Mitral valve rheumatoid nodule complicated by infective endocarditis.

We report a case of a 73-year-old male with rheumatoid arthritis presenting with acute abdominal and back pain and rapidly developing multiorgan failure. A positive blood culture (Staphylococcus aureus, Candida species) followed by transoesophageal sonography established a diagnosis of mitral valve infective endocarditis. At the autopsy, the heart examination revealed fibrinous pericarditis and multiple small vegetations on the mitral valve. The mitral valve itself showed no significant damage. Surprisingly, the histological examination of the mitral valve showed granulomatous inflammation with central fibrinoid necrosis and peripheral palisade of histiocytes, with occasional giant cells and lymphocytic inflammatory infiltrate - findings consistent with a rheumatoid nodule. Infective vegetations were overlying the nodule. Due to its relative frequency, a possibility of cardiac involvement by rheumatoid arthritis and its potential infective complications should be considered in patients with appropriate history and clinical symptoms.

Case 238: Spontaneous Pneumothorax Secondary to Intrapulmonary Necrobiotic Rheumatoid Nodule.

History A 54-year-old white woman with a history of rheumatoid arthritis who was taking glucocorticoids and methotrexate presented to the emergency department in December with worsening shortness of breath and chest heaviness for 1 week. She reported additional symptoms of weakness, headache, and arthralgia primarily involving her bilateral hands, wrist, ankles, and feet. She denied experiencing fevers, syncope or presyncope, focal neurologic deficits, chest pain, nausea, vomiting, unintentional weight loss, or recent trauma. Additional medical history included hypertension, asthma, degenerative disk disease, and migraine, all of which were reportedly controlled with medications. This patient had a smoking history of 80 pack-years, but she had quit smoking 2 months prior to presentation. She denied abuse of alcohol or recreational drugs and reported she was up-to-date on her immunizations, including those for pneumonia and flu. Family history was pertinent for breast cancer in her mother, sister, and maternal aunt. The patient reported normal findings at screening mammography and colonoscopy. A physical examination was remarkable for slightly asymmetric breath sounds, which appeared to be diminished on the right side. This patient had multiple joint deformities, most notably in the bilateral metacarpophalangeal joints. Initial electrocardiography findings and cardiac biomarkers were negative. Her complete blood count and basic metabolic profile were unremarkable. Posteroanterior and lateral chest radiographs were obtained in the emergency department. Subsequently, computed tomography (CT) of the chest was performed.

The prevalence and risk factors of rheumatoid arthritis-associated interstitial lung disease: a systematic review and meta-analysis.

BACKGROUND: Interstitial lung disease (ILD) is the most widespread and fatal pulmonary complication of rheumatoid arthritis (RA). Existing knowledge on the prevalence and risk factors of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is inconclusive. Therefore, we designed this review to address this gap. MATERIALS AND METHODS: To find relevant observational studies discussing the prevalence and/or risk factors of RA-ILD, EMBASE, Web of Science, PubMed, and the Cochrane Library were explored. The pooled odds ratios (ORs) / hazard ratios (HRs) with 95% confidence intervals (CIs) were estimated with a fixed/ random effects model. While subgroup analysis, meta-regression analysis and sensitivity analysis were carried out to determine the sources of heterogeneity, the I2 statistic was utilized to assess between-studies heterogeneity. Funnel plots and Egger's test were employed to assess publication bias. Following the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) guidelines, our review was conducted. RESULTS: A total of 56 studies with 11,851 RA-ILD patients were included in this meta-analysis. The pooled prevalence of RA-ILD was 18.7% (95% CI 15.8-21.6) with significant heterogeneity (I2 = 96.4%). The prevalence of RA-ILD was found to be more likely as a result of several identified factors, including male sex (ORs = 1.92 95% CI 1.70-2.16), older age (WMDs = 6.89, 95% CI 3.10-10.67), having a smoking history (ORs =1.91, 95% CI 1.48-2.47), pulmonary comorbidities predicted (HRs = 2.08, 95% CI 1.89-2.30), longer RA duration (ORs = 1.03, 95% CI 1.01-1.05), older age of RA onset (WMDs =4.46, 95% CI 0.63-8.29), positive RF (HRs = 1.15, 95%CI 0.75-1.77; ORs = 2.11, 95%CI 1.65-2.68), positive ACPA (ORs = 2.11, 95%CI 1.65-2.68), higher ESR (ORs = 1.008, 95%CI 1.002-1.014), moderate and high DAS28 (≥3.2) (ORs = 1.87, 95%CI 1.36-2.58), rheumatoid nodules (ORs = 1.87, 95% CI 1.18-2.98), LEF use (ORs = 1.42, 95%CI 1.08-1.87) and steroid use (HRs= 1.70, 1.13-2.55). The use of biological agents was a protective factor (HRs = 0.77, 95% CI 0.69-0.87). CONCLUSION(S): The pooled prevalence of RA-ILD in our study was approximately 18.7%. Furthermore, we identified 13 risk factors for RA-ILD, including male sex, older age, having a smoking history, pulmonary comorbidities, older age of RA onset, longer RA duration, positive RF, positive ACPA, higher ESR, moderate and high DAS28 (≥3.2), rheumatoid nodules, LEF use and steroid use. Additionally, biological agents use was a protective factor.

