Agranulocytosis in Sjögren's syndrome: two case reports and analysis of 11 additional reported cases.

OBJECTIVES: To report 2 patients who presented with agranulocytosis that was found to be immune-mediated and associated with occult primary Sjögren's syndrome (primary SS) and to identify and study similar cases reported in the literature. METHODS: Two patients encountered in 2 large medical centers over a period of 5 years were studied in detail. All reported cases of agranulocytosis in primary SS identified through a MEDLINE search were reviewed. RESULTS: Two patients presented with marked systemic symptoms alone or associated with recurrent infections. Agranulocytosis with either a pattern of maturation arrest or a hypercellular reactive bone marrow was found and was associated with "acute phase" markers, hypergammaglobulinemia, a small paraprotein peak, and high rheumatoid factor titers. A diagnosis of immune-mediated agranulocytosis associated with an occult primary SS was established and was successfully treated with intravenous immunoglobulins or prednisone. Both patients subsequently developed skin vasculitis. This rare association of agranulocytosis and Sjögren's syndrome was identified in 11 other cases and was the presenting manifestation of primary SS in 10 of 13 (77%) patients. CONCLUSIONS: Agranulocytosis should be recognized as a rare but well-established association of primary SS. Bone marrow neutrophil production may be affected, or neutrophils may be destroyed in the circulation, by both humoral and cellular immune-mediated mechanisms. Agranulocytosis or neutropenia should be added to the varied hematologic manifestations of primary SS and may be its presenting feature and an important clue to diagnosis.

Risk factors for disability among U.S. adults with arthritis.

This article studies risk factors for physical and social disability among U.S. adults ages 55+ who have arthritis, compared to non-arthritis persons of those ages. The dependent variables refer to difficulties in walking, physical functioning (motions and strength), personal care, and household care. The data set is the Supplement on Aging (SOA) (n = 16,148) that accompanied the 1984 National Health Interview Survey. The SOA data are cross-sectional; relationships of risk factors to disability suggest causation but do not directly demonstrate it. Logistic regressions show that risk factors are similar for arthritis and non-arthritis people, with one important exception. (1) The similarities are: For both groups, odds of disability rise with age, diminish with education, and are higher for non-whites and non-married persons. Disability rises with number of chronic diseases and impairments, and it is elevated for underweight persons (Body Mass Index (BMI) less than 20; further analysis indicates this reflects incomplete control of their severe illness status). Long duration of arthritis and recent medical care for it are associated with disability. (2) The exception is: Severe overweight (BMI greater than or equal to 30) is a disability risk factor for arthritis people, but not for non-arthritis people. Previous research has shown that obesity/overweight is a risk factor for etiology of osteoarthritis; our analysis now shows its continued importance for disability when the disease is present.

The role of arthroscopy in the differential diagnosis of osteoarthritis of the knee.

The arthroscope, currently used in OA mostly as a tool to deliver surgical therapy, can play an important diagnostic role in patients with knee pain for whom OA is part of the differential diagnosis. Concepts that may seem foreign to clinicians who take care of patients with knee OA (that is, of arthroscopy as a purely diagnostic tool and of knee OA as a clinical situation for which a differential diagnosis might be rendered) are supported by an appreciation for the heterogeneity of conditions of which OA is a part as well as an awareness of the pathoanatomic complexity of symptomatic knee OA as revealed by recent MRI and arthroscopy studies. Inspection of the perplexing OA knee with one of the newly available needle arthroscopes in an ambulatory office setting may be preferable to referral for conventional operating room--based arthroscopy, as this new technique adequately delineates intra-articular anatomical abnormalities (for which surgical therapy is not always indicated) and provides saline lavage to the joint (considered by many as a major factor in the improvement of symptoms reported by many patients with knee OA after arthroscopy). At present arthroscopy can serve to aid differential diagnostic efforts in five clinical situations involving knee OA: painful swollen knee with normal radiographs and noninflammatory fluid, clinical and radiographic OA with pain out of proportion to radiographic findings and refractory to conventional medical therapy, chronic stable (radiographic) OA with profound worsening of symptoms, OA with predominate "mechanical" symptoms, and OA with unexpected synovial fluid characteristics. Future use of needle arthroscopy in knee OA could serve to define the intraarticular correlates of pain in OA, to identify specific subgroups of knee OA upon which prospective randomized testing of arthroscopic surgical interventions could be conducted, and to quantitatively assess the effects of new treatment modalities on articular cartilage and other intraarticular structures.

Diagnostic arthroscopy in the arthritis patient.

