Remitting Seronegative Symmetrical Synovitis With Pitting Oedema Associated With Malignancy: A Case Report.

Remitting seronegative symmetrical synovitis with pitting oedema is a rare rheumatological condition, predominating in the elderly male. It is characterised by the abrupt onset of marked pitting oedema, symmetrical distal synovitis, absence of rheumatoid factor and an excellent response to glucocorticoids. RS3PE may be the harbinger of a malignancy so the diagnosis should prompt evaluation and exclusion of such condition; in these cases, the response to glucocorticoids is only partial and treating the neoplasia is essential. The differential diagnosis includes late-onset rheumatoid arthritis, polymyalgia rheumatica and calcium pyrophosphate crystal-related arthritis. We present the case of a patient with remitting seronegative symmetrical synovitis with pitting oedema associated with clear cell renal cell carcinoma.

Understanding Palmar Fasciitis and Polyarthritis Syndrome as a Rheumatologic Paraneoplastic Syndrome: A Case Report.

Palmar fasciitis and polyarthritis syndrome (PFPAS) is an exceedingly rare rheumatologic condition characterized by fibrotic changes in the palmar fascia with joint pains. It is known to be associated with gynecological malignancy, especially ovarian adenocarcinoma, gastric cancer, pancreatic, prostate, breast, and lung cancer. We present a unique case of a 75-year-old Caucasian female with PFPAS preceding the diagnosis of ovarian cancer by eight months. Our case highlights the importance of considering PFPAS as a potential paraneoplastic syndrome. It underscores the need for increased awareness and further studies to enhance the early detection of underlying malignancies in patients presenting with similar nonspecific hand symptoms.

The protocol of a clinical effectiveness trial comparing standard step-up care, early combination DMARD therapy and early use of TNF inhibitors for the treatment of moderate to severe psoriatic arthritis: the 3-arm parallel group SPEED randomized controlled trial.

Objectives: The aim of the Severe Psoriatic arthritis - Early intervEntion to control Disease trial is to compare outcomes in psoriatic arthritis (PsA) patients with poor prognostic factors treated with standard step-up conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs), combination csDMARDs or a course of early biologics. Design: This multicentre UK trial was embedded within the MONITOR-PsA cohort, which uses a trial within cohort design. Methods and analysis: Patients with newly diagnosed PsA and at least one poor prognostic factor (polyarthritis, C-reactive protein >5 mg/dL, health assessment questionnaire >1, radiographic erosions) were randomized equally and open-label to either standard care with 'step-up' csDMARD therapy, initial therapy with combination csDMARDs (methotrexate with either sulfasalazine or leflunomide) or to early biologics induction therapy (adalimumab plus methotrexate). The primary outcome is the PsA disease activity score at week 24. Ethics: Ethical approval for the study was granted by the South Central Research Ethics Committee (ref 18/SC/0107). Discussion: Treatment recommendations for PsA suggest more intensive therapy for those with poor prognostic factors but there are no studies that have previously used prognostic factors to guide therapy. Applying initial intensive therapy has shown improved outcomes in other inflammatory arthritides but has never been tried in PsA. Combination csDMARDs have shown some superiority over single therapies but there are limited data and concerns about side effects. Early use of biologics has also been shown to be superior to methotrexate but these drugs are costly and not usually funded first line. However, if a short course of biologics can rapidly suppress inflammation allowing treatment to be withdrawn and response maintained on methotrexate, this may be a cost-effective model for early use. Trial registration: ClinicalTrials.gov (NCT03739853) and EudraCT (2017-004542-24).

