Pancreatitis, Panniculitis, and Polyarthritis Syndrome as the First Symptom of Necrotizing Pancreatitis.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a very rare cutaneous manifestation found in patients with acute pancreatitis. We report the case of a 44-year-old man presenting with erythematous, painful lesions on the lower extremities and ankle swelling. The rheumatology service was consulted for possible erythema nodosum. Extensive workup revealed elevated lipase and amylase levels, and computed tomography of the abdomen and pelvis revealed acute pancreatitis with necrotizing lesions and peripancreatic thoracic collections. There were also changes of chronic pancreatitis. The original skin manifestations were eventually identified as pancreatic panniculitis by skin biopsy. The patient was treated for pancreatitis and pleural effusions, and his skin and joint symptoms completely resolved. Pancreatic panniculitis with polyarthritis is rare but may be the first presenting symptom of pancreatic disease. Rheumatology may be consulted for these patients especially if there are only skin and joint manifestations and no abdominal pain. Misdiagnosis of pancreatitis can lead to poorer outcomes and delay in care. Therefore, pancreatic disease should be on the differential for any patient with panniculitis and polyarthritis.

A Review of Antisynthetase Syndrome-Associated Interstitial Lung Disease.

Our objective in this review article is to present a clinical case of a patient with antisynthetase syndrome (ASyS) and provide an overview of the pathogenesis, classification criteria, antibody profiles, clinical features, and current knowledge of treatment options, focusing on interstitial lung disease (ILD). ASyS is an uncommon autoimmune disease with a heterogenous clinical presentation characterized by the presence of autoantibodies against an aminoacyl-tRNA synthetase and manifested by myositis, fever, inflammatory arthritis, Raynaud's phenomenon, mechanics hands, and ILD. ASyS-associated ILD (ASyS-ILD) is the most serious complication of ASyS, which may evolve to rapidly progressive ILD; therefore, it often requires thorough clinical and radiologic evaluation including recognition of a specific clinical phenotype associated with the antisynthetase antibodies (ASAbs) to guide therapeutic interventions.

A Case of Cytomegalovirus-Induced Hemophagocytic Lymphohistiocytosis in a Patient with an Underlying Rheumatic Disease.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition caused by overproduction of inflammatory cytokines and overactivation of macrophages that can progress to multiorgan dysfunction and failure. Although there are guidelines that attempt to recognize the condition in its early stage, diagnosis can be very challenging due to heterogeneous presentations of HLH. Symptoms and clinical findings include fever, neurologic complaints, respiratory issues, liver dysfunction, cytopenias, amongst others most of which are not specific to HLH. In addition, response to treatment can be highly variable, necessitating an individualized treatment plan based on the presentation. We present a case of a 21-year-old female with a history of biopsy-proven inflammatory myositis on azathioprine and prednisone who presented with fever, hypotension, and pancytopenia. Additional imaging studies showed multiorgan involvement, including pneumonia, pyelonephritis, and splenomegaly. A bone marrow biopsy of her iliac crest showed hemophagocytosis and the infectious workup confirmed cytomegalovirus (CMV) infection, which led to the diagnosis of CMV-induced HLH. She was treated initially with anakinra for macrophage activation syndrome (MAS) in addition to dexamethasone and ganciclovir. Unfortunately, she did not respond to anakinra and was subsequently switched to etoposide with dexamethasone and valganciclovir, which subsequently helped our patient to recover clinically. Our case highlights the challenging nature of HLH and the importance of early detection and a personalized treatment plan in achieving optimal outcomes in patients with HLH.

Anti-Signal Recognition Particle Antibody-Associated Severe Interstitial Lung Disease Requiring Lung Transplantation.

