intubation for rapid respiratory deterioration, with CT thorax suggesting ARDS. Bronchoscopy was done and confirmed DAH. Her course was further complicated with retinopathy and acute pancreatitis associated with SLE. She was treated with IV steroids, IV cyclophosphamide, and plasmapheresis, with significant clinical improvement and successful extubation. She delivered a healthy baby at 32 weeks gestation. CONCLUSIONS Early recognition and initiation of treatment is critical to survival in DAH and requires a high index of clinical suspicion. Treatment includes high-dose steroids, cyclophosphamide, and plasma exchange. Pregnancy increases the risk of adverse outcome in SLE. Seven cases of DAH in pregnant patients with SLE have been reported. Here, we report a catastrophic presentation of DAH, acute pancreatitis, and retinopathy in a pregnant patient with newly diagnosed SLE.

A Rare Case of Diffuse Large B Cell Lymphoma Presenting as a Cardiac Mass.

BACKGROUND Primary mediastinal diffuse large B cell lymphoma (DLBCL) presenting as a large intracardiac tumor is extremely rare and has not been significantly reported in the literature. Cardiac lymphoma consists of 2 subtypes: mediastinal DLBCL invading the heart and primary cardiac lymphoma. Both subtypes have a poor prognosis and are treated similarly. Mediastinal DLBCL is a life-threatening condition that, if diagnosed early, has a better survival rate. This is a rare case of a mediastinal DLBCL invading the right atrium as a large intracardiac mass, causing partial obstruction of the tricuspid valve without hemodynamic compromise. CASE REPORT A 57-year-old female presented with unintentional weight loss, fatigue, exertional dyspnea, and cough for 8 weeks. Transesophageal echocardiogram showed a mass (3.5×3.5 cm) in the posterior wall of the right atrium partially obstructing the tricuspid valve. Biopsy revealed DLBCL. Given new-onset lymphoma, a human immunodeficiency virus (HIV) test was done and came back positive. CD4 count was 100 cells/mm³. Chemotherapy was initiated with rituximab, cyclophosphamide, epirubicin, vincristine, and prednisone (R-CHOP). Highly active anti-retroviral (HAART) therapy was started for HIV. After treatment with R-CHOP and HAART, the patient had complete resolution of the mass and symptoms on follow-up imaging and evaluation at 6 months. CONCLUSIONS Mediastinal DLBCL invading the heart is a life-threatening form of non-Hodgkin's lymphoma (NHL) and early diagnosis and treatment is critical as prognosis is poor especially if diagnosed in later stages of the disease. Testing for HIV is important as 5% of HIV patients are susceptible to developing NHL.