Pancreatitis, Panniculitis, and Polyarthritis Syndrome in Two Patients: A Case Series and Literature Review.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome presents a unique challenge in diagnosis and management because of its rarity and heterogeneous initial presentation. This manuscript presents a case series of two patients with PPP syndrome, shedding light on the diagnostic process and care for this uncommon condition. PPP syndrome is characterized by the simultaneous occurrence of pancreatitis or pseudocysts alongside polyarthritis and panniculitis. While its exact pathophysiology remains obscure, pancreatic inflammation is assumed to trigger the hematogenous dissemination of pancreatic enzymes, leading to fat necrosis and subsequent panniculitis, as well as chondronecrosis and/or osteonecrosis causing polyarthritis. Despite its recognition in medical literature since the late 1980s, PPP syndrome remains poorly understood, with only a limited number of cases reported globally. Its rarity and varied initial manifestations often result in misdiagnosis, causing delays in appropriate treatment. The presented case series highlights key clinical features and diagnostic clues of PPP syndrome. Both patients exhibited initial symptoms of inflammatory polyarthritis, accompanied by characteristic findings of "ghost cells" on skin biopsy. Additionally, radiographic and laboratory evidence revealed pancreatic changes consistent with this syndrome. This case series underscores the importance of multidisciplinary collaboration in managing PPP syndrome. Early recognition and accurate diagnosis are pivotal in initiating prompt and effective therapeutic interventions, thereby improving patient outcomes and minimizing long-term sequelae.

An Enigmatic Case of Adult-Onset Still's Disease.

An inflammatory condition known as adult-onset Still's disease (AOSD) is typified by quotidian (daily) fevers, arthritis, and a transient rash. This report details the case of a 21-year-old female patient who presented to our hospital with polyarthritis, a red rash, and a high-grade fever for more than three months. History was suggestive of AOSD, which was further proved by investigation and Yamaguchi criteria for AOSD. Steroids and methotrexate were started, to which the patient responded very well and thereafter.

A rare case of polyarthritis panniculitis and pancreatitis.

Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin lesions (panniculitis), and pancreatic inflammation (pancreatitis), known as PPP. The pathogenesis of this phenomenon is not fully understood but is believed to involve lipolysis by pancreatic enzymes at lipid-rich skin and joint sites. PPP primarily affects middle-aged males with a history of alcohol use disorder. Diagnosis can be challenging due to the absence of typical abdominal symptoms. Delayed diagnosis may significantly worsen outcomes. Supportive therapy is the mainstay, but resolution requires addressing the underlying pancreatic abnormality. We present a case of a patient with a history of alcohol use disorder and recurrent acute pancreatitis who developed joint pain and skin rash. Extensive work-up ruled out other causes, and imaging and biopsy confirmed the diagnosis of PPP. Symptomatic management and treatment of the underlying pancreatic abnormality led to complete resolution of symptoms. Our case serves to raise awareness of this rare but potentially fatal syndrome.

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.

Leflunomide with prednisone or nonsteroidal anti-inflammatory drug therapy is safe and tolerated for long-term treatment of immune-mediated polyarthritis in 27 dogs.

OBJECTIVE: To retrospectively evaluate safety and tolerance of leflunomide for long-term treatment of canine idiopathic immune-mediated polyarthritis (IMPA). ANIMALS: 27 dogs with clinical signs and synovial fluid cytology supportive of IMPA with ≥ 6 months' follow-up after starting leflunomide. METHODS: Medical records were reviewed to identify dogs prescribed leflunomide for treatment of IMPA from February 2012 to May 2022. Initial leflunomide doses of 2 to 4 mg/kg once daily were prescribed and were titrated to the lowest effective dose with concurrent anti-inflammatory therapy. Complete blood count, serum chemistry, and clinical signs were monitored throughout the course of treatment. RESULTS: Adverse effects potentially attributable to leflunomide noted in 9 of 27 dogs (33%) included vomiting, diarrhea, lethargy, decreased or absent appetite, polyuria and polydipsia, and secondary antibiotic responsive infection and were self-limiting or resolved with outpatient therapy. Alkaline phosphatase (ALP) and alanine aminotransferase (ALT) elevation were documented in all dogs prescribed leflunomide plus prednisone, with persistent liver enzyme elevation in 6 of 9 dogs (67%) and normalization after antibiotic therapy in 3 of 9 dogs (33%). The majority of dogs prescribed leflunomide plus NSAID (11/17 [65%] dogs) did not experience liver enzyme elevation; 2 of 17 (12%) dogs developed transient antibiotic-responsive liver enzyme elevations, and 4 of 17 (23%) dogs had persistent liver enzyme elevation. CLINICAL RELEVANCE: Leflunomide was well tolerated for long-term management of IMPA. A significant difference in liver enzyme elevation was identified between dogs prescribed prednisone versus NSAID in combination with leflunomide. Leflunomide with NSAID therapy resulted in less hepatotoxicity compared with leflunomide with prednisone.