Anti-signal recognition particle (SRP) antibodies are typically associated with immune-mediated necrotizing myopathy. Some patients with anti-SRP antibodies may have extramuscular manifestations including mild respiratory symptoms secondary to interstitial lung disease. We present a case of a 40-year-old female presenting with acute hypoxic respiratory failure secondary to anti-SRP antibody-associated interstitial lung disease with mildly elevated creatinine kinase but without evidence of necrotizing myopathy on muscle biopsy. After a complicated six-month hospitalization, the patient successfully received double lung transplantation and was eventually discharged on room air. Unexplained worsening interstitial infiltrates leading to persistent hypoxic respiratory failure in the setting of nonspecifically elevated creatinine kinase should warrant consideration of an underlying connective tissue disease, including myositis with anti-SRP antibody-associated interstitial lung disease. In rare cases, interstitial lung disease may be severe requiring lung transplantation.

Rheumatology Care Using Telemedicine.

Introduction: People living in many parts of the world have limited access to diagnostic studies and therapies for rheumatologic, musculoskeletal, and connective tissue diseases. The challenge has been particularly poignant for rural areas of low- and middle-income countries. Objectives: We report on the implementation of a telemedicine program in Iran for the evaluation and treatment of patients with rheumatologic and musculoskeletal diseases. More than 4,800 patients were seen remotely over a span of 5 years by a rheumatologist in the United States. The remote rheumatologist was aided by a general physician and a nurse at a local charity hospital in northeastern Iran that has a catchment area that includes rural regions extending to the border of Afghanistan. Seventy to 90 patients were evaluated online by the remote rheumatologist 3 days a week. A subset of patients was evaluated by the rheumatologist in person every 4 months. Materials and Methods: The population of rheumatology patients was evaluated using descriptive statistics. Information collected included demographic information consisting of age, gender, and primary rheumatologic diagnosis. Results: The average age of patients who were seen was 52 years and 89% of patients were women. Approximately 50% of patients were Afghan refugees. The most common disorders included osteoarthritis (1,149, 23.6%), rheumatoid arthritis (653, 13.4%), axial spondyloarthropathies (647, 13.3%), lumbar spinal stenosis (427, 8.8%), meniscal tear of the knee (326, 6.7%), and psoriatic arthritis (217, 4.5%). Certain conditions were lower than expected such as lupus (19, 0.4%) and fibromyalgia (169, 3.5%). Diagnostic tests included serologic tests (1,328, 27.3%), plain radiographs (946, 19.5%), magnetic resonance imaging (899, 18.5%), bone densitometry (147, 3.0%), and electromyography and nerve conduction study (132, 2.7%). The most common medications prescribed were non-steroidal anti-inflammatory drugs (791, 16.3%), methotrexate (764, 15.7%), pregabalin (234, 4.8%), duloxetine (230, 4.7%), sulfasalazine (177, 3.6%), etanercept (97, 2.0%), tofacitinib (64, 1.3%), adalimumab (18, 0.4%), and infliximab (9, 0.2%). Conclusions: Telemedicine is becoming more prevalent. We report the successful use of this service in evaluation and management of rheumatic diseases in a region with limited access to rheumatologic care. We have shown that patients can be seen, evaluated, and successfully treated with a variety of medications, including biologic agents.

Satisfaction with Subcutaneous Golimumab and its Auto-Injector among Rheumatoid Arthritis Patients with Inadequate Response to Adalimumab or Etanercept.