Bamboo nodes associated with mixed connective tissue disease as a cause of hoarseness.

Vocal fold lesions related to autoimmune diseases are rheumatoid nodules and, to a lesser extent, bamboo nodes. Mostly transverse, they are located in the middle third of the vocal cord and exhibit a yellowish appearance. The characteristic shape of these lesions led to their name. These vocal fold deposits may interfere with the normal vibratory cycle during phonation and thus may be an unusual cause of hoarseness. We present a 43-year-old woman with known mixed connective tissue disease and a dysphonia. Laryngostroboscopy showed bamboo nodes as described above. We applied several laryngeal injections of cortisone as described previously in the literature. Since this treatment did not lead to a sufficient voice improvement, we attempted to surgically remove the deposits. After the surgery, the voice improved considerably. In all patients with rheumatic diseases who suffer from a rough, breathy, or unstable voice, a laryngostroboscopic examination should be done. If, however, a bamboo node lesion of the vocal folds is found by the laryngologists, an associated autoimmune disorder must be assumed, and adequate diagnostic procedures have to be initiated. Local laryngeal injections (1-3 times) with steroids should be the first line of therapy. In unsuccessful cases, subsequent surgery can be a useful treatment of bamboo nodes to stabilize and improve voice quality.

Cutaneous necrobiotic conditions associated with rheumatoid arthritis: important extra-articular involvement.

Rheumatoid arthritis (RA) presents with various skin conditions as extra-articular manifestations. Rheumatoid nodule is the representative specific skin lesion, histologically exhibiting central necrosis (necrobiosis) surrounded by palisaded macrophages, and being further perivascularly infiltrated with inflammatory cells in the outer regions. Also, there are several skin lesions which histologically show necrobiotic conditions with altered connective tissue degeneration. Necrobiosis may be closely associated with the pathogenesis of RA, i.e., collagen degeneration, recruitment of activated neutrophils, production of various cytokines, and vascular injury. On the other hand, rheumatoid nodule is suggested to develop during therapies with certain drugs such as methotrexate and biologics. These findings may be a clue to understanding the pathomechanisms of rheumatoid nodules. This paper describes several necrobiotic conditions associated with RA, and also discusses the possible pathogenesis and differential diagnosis of rheumatoid nodules. Necrobiosis is the major pathologic condition of cutaneous involvement associated with RA.

Intrapulmonary rheumatoid nodules in a patient with long-standing rheumatoid arthritis treated with leflunomide.

Rheumatoid nodules are well established manifestations of rheumatoid arthritis but in the lungs they are very rare according to the literature. In our study we present the case of a 34-year-old woman with rheumatoid arthritis and secondary Sjögren's syndrome who developed multiplex rheumatoid nodules in the lungs 3 years after initiating leflunomide therapy. During leflunomide therapy we did not detect inflammation in the joints. Surprisingly, in November 2005 she started to cough, had low grade fever and low back pain. On the chest X-ray there were multiplex necrobiotic nodules in the lungs. All bacteriological, viral and fungal investigations including tuberculosis, serological tests and cytology were negative. The X-ray, video-associated thoracoscopy and repeated biopsy of the lung followed by histology of the samples proved intrapulmonary rheumatoid nodules, caused by leflunomide.

A rare case of enterobacter endocarditis superimposed on a mitral valve rheumatoid nodule.