Pitfalls and pearls in diagnosing inflammatory arthritis in older patients.

Given current demographic shifts, the number of older adults continues to grow, with almost half of patients over 65 being diagnosed with some form of arthritis. Rheumatic diseases pose unique diagnostic challenges in older patients due to the convergence of physiologic changes of aging, confounding difficulties to care, and atypical disease manifestations. This review summarizes the current published evidence to guide clinicians in evaluating geriatric patients with rheumatologic concerns, focusing on inflammatory arthritis. Using the background of epidemiologic data on various musculoskeletal diseases, clinical presentations, current diagnostic tests, and known physiologic changes of aging, this review highlights five diagnostic pitfalls in inflammatory polyarthritis among older patients. The pitfalls include: 1) broader differential diagnosis; 2) atypical presentations; 3) communication, cognitive, and social impairments; 4) the role of chronological vs. biological age; and 5) anchoring bias by assuming older adults are simply "older young adults". These pitfalls are discussed in the context of geriatric principles such as the "hallmarks of aging" and the expected pathophysiologic changes of organ systems. Furthermore, the review discusses the strengths and weaknesses of diagnostic tests used in arthritis and introduces some of the geriatric assessment tools that systematically evaluate multimorbidity and geriatric syndromes. With familiarity of the potential diagnostic pitfalls, knowledge of both normal and pathologic aging processes, awareness of the difference between biological and chronological age, and the ability to use geriatric assessment tools to better characterize older patients, clinicians will be better able to diagnose and manage rheumatic conditions in this population.

Scleroderma Associated With Organising Pneumonia and Polyarthritis: A Report of a Rare Case.

Cryptogenic organising pneumonia (COP) is a form of idiopathic diffuse interstitial lung disease (ILD) that develops in response to a variety of unknown irritants. An essential component of the development of organising pneumonia (OP) is damage to type II pneumocytes and the alveolar basement membrane. An autoimmune illness called systemic sclerosis (SSc) has a significant death rate from cardiopulmonary involvement such as pulmonary hypertension and ILD. Arthritis is an autoimmune disorder, in which the patients experience extra-articular symptoms such as ILD during the course of their disease, and COP frequently coexists with these conditions. It is exceedingly uncommon for OP to occur as the initial sign of arthritis, and its clinical characteristics are still unclear. Scleroderma and inflammatory polyarthritis related to COP are presented in this report.

Diagnostic Utility of Thoracic Radiography and Abdominal Ultrasonography in Canine Immune-Mediated Polyarthritis: 77 Cases.

Thoracic radiography and abdominal ultrasonography are part of standard diagnostic investigations in cases of canine immune-mediated polyarthritis (IMPA). However, the clinical importance of thoracic and abdominal imaging towards the management of canine IMPA currently remains unknown. The primary aim of this study was to describe the findings documented on thoracic radiography and abdominal ultrasonography in dogs diagnosed with IMPA, and to evaluate the diagnostic utility of thoracic radiography and abdominal ultrasonography in the initial approach and management of these cases. Seventy-seven dogs diagnosed with IMPA who underwent thoracic radiography and abdominal ultrasonography at a single referral hospital between 2008 and 2022 were included. The diagnostic imaging studies of these 77 dogs were reviewed by one blinded board-certified diagnostic imaging specialist for quality assurance. The medical records, including the diagnostic imaging reports of these dogs, were then reviewed by three blinded board-certified internal medicine specialists. Using a modified version of a previous question and scoring system, the three internal medicine specialists then generated an answer for the overall diagnostic utility and a diagnostic utility score for thoracic radiography and abdominal ultrasonography for each case. The abnormal findings identified in radiography and ultrasonography were described. In the cases where the findings were considered significant enough to immediately affect the case management, the results of the further investigations that were subsequently performed were also described. No abnormalities were detected in thoracic radiography for 30 cases, and none were detected in abdominal ultrasound for 6. The majority of the internists considered thoracic radiography to be not useful in the overall case management at the time of IMPA diagnosis in 70 cases, and considered abdominal ultrasonography to be not useful in the overall case management in 57 cases. The majority of the internists agreed on the utility of thoracic radiography in 95% of the cases, and in 61% of the cases for abdominal ultrasonography. The most common finding in the thoracic radiography was a mild bronchial pulmonary pattern, and the most common in the abdominal ultrasonography was mild lymphadenomegaly. Therefore, although thoracic radiography and abdominal ultrasonography identified numerous abnormal findings in this population of dogs, in the majority of the cases, the findings were deemed not useful towards the overall case management at the time of the initial diagnosis of IMPA. Thus, the use of thoracic radiography and abdominal ultrasonography should be taken into careful consideration when considering initial diagnostic investigations for canine IMPA.