BACKGROUND: Patient perceptions of treatment success, including satisfaction/preference, may complement clinical efficacy assessments. OBJECTIVE: Our objective was to evaluate satisfaction with subcutaneous golimumab and its auto-injector in patients with rheumatoid arthritis (RA) and an inadequate adalimumab/etanercept response. METHODS: In the multicenter, assessor-blinded GO-SAVE study, 433 patients with active RA (28-joint Disease Activity Score incorporating erythrocyte sedimentation rate [DAS28-ESR] ≥ 3.6 and six or more swollen and six or more tender joints) despite methotrexate and past adalimumab/etanercept treatment received open-label subcutaneous golimumab 50 mg every 4 weeks (q4w) through week 12. Week 16 responders (DAS28-ESR improvement from baseline > 1.2 and score ≤ 3.2) continued therapy through week 52; nonresponders were randomized (1:2) to double-blind subcutaneous golimumab 50 mg q4w or intravenous golimumab 2 mg/kg [weeks 16, 20, every 8 weeks (q8w)]. Patients rated satisfaction with their injection experience on a 5-point scale (1 = very dissatisfied; 5 = very satisfied) at screening, week 8 (all enrolled patients), and week 44 (for patients continuing open-label subcutaneous golimumab 50 mg q4w). Discomfort, pain, stinging, burning, and redness related to injection were assessed (none, mild, moderate, severe). RESULTS: Similar proportions of patients (N = 433) had most recently received adalimumab (50.3%) or etanercept (49.7%) prior to golimumab. Overall satisfaction (somewhat/very) with the golimumab injection experience was reported by 84.4% of patients at week 8 versus 63.4% of patients who were satisfied with prior adalimumab/etanercept. Patients receiving open-label subcutaneous golimumab through week 44 (N = 75) reported much less discomfort (60.9%), redness (60.9%), pain (59.4%), stinging (67.2%), and burning (65.6%) with the golimumab injection than with their previous tumor necrosis factor antagonist medication injection. CONCLUSION: Most patients with RA receiving golimumab following adalimumab/etanercept inadequate response were satisfied with their overall golimumab experience, including its auto-injector versus their previous injection device. CLINICAL TRIALS.GOV: NCT01004432; EudraCT 2009-010582-23.

Clinical response to golimumab in rheumatoid arthritis patients who were receiving etanercept or adalimumab: results of a multicenter active treatment study.

OBJECTIVE: Evaluate the efficacy and safety of subcutaneous (SC) golimumab + methotrexate (MTX) in patients with active rheumatoid arthritis (RA) despite etanercept + MTX or adalimumab + MTX therapy and evaluate whether intravenous (IV) golimumab could rescue patients who were nonresponders to SC golimumab. METHODS: In this multicenter, assessor-blinded, active-switch study of patients with RA (n = 433) with inadequate response to etanercept or adalimumab + MTX, patients continued MTX and received open-label SC golimumab 50 mg every 4 weeks through week 12. DAS28-ESR good responders at week 16 continued open-label SC golimumab through week 52 (Group 1); nonresponders were randomized to double-blind golimumab SC 50 mg (Group 2-SC) or IV 2 mg/kg (Group 2-IV). Week 14 ACR20 was the primary endpoint; assessments continued through week 52 and for patients in the voluntary long-term extension through week 76. A major secondary endpoint was the proportions of patients with ACR20 response at week 52 relative to week 16 in Group 2-SC and Group 2-IV. RESULTS: At week 14, 34.9% (p < 0.001) achieved an ACR20. At week 52, patients in Group 1 (n = 75) achieved an ACR20 (62.7%). In Groups 2-SC (n = 91) and 2-IV (n = 184), 13.2% and 9.2% had an ACR20 at week 52 relative to week 16, with no significant difference between the randomized groups; 42.9% and 47.8% achieved DAS28-ESR response relative to week 0. Through week 16, 4.6% of patients had a serious adverse event. No differences in the rates or types of adverse events were observed between SC and IV golimumab from weeks 16 to 52. The trial limitations included a higher than expected discontinuation rate as a result of a programming error. CONCLUSION: SC golimumab + MTX significantly suppressed disease activity in RA patients with inadequate response to etanercept and/or adalimumab + MTX. Patients randomized to Groups 2-SC and 2-IV had lower response rates than Group 1, with no difference between SC or IV mode of administration. The safety profile with IV golimumab was comparable to that established with SC golimumab. TRIAL REGISTRATION: NCT01004432, EudraCT 2009-010582-23.