The arthroscope can play an important diagnostic role in the arthritis patient. The major utility of this procedure is in the patient with unexplained knee pain and swelling or in the patient with an established knee arthritis whose symptoms are disproportionate to radiographic findings or refractory to standard-course medical therapy. Technologic advances have led to the production of smaller instruments, making office-based diagnostic arthroscopy a practical, cost-effective alternative in the evaluation of these patients, and supporting the clinical argument for it as a procedure distinct from conventional arthroscopy. Separate clinical scenarios further subdivide the indications for diagnostic arthroscopy and define potential intra-articular abnormalities that, if found, can justify alterations in or additions to therapeutic plans, including arthroscopically directed tissue resection and modification or application of tissue-modifying agents. The research capabilities of needle arthroscopy are only just beginning to be realized; opportunities now exist for design of prospective clinical trials in which patients are randomized based on intra-articular abnormalities, and for the serial assessment of specific treatment effects on gross, microscopic, and molecular features of target tissue as identified by the arthroscope.

Comparing information-gathering strategies of medical students and physicians in diagnosing simulated medical cases.

Using a broad range of written patient management problems (PMPs), this study examined (1) how each of three medical information-gathering processes (history-taking, physical examination, and diagnostic studies) influenced 175 second-year medical students' formulations of the differential (i.e., plausible) and the principal (i.e., most probable) diagnoses for each of 14 PMPs, and (2) the extent to which these results paralleled the emphases that experienced clinicians placed on these same information-gathering processes regarding each of the same PMPs. The results suggest that in ten of the 14 PMPs the students appeared to rely on specific information-gathering strategies in formulating their diagnoses, and that both similarities and differences existed between the levels of emphasis placed by the students and physicians on each of the three processes. In general, the physicians placed greater emphasis on the importance of the history, whereas the students relied more on diagnostic studies. These variations have implications for selecting medical problems for purposes of instruction and evaluation of students.

Arthritis and Aerobic Exercise: A Review.

In brief: Despite increasing evidence that regular aerobic exercise yields many benefits for patients with arthritis, patients often are advised to curtail physical activity. Findings from studies of patients with either rheumatoid arthritis or osteoarthritis who participated in an aerobic exercise program show that the subjects made significant gains in aerobic capacity, functional status, muscle strength, and other aspects of performance. In addition, they improved in subjective aspects that might have a positive impact on quality of life, including pain tolerance, joint pain, mood, and social activity. The authors discuss some questions that remain unanswered and present guidelines for physicians who wish to prescribe aerobic exercise for their arthritis patients.

Tidal irrigation in septic arthritis of the knee: a potential alternative to surgical drainage.

OBJECTIVE: To evaluate the efficacy of tidal irrigation (TI: repeated distention and irrigation of joint with saline under local anesthesia through 14 gauge Vere's needle) as a method to effect drainage in septic arthritis of the knee. METHODS: Eleven episodes of septic arthritis of the knee in 10 patients were approached with TI when drainage of purulent material could no longer be satisfactorily accomplished with repeated arthrocenteses. Clinical and synovial fluid (SF) data were systematically collected and analyzed. RESULTS: Four out of 11 episodes resolved without resorting to further surgical drainage. Cases responding to TI involved significantly older patients with lower volume effusions at presentation. Cases not responding to TI more frequently involved gram negative infection (3/7 cases vs 0/4). Variables of SF obtained preceding and following the first TI predicted outcome, with 3/4 responding cases demonstrating both a drop in white blood cell count of > or = 25% and a decrement of > or = 50% in volume while none of the cases eventually requiring surgery responded in this fashion (p < 0.05). CONCLUSIONS: TI can potentially supplant conventional surgical drainage in some cases of septic knee arthritis that do not respond to medical management.

Septic arthritis due to group C streptococcus: report and review of the literature.

A case of polyarticular septic arthritis due to Lancefield group C streptococcus is described and the clinical details of 9 reported cases of septic arthritis due to group C streptococcus reviewed. Several features of the presentation and course of these patients, including polyarticular involvement (4/10), bacteremia (5/10), clinically important dysfunction in other systems (4/10 patients: cardiac, 3; CNS, 2; pneumonia, 1; gastrointestinal, 1), fatal outcome (3/10 patients, 2 during the course of active infection) and poor functional outcome in affected joints (4/7 surviving patients), serve to place group C streptococcus septic arthritis as a syndrome distinct from that usually expected with streptococcal septic arthritis. Specific features of the bacteriology of group C streptococcus are reviewed (including confusion with group A streptococcus when analyzed using nonserologic methods, occasional zoonotic source, and frequency of tolerance to penicillin) in order to emphasize the importance of both the specific identification of this particular streptococcal strain and the initiation of aggressive antibiotic therapy when group C streptococcus is identified as the causative agent in a case of septic arthritis.

Diagnostic arthroscopy.