We present the case of a 56-year-old man with longstanding seropositive active erosive and deforming rheumatoid arthritis with no peripheral rheumatoid nodules; he immigrated from the former Soviet Union (where he did not receive any disease-modifying antirheumatic drugs) to Israel in 1995. In February 2005, he had a buccogingival mucosal abscess on his lower lip, which was treated by surgical drainage, followed by prolonged antibiotic therapy. One and a half years later, he had 2 episodes of transient ischemic attacks characterized by speech difficulties and moderate weakness on his right side. Transesophageal echocardiogram revealed a mass on the anterior mitral valve leaflet. Repeated blood cultures were negative, and the patient was afebrile all the time. The patient underwent mitral valve replacement and the histologic findings of the mass were typical of both a rheumatoid nodule and bacterial endocarditis. The patient recovered fully after 6 weeks of antibiotic therapy. Emboli from a rheumatoid nodule should always be considered in patients with rheumatoid arthritis who present with transient ischemic attacks.

[A case of rheumatoid arthritis with pneumothorax due to subpleural pulmonary rheumatoid nodules].

Pneumothorax secondary to nodular rheumatoid lung disease is a rare complication of rheumatoid arthritis. Here we report a case of rheumatoid arthritis with pneumothorax due to subpleural pulmonary rheumatoid nodule. A 74-year-old woman with a 14-year history of rheumatoid arthritis has admitted to our hospital due to dyspnea and right chest discomfort, and her chest X-ray film and computed tomography revealed right pneumothorax. Her chest X-ray and computed tomography findings before the onset of pneumothorax had demonstrated multiple subpleural cavitary nodules in both lungs. She had taken antifungal agents under a diagnosis of pulmonary fungal infection for a year without any change of her chest radiological findings. After incomplete reexpansion with sustained air leakage by right chest tube drainage, video-assisted thoracic surgery was performed. The pulmonary cavitary nodules of her right middle and lower lobes were with successfully excised. The histopathology of excised subpleural lung nodules showed typical features of rheumatoid nodules, and the cavitation of the rheumatoid nodule in right S5 had fistula formation to the pleural space, and thus was thought to have caused the pneumothorax.

Lumbar radiculopathy caused by extradural rheumatoid nodules. Case report.

The authors report on a 51-year-old woman with a 9-year history of rheumatoid arthritis (RA) who presented with symptomatic rheumatoid nodules in the lumbar extradural region with compression on the L-5 nerve roots bilaterally. She had also suffered from dysesthesia in the right lower leg and intermittent claudication. Magnetic resonance imaging revealed masses compressing the dural sac, and on lumbar myelography and computed tomography myelography a filling defect at L4-5 was revealed, which was compressing the dural sac posterolaterally on both sides. The masses were surgically removed. On histological examination the typical characteristics of rheumatoid nodules were found. Soon after the operation all of the patient's symptoms disappeared. There have been few reports on extradural rheumatoid nodules. Patients with RA usually complain of articular symptoms, and in fact the patient in the present study had been referred to the authors' institution for total hip arthroplasty. However, various symptoms other than those arising from articular lesions were found clinically. The authors believe that if patients with RA are also examined for extraarticular lesions, it is likely that these will be more frequently detected.

[The functional condition of the left ventricle in patients suffering from rheumatoid arthritis with subcutaneous rheumatoid nodules].

The purpose of the study was to evaluate the systolic and diastolic left ventricular (LV) function in patients with the system form of rheumatoid arthritis (RA) with subcutaneous rheumatoid nodules (SRN). The subjects, 105 patients with proved RA, were divided into two groups. The main group consisted of 40 patients suffering from system RA with SRN. The comparison groups included 40 patients suffering from the articular-and-visceral form of RA without SRN, and 25 patients with the articular form of RA. The systolic LV function was evaluated in all the 105 RA patients using M-mode Echo CG. In 20 patients with SRN systolic and diastolic LV function was evaluated with radionuclide ventriculography (RVG). The hemodynamic parameters obtained with Echo CG and RVG were processed according to variation statistics rules, presenting the data in groups as a mean and the standard error (M +/- m); the results were compared between the patients and between the patients and healthy controls. The significance was estimated using Student criterion (p < 0.05). In the group of RA patients with SRN, Echo CG and RVG found a significant decrease in LV systolic function parameters vs. patients without SRN and healthy controls. RVG revealed LV diastolic dysfunction in RA patients with SRN. Systolodiastolic LV dysfunction in these patients suggests myocardial involvement. Present-day instrumental methods of the evaluation of functional heart condition may be used to evaluate systolic and diastolic LV function in RA patients, especially during the dynamic observation of the effectiveness of RA treatment with modern anti-rheumatic preparations.