Cryoglobulin crystal arthropathy in a patient with multiple myeloma.

Joint involvement is unusual in patients with monoclonal gammopathies. It has been characteristically described as a rheumatoid-like seronegative polyarticular erosive arthropathy, which also has been related to crystal deposition of cryoglobulins in the synovium and several other tissues. This report describes the case of a 57-year-old African American woman with a seronegative polyarthritis associated with deposition of nonbirefringent or weakly positive birefringent rhomboid-shaped crystals in the synovial fluid. The patient, who was subsequently diagnosed with multiple myeloma, showed good clinical response to oral and intra-articular corticosteroids. Type II cryoglobulins were identified in the serum as well as in the synovial fluid. It is important to consider this association as part of the differential diagnosis of a patient with multiple myeloma and arthritis.

Inflammatory arthritis: an overview for primary care physicians.

Continuing advances in the treatment of inflammatory arthritides such as rheumatoid arthritis (RA), ankylosing spondylitis (AS), and psoriatic arthritis (PsA) have made remission a realistic goal for patients. Despite these advances, early diagnosis of inflammatory arthritis by primary care physicians (PCPs) and subsequent referral to a rheumatologist remain a challenge. Delayed diagnosis and referral, which may extend to several years in some cases, may lead to irreversible joint destruction and compromised function. The aim of this review is to aid PCPs in preventing the potential delay in disease recognition and patient referral by highlighting the currently accepted criteria for disease activity, clinical response, and remission of RA, AS, and PsA. In addition, a discussion of the benefits and risks of the currently approved traditional disease-modifying antirheumatic drugs and biologic treatments, and the importance of comanagement of these conditions across specialties, will be addressed. Because PCPs are often the first point of contact for disease recognition, they can play a critical role in the management of these patients.

The complexity of the differential diagnosis for the inflammatory arthritides.

Increasing evidence reveals that patients who have inflammatory arthritis experience structural damage early in the course of the disease. To effectively minimize the destruction caused by chronic inflammation, it is necessary to identify these patients soon after the onset of symptoms. However, differential diagnosis is not always straightforward. This article reviews selected underlying issues that complicate the differential diagnosis of the inflammatory arthritides and describes key features of the typical presentations for 3 of the commonly seen forms of inflammatory arthritis--rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis.

A case of persistent parvovirus B19 infection with bilateral cartilaginous and ligamentous damage to the wrists.

We describe a case of persistent parvovirus B19 infection in a 48-year-old female physician that was complicated by prolonged fatigue and arthritis associated with cartilaginous and ligamentous damage in both wrists. Nineteen months after presentation, intravenous immunoglobulin therapy resulted in clearance of parvovirus B19 viremia and a significant improvement in the symptoms of fatigue and arthritis.

Spondyloarthropathies.

The spondyloarthropathies include ankylosing spondylitis, reactive arthritis (including Reiter's syndrome), psoriatic arthritis, inflammatory bowel disease-associated spondyloarthropathy, and undifferentiated spondyloarthropathy. These diseases are linked by their association with the HLA-B27 gene and by the presence of enthesitis as the basic pathologic lesion. Additional clinical features include inflammatory back pain, dactylitis, and extra-articular manifestations such as uveitis and skin rash. The history and physical examination are the major diagnostic tools, although radiographic evidence of sacroiliitis is helpful. Therapeutic options include nonsteroidal anti-inflammatory drugs, sulfasalazine, methotrexate, and tumor necrosis factor-alpha inhibitors. Early recognition and appropriate treatment can help to limit disability.

Spondyloarthropathies: using presentation to make the diagnosis.

Diagnosing the spondyloarthropathies--chronic inflammatory diseases of the spine and peripheral joints that share several distinctive features--is challenging and depends on careful evaluation of the history, physical examination, and radiographs. The recent use of tumor necrosis factor inhibitors is exciting and may represent true disease-modifying drugs for these conditions.