Arthroscopy has served a diagnostic role for most of this century, but found widespread popularity only when operative interventions were coupled with the procedure. The untapped potential inherent in directly observing the pathoanatomy underlying various rheumatologic disorders is being unlocked by developments on several fronts that have taken arthroscopy away from the operating room environment. Information from arthroscopy can influence diagnosis and treatment in certain non-traumatic knee disorders, particularly when the cause of synovitis is not evident from other clinical features and when knee symptoms are accompanied by bland synovial fluid and X-rays that are normal or show only minimal changes of osteoarthritis. Other joints can now be arthroscoped, which may prove useful for rheumatological diagnosis and evaluation, particularly for the smaller joints of the upper extremity commonly affected in 'early' disease states.

Bacterial arthritis.

Reports pertinent to bacterial arthritis in 1997 included two large, multi-year surveys of joint infection in patients from defined European health districts, noting trends including the declining incidence of gonococcal arthritis and an increasing number of prosthetic joint infections. Children with infected joints generally fare better than adults despite having proportionately more infections due to gram-negative organisms, of which Hemophilus influenzae comprises an ever smaller portion as the fastidious Kingella kingea is emerging. Joint infections remain an uncommon complication of immunodeficiency due to HIV, with responsible agents, affected sites, and clinical course also influenced by certain HIV comorbidities such as intravenous drug user and hemophilia. The rare immunodeficient patient with hypogammaglobulinemia retains a nearly unique susceptibility to joint infection with mycoplasmas, which can cause considerable morbidity if not promptly recognized and treated. Polymerase chain reaction can detect remnants of bacteria in the face of negative conventional cultures, but inoculation of synovial fluid into blood cultures bottles may be a more immediate and practical method to increase the yield in suspected septic arthritis.

Bronchiolitis obliterans organizing pneumonia and Sjögren's syndrome.

A 52-year-old man developed a systemic illness with fever, malaise, cough, dry eyes and mouth. Investigation revealed bronchiolitis obliterans organizing pneumonia associated with retinal vasculitis, hepatic and renal involvement, lymphocytic sialoadenitis and symptoms of the sicca complex. High dose steroid treatment resulted in resolution of his symptoms and prolonged remission.

Scleromyxedema with systemic involvement mimics rheumatic diseases.

Scleromyxedema is an infiltrative skin disease produced by hyaluronic acid deposition in the dermis. A benign monoclonal gammopathy is usually present. We report 2 patients with scleromyxedema and systemic illnesses. Both patients had muscle weakness, dysphagia, and weight loss in addition to the skin changes. One also had sclerodactyly, telangiectasias, and Raynaud's phenomenon. Scleromyxedema with systemic involvement may mimic rheumatic diseases.

Detection of intraarticular abnormalities in osteoarthritis of the knee. A pilot study comparing needle arthroscopy with standard arthroscopy.

OBJECTIVE: To determine whether intraarticular abnormalities in osteoarthritis (OA) of the knee can be detected as well by needle arthroscopy as by standard arthroscopy. METHODS: Needle arthroscopy followed by standard arthroscopy was performed on 10 patients with knee OA (diagnosed according to American College of Rheumatology criteria) whose symptoms were not entirely attributable to the OA and were therefore an indication for further evaluation. Each knee was assessed for abnormalities of the menisci, articular cartilage (6 sites), and synovium (6 sites). RESULTS: Evaluation of the 18 menisci visualized with both techniques yielded the same results: 6 abnormal and 12 normal. Among the 54 articular cartilage sites evaluable with both procedures, 16 were judged normal by both needle arthroscopy and standard arthroscopy. Of the 38 cartilage sites judged abnormal by standard arthroscopy, 34 (89%) were abnormal by needle arthroscopy. Both techniques indicated cartilage changes were the same at 42 (78%) of the 54 sites; changes at the other 12 sites were 1 grade higher by standard arthroscopy than by needle arthroscopy. Both needle arthroscopy and standard arthroscopy revealed 51 evaluable sites in the synovium. Of 34 areas judged abnormal by standard arthroscopy, 24 (71%) were also judged abnormal by needle arthroscopy; 17 areas were judged normal by both techniques. The 2 techniques assigned the same macroscopic score in 27 (53%) of 51 areas of the synovium, with a higher grade by standard arthroscopy in all but 1 of the other 16 areas. CONCLUSION: These pilot data suggest that in knee OA, needle arthroscopy can 1) accurately detect meniscal abnormalities, 2) detect cartilage abnormalities, but may underestimate the severity, and 3) detect most synovial abnormalities, but often underestimates the severity. Needle arthroscopy is a potentially valuable rheumatologic tool for the assessment of OA of the knee.

Utility of needle muscle biopsy in a university rheumatology practice.