Immune-mediated polyarthritis and anterior uveitis secondary to zonisamide administration in a dog with refractory epilepsy.

The objective of this article is to describe a case of suspected zonisamide-induced immune-mediated polyarthritis (IMPA) and anterior uveitis in a dog. A 7-year-old male neutered Siberian Husky with a history of refractory idiopathic epilepsy was presented for cluster seizures. Following the addition of zonisamide to the antiepileptic regime, the dog developed new IMPA and anterior uveitis. Within a few weeks of discontinuation of the zonisamide, the dog's IMPA and anterior uveitis resolved. These immune-mediated conditions were thus presumed to be an idiosyncratic reaction to zonisamide. To our knowledge, this is the first report of IMPA and anterior uveitis in dogs associated with zonisamide administration at its recommended dose.

Retinal Vasculitis in a Patient with Rhupus.

This case report describes a case of retinal vasculitis in a patient with Rhupus, which has not been reported earlier in the literature. A 39-year-old female patient with a history of Rhupus presented with sudden vision loss in her left eye. The patient was treated earlier, for her polyarthritis, with oral corticosteroids, hydroxychloroquine, and oral methotrexate, which were discontinued after 1 year of treatment. At presentation, fundus examination of the left eye revealed mild vitritis, perivenous sheathing, and hemorrhagic periphlebitis. The patient was administered three doses of pulse corticosteroid, resulting in a reduction in retinal vasculitis and macular edema. The patient was continued on oral corticosteroids and started on oral methotrexate and hydroxychloroquine. At 6-week follow-up, the patient's vision had improved to 6/9, and fundus examination revealed resolving phlebitis and retinal hemorrhages. This case also highlights the importance of prompt diagnosis and treatment of retinal vasculitis in patients with Rhupus.

Synovial extramedullary hematopoiesis in a dog.

OBJECTIVE: Synovial extramedullary hematopoiesis is a rarely reported condition in humans and, to date, has never been reported in canines. This case report describes the clinical presentation, diagnostic work-up, treatment, and outcome of a canine case confirmed to have hematopoietic tissue within multiple joints. ANIMAL: A client-owned canine. CLINICAL PRESENTATION, PROGRESSION, AND PROCEDURES: The clinical presentation was most consistent with immune-mediated polyarthritis, and arthrocentesis was performed in multiple joints for cytological evaluation and culture. Cytology revealed evidence of extramedullary hematopoiesis, and shortly thereafter the dog was diagnosed with immune-mediated hemolytic anemia and thrombocytopenia. TREATMENT AND OUTCOME: Pregabalin, prednisolone, clopidogrel, and cyclosporine were started, and after several recheck appointments and dose adjustments, the dog's clinical signs resolved for all conditions. CLINICAL RELEVANCE: Unusual sites of extramedullary hematopoietic tissue may result in a clinical presentation for which more traditional etiologies and differentials are not applicable.

Role of DLA-DRB1 amino acids outside the shared epitope in dachshund susceptibility to immune-mediated polyarthritis.