Tumefactive necrobiotic granulomas (nodulosis) of the pancreas in an adult with long-standing rheumatoid arthritis.

Rheumatoid nodules are well-documented clinical and pathologic lesions in patients with seropositive rheumatoid arthritis (RA). The current report documents the occurrence of rheumatoid nodulosis of the pancreas in an adult woman with a 7-year history of seropositive RA who presented with upper abdominal pain and was found to have multiple masses in the body and tail of the pancreas by imaging studies. An elevated serum pancreatic polypeptide (PP) and the development of new lesions in the pancreas prompted a subsequent distal pancreatectomy. The lesions in the pancreas proved to be necrobiotic palisading and hyalinizing granulomas upon pathologic examination. Also, of interest, elevation of serum PP has been observed in patients with RA and other systemic noninfectious and infectious inflammatory disorders in the absence of a pancreatic or intestinal neuroendocrine neoplasm.

Multiple rheumatoid nodules with rapid thin-walled cavity formation producing pneumothorax.

We report a rare case of multiple rheumatoid nodules that on chest CT demonstrated peripheral location with thin-walled cavity formation, rapid increase in size within several months, and complicating pneumothorax, pneumomediastinum, and subcutaneous emphysema due to bronchopleural fistula formation.

Ascites and other incidental findings revealing undiagnosed systemic rheumatoid arthritis.

We describe a case of a 43-year-old man presenting to the gastroenterology outpatient department with exudative ascites. Mediastinal lymphadenopathy, pericardial effusion and pleural effusion were detected on further imaging. Further clinical examination revealed subcutaneous nodules on the left arm, which were confirmed to be rheumatoid nodules on histology. Inflammatory markers were elevated with positive serology for rheumatoid factor and anticyclic citrullinated protein antibody. Our investigations excluded tuberculosis, pancreatitis and malignancy in the patient. Following review by a rheumatologist, a diagnosis of systemic rheumatoid arthritis (RA) was made. Pleuritis and pericarditis are well recognised as extra-articular manifestation of RA. Ascites, however, is rarely recognised as a manifestation of RA. Our literature search revealed two other cases of ascites due to RA disease activity, and both patients had long-standing known RA. This case adds to the discussion on whether ascites and peritonitis should be classified as extra-articular manifestations of RA.

Seizures due to central nervous system rheumatoid meningovasculitis.

A patient with rheumatoid arthritis and seizures had rheumatoid meningovasculitis on brain biopsy. Infection was excluded as a cause of the seizures and cerebrospinal fluid abnormalities, which resolved with corticosteroids and azathioprine therapy.

Rheumatoid nodules. Review of the spectrum of associated conditions and proposal of a new classification, with a report of four seronegative cases.

The literature relating to prevalence, characteristics, differential diagnosis, histologic features, pathogenesis, complications, and prognosis of rheumatoid nodules is tabulated and reviewed. The broad spectrum of conditions associated with rheumatoid nodules is discussed. A new classification of these clinical entities, reflecting disease characteristics and prognosis, is proposed. This classification is based on age, association or lack thereof with rheumatoid arthritis or rheumatic features, the presence or absence of rheumatoid factors, and the location of the rheumatoid nodules. Four cases of rheumatoid nodules in patients without rheumatoid factor are presented. Three patients had classic or definite rheumatoid arthritis, and one had palindromic rheumatism. In follow-ups lasting one to 15 years, significant permanent joint deformities, bony erosions, or extra-articular manifestations of rheumatoid arthritis have not developed in any of the patients. All have experienced significant periods of remission.

Aortic valve rheumatoid nodules producing clinical aortic regurgitation and a review of the literature.

The majority of cardiac involvement in rheumatoid arthritis (RA) is an incidental finding at postmortem, as less than 3% of patients with RA have clinical cardiac signs or symptoms. Most cardiac involvement in RA involves the pericardium and has been known since Charcot first described an RA patient with pericarditis in 1881. Cardiac involvement takes two different forms: non-specific inflammatory changes and specific granuloma formation. Specific rheumatoid nodules in the heart are an infrequent complication of RA. This is the first case report of a surgically excised heart valve with rheumatoid nodules. A 74-year-old RA patient with a high seropositive rheumatoid factor presented with severe aortic regurgitation and underwent a valve replacement. The native aortic valve showed significant stenosis with multiple, classic rheumatoid nodules.