OBJECTIVE: To evaluate the utility of percutaneous needle muscle biopsy (NMB) as an alternative to open biopsy for the evaluation of patients with myopathy encountered in a university hospital based rheumatology setting. METHODS: Chart review of all patients (n = 30) who underwent NMB at our institution over a 4-year period. Patients were grouped according to clinical outcome. Records of 91 patients who underwent open biopsy during the same period were reviewed for comparison. RESULTS: NMB results either confirmed or added to the prebiopsy diagnostic suspicion in 11 patients and excluded a low prebiopsy suspicion in 13, thus yielding useful clinical information in 24 of 30 patients (80%). Biopsy left the suspected diagnosis unresolved more frequently in patients undergoing NMB compared to patients who had open biopsy during the same time period (6/30 vs 1/91). In the 6 cases unresolved by NMB, subsequent open biopsy added additional information in only 2 of 5 patients. Sensitivity of NMB for inflammatory myopathy was 83% in the 23 patients suspected of having this disease. NMB could be arranged twice as fast as open biopsy, and provided adequate tissue for evaluation in all cases. CONCLUSIONS: Our results suggest that NMB is an effective tool for obtaining useful diagnostic information, particularly pertaining to the presence of an inflammatory myopathy. The convenience, low morbidity, and sensitivity of NMB make it a procedure worthy of wider application by rheumatologists.

Muscle biopsy.

Muscle biopsy remains a critical component in the evaluation of myopathies. Recent literature demonstrates that percutaneous biopsy instruments can effectively procure muscle tissue for analysis in clinical and research settings. We review issues of muscle biopsy technique and specimen processing in the context of available clinical methods for tissue analysis. A survey of recent literature illustrates contributions made by the histologic, histochemical, and ultrastructural aspects of muscle analysis to our understanding of the pathophysiologic mechanisms and clinical manifestations of selected inflammatory, mitochondrial, and infectious myopathies.

Group G streptococcal arthritis and bowel disease: a rare enteropathic arthropathy.

Group G streptococci may be seen as normal flora in many parts of the body, including the gastrointestinal tract. They are rarely pathogens in humans, but they have been isolated from septic joints in debilitated patients. Three patients with group G streptococcal arthritis were further evaluated using contrast studies of the colon. Abnormalities, including an occult carcinoma and a colocutaneous fistula, were found. We conclude that group G streptococcal arthritis may be associated with gastrointestinal abnormalities that allow a portal of entry for an otherwise innocuous organism, and that this represents a rare enteropathic arthropathy.

Tidal irrigation versus conservative medical management in patients with osteoarthritis of the knee: a prospective randomized study. Tidal Irrigation Cooperating Group.

To determine the efficacy of tidal knee irrigation for knee pain due to osteoarthritis (OA), we conducted a randomized, single blind, 14-week prospective trial comparing medical management with tidal knee irrigation in 77 patients with non-end stage OA of the knee and unilateral pain refractory to standard medical treatment. Fifty-seven patients completed the study. Statistically significant differences (p less than 0.05) favoring tidal knee irrigation over medical management were pain after 50' walk, pain after 4-stair climb, most intense pain in previous day, frequency of knee stiffness with inactivity, days of morning knee stiffness in previous week, physician assessment of knee tenderness and overall assessments of therapy effectiveness by both patient and physician. Tidal knee irrigation results in more favorable improvement of pain due to OA than can be accomplished with traditional medical management.

Polymyositis evolving after rhabdomyolysis associated with HMG-CoA reductase inhibitors: a report of two cases.

3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors are commonly used for treatment of hyperlipidemia and its deleterious effects. Myotoxicity has been associated with use of these agents. We report two cases of inflammatory myopathy in patients receiving these agents that did not respond to drug withdrawal and required immunosuppressive treatment. One of these patients developed an antibody to histidyl tRNA synthetase or Jo-1, an autoantibody associated with idiopathic inflammatory myopathies. We suggest that HMG-CoA reductase inhibitor-associated myotoxicity may trigger an immune-mediated inflammatory myopathy. Patients whose muscle abnormalities do not resolve with drug withdrawal should be considered for muscle biopsy.

Case differences in the problem solving strategies of medical students and physicians.

Using a broad range of written patient management problems, this study examined 1) the influences of three medical information-gathering processes (history-taking, physical examination, and diagnostic studies) on the formulation of the differential and principal diagnosis by 175 medical students and 2) the extent to which the information-gathering processes used by the students paralleled the emphasis that experienced clinicians placed on these same processes. Results suggested that in 11 of 14 cases, the students appeared to be relying on specific information-gathering strategies in the formulation of the differential diagnosis, and that there were both similarities and differences in the relative emphases that these processes received from students and physicians.