Canine immune-mediated polyarthritis (IMPA) is an idiopathic disorder encompassing both erosive and non-erosive forms of rheumatoid arthritis (RA), with a clinical picture similar to human RA. Resemblance in major histocompatibility complex (MHC)-associated risk between the two was first noted within the specific amino acid motif known as the shared epitope (SE) on human leukocyte antigen DRB1. Following further identification of amino acids conferring risk for human RA outside the SE, this study was designed to examine amino acids both within and outside the classic SE in dachshunds, a breed with reported susceptibility to IMPA in Japan. Genome-wide association studies have linked positions 11, 13 and 71 with strong risk for human RA and important roles in antigen presentation to T cells. Sequence based genotyping of 16 case and 64 control dachshunds revealed strong associations comparable to human RA between IMPA risk and valine at position 11 (Val-11), phenylalanine at 13 (Phe-13), and arginine at 71 (Arg-71) on the dog leukocyte antigen (DLA)-DRB1 molecule (OR 2.89, 95%CI 1.3-6.4, p = 0.009), while association with the classic SE was significant only regarding homozygote frequency of the QRRAA haplotype-also carrying Val 11 and Phe 13 outside the SE (p = 0.04). Moreover, limited range in possible combinations of amino acids at positions 11, 13 and 71 starting with Val-11 among all DLA-DRB1 alleles registered with the GenBank and IPD-MHC canine databases, suggested potential of further single-breed analyses in dachshunds to clarify the disorder in terms of diagnosis, treatment, and epigenetic control, while clinical and immunopathogenetic similarities between human and dachshund RA also suggested the possibility of gaining insight into RA per se through study of canine IMPA as a spontaneous model of human RA.

Paraneoplastic Polyarthritis in a Patient with Synchronous Lung and Colorectal Malignancy.

Arthritis is an unusual manifestation of paraneoplastic syndrome, appearing in a variety of cancers, including pulmonary and colorectal. It can often pose a diagnostic challenge to physicians, since it may be difficult to distinguish from more commonly encountered rheumatic illnesses. Moreover, synchronous cancers are rare and unexpected in patients with symmetrical polyarthritis. Hypertrophic pulmonary osteoarthropathy is to be considered in patients with polyarthritis and lung neoplasia. The aim of this report is to highlight the case of a patient presenting with paraneoplastic polyarthritis, which led to identifying the presence of underlying synchronous lung and colorectal malignancies. Lymph node biopsy was performed raising suspicion of Caplan's syndrome but lung lobectomy confirmed adenocarcinoma. Rheumatologists should be reacquainted with rheumatic manifestations in malignant diseases.

Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS).

A prolonged fever in a child can be due to a range of causes including infectious, autoimmune, malignant, or genetic in etiology. In our report, we present the case of a previously healthy three-year-old female diagnosed with macrophage activation syndrome (MAS) due to complications of systemic juvenile arthritis (sJIA). MAS is considered a secondary subtype of hemophagocytic lymphohistiocytosis (HLH), a rare and life-threatening group of syndromes characterized by overstimulation of the immune system leading to systemic inflammation. Through our case, we wanted to bring awareness to this uncommon group of diseases as well as discuss the importance of differentiating between its subtypes. While HLH and MAS have similar clinical presentations, the treatment regimen for each is distinct. Moreover, further research should be conducted to create standardized criteria and treatment guidelines that are evidence-based in order to properly manage these patients.

Associations of inflammatory polyarthritis with clinical and radiological findings of temporomandibular disorders.

OBJECTIVE: To evaluate the association of different forms of inflammatory polyarthritis with clinical signs of temporomandibular disorders (TMD) and radiological findings in temporomandibular joint (TMJ), based on a nationwide health survey in Finland. The aim was also to assess the presence of clinical and radiological TMD findings in subjects with increased risk for developing rheumatoid arthritis (RA). MATERIAL AND METHODS: A nationally representative sample included 6331 Finnish adults who participated in the Health 2000 Survey (BRIF8901). Subjects were examined for signs of TMD, findings in panoramic radiograph of TMJ, musculoskeletal health and serology (rheumatoid factor, RF, and anti-cyclic citrullinated peptide, aCCP). RESULTS: Sixty-four percent of seronegative RA and 60% of seropositive RA subjects had at least one sign of TMD. While adjusting for confounding factors (gender, age, dentures and smoking history), RA was significantly associated with crepitation and abnormal radiological findings in TMJ. Seronegative RA was also associated with restricted mouth opening. Systemic autoimmunity associated with RA ("at risk of RA") was not associated with clinical or radiological TMD findings. CONCLUSIONS: Clinical and radiological findings of TMD are more prevalent among subjects with inflammatory polyarthritis than among the population in general in the Finnish adult population.