Calcium apatite crystals in synovial fluid rice bodies.

BACKGROUND: Rice bodies can occur in the joints in many rheumatic conditions, but they are most common in rheumatoid arthritis. They are generally believed to occur rarely in patients with osteoarthritis, but one study reported rice bodies with apatite crystals. OBJECTIVE: To report on a series of joint fluids with rice bodies containing apatite clumps and examine their clinical pictures. METHODS: All synovial fluid analysis reports for 10 years were reviewed for rice bodies and eight patients were reported on. A series of patients with a variety of diseases with synovial fluid rice bodies found to contain calcific material is described. All were examined by compensated polarised light and alizarin red stain, and four were examined by electron microscopy. RESULTS: The eight patients all had alizarin red S chunks embedded throughout the rice body. Transmission electron microscopy disclosed the presence of a matrix of collagen, fibrin, and amorphous materials containing typical apatite crystals. Clinical diagnoses, radiographic findings, and leucocyte counts varied, but six of the eight patients had had previous repeated corticosteroid injections into the joints. CONCLUSION: Aggregates of apatites may be more common than previously recognised in rice bodies as they are not routinely sought. Whether they are a result of joint damage or depot steroid injections and whether that might contribute to further joint injury now needs to be investigated.

Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature.

OBJECTIVE: To give a comprehensive review of transverse myelopathy (TM), a rare but serious condition reported in 1-2% of patients with systemic lupus erythematosus (SLE). METHODS: 14 patients with SLE and TM were evaluated and 91 additional cases published in the English and German literature reviewed. RESULTS: TM presented either as the initial manifestation or within five years of the diagnosis of SLE. Most patients presented with a detectable sensory deficit at the thoracic level. In our 14 patients, 22% of the patients showed complete neurological recovery, whereas in the total patient population of 105 (our cases plus those reviewed in the literature), complete recovery was observed in 50%, partial recovery in 29% and no improvement or deterioration in 21%. Treatment with intravenous methylprednisolone followed by cyclophosphamide seemed to be most effective. Seventy per cent of the total patient population had abnormal magnetic resonance imaging findings. In our group of 14 patients, those with higher disease activity (measured by the SLAM) at onset of TM were treated more aggressively (for example, with plasmapheresis and intravenous pulse cyclophosphamide). TM in our patients was associated with antiphospholipid antibodies in 43% of the cases as compared with 64% of the total patient population. Optic neuritis occurred in 48% of the total patient population with SLE and TM, suggesting an association. CONCLUSIONS: TM in SLE is a poorly understood entity. Outcome might be more favourable than previously suggested. There is an association of TM with antiphospholipid antibodies in SLE patients. Treatment including intravenous cyclophosphamide may improve the final outcome. This report emphasises the need for multicentre trials to establish guidelines for optimal treatment.

Treatment of acquired factor VIII inhibitor using intravenous immunoglobulin in two patients with systemic lupus erythematosus.

Acquired inhibitors of factor VIII have rarely been reported in the presence of systemic lupus erythematosus (SLE). Treatment is usually corticosteroids, with or without immunosuppressive agents such as cyclophosphamide or azathioprine. We report here case histories of 2 patients, one with documented SLE, the other with a forme fruste of SLE. Both patients had inadequate responses to corticosteroids and immunosuppressive agents. Both responded to intravenous immunoglobulin, with a decrease in their titers of factor VIII inhibitor and significantly decreased frequency of bleeding episodes despite persistent low-level inhibitor titers. Better control of their SLE symptoms and findings was also observed. Previously reported treatments of acquired factor VIII inhibitors in SLE are discussed.

Acute rheumatic fever.

During the first half of this century rheumatic fever was a common disease with significant morbidity and mortality in the United States. In the 1980s, when many clinicians were hoping this disease was a disease of the past, anxieties were renewed when outbreaks were reported in several areas around the country. Although the etiology still eludes us, insight has been gained. Environmental and genetic factors are believed to play a role in the epidemiology of this disease. Additionally, the implicated organism, the group A streptococcus, has many strains, and differences in its many proteins may determine their potential for rheumatic fever. The mechanisms leading to disease are not clear, but the streptococcus has been implicated as a source of antigens with cross-reactivity with human tissues and has been shown to modify immune mechanisms. Clinical aspects are briefly reviewed and physicians are reminded to consider rheumatic fever as a diagnostic possibility in the appropriate settings.

Arthritis problem indicator: preliminary report on a new tool for use in the primary care setting.

Improving the quality of life for people with rheumatic disease involves timely identification of problem areas and application of appropriate interventions. In response to a 1987 Commonwealth of Pennsylvania study, which reported a wide variety of unmet needs in arthritis patients and their families, the Arthritis Problem Indicator (API) was developed. It is a single-page, self-report, low-cost tool. A mixed rheumatology population (n = 50) and their primary care physicians participated in a pilot study. The study revealed that the seven most common areas of patient concern were pain, weight control, sleep, mobility/walking, activities of daily living, community access, and depression/anxiety. The physicians reported that the patient's answers on the API led them to initiate new treatment or referral for 32% of the patients. The physicians also stated that for 80% of the patients, the API was helpful in providing information about the patient. The API is easily interpreted by health professionals and designed to be an indicator of problem areas frequently associated with arthritis.

Renal vascular lesions in systemic lupus erythematosus.

In the past, necrotizing vasculitis has been considered to be one of the dominant intrarenal vascular abnormalities in systemic lupus erythematosus (SLE). To test the validity of this statement, 70 consecutive renal biopsies from patients with SLE were reviewed. Light microscopy (LM) and immunofluorescence (IF) studies documented abnormalities, including thrombosis and nephrosclerosis, in 30 patients (43 percent), but no cellular infiltration of the vessel walls or other evidence of acute necrotizing vasculitis was seen. It is concluded that while intrarenal vasculopathy with thrombosis and nephrosclerosis is a common finding in SLE, our data and recently published studies suggest that acute necrotizing vasculitis occurs rarely, if at all, in SLE nephritis.

Methotrexate in the treatment of rheumatoid arthritis.

Methotrexate is an effective agent for the treatment of rheumatoid arthritis. It is now widely prescribed for patients who have not tolerated or responded to gold compounds or penicillamine. Minor adverse reactions are common, and fatal pulmonary toxicity or cirrhosis can occur. The drug does not produce disease remissions, but continued administration helps reduce pain, stiffness and swelling. Within the past year, the Food and Drug Administration has approved methotrexate for use in treating rheumatoid arthritis.

Acute visual loss in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) often presents as a multisystem disease that can be difficult to diagnose. Although ocular symptoms are infrequent, actual acute visual loss has been reported. A review of four cases of acute visual loss from a lupus clinic revealed that two patients had visual loss as a presenting sign of SLE. One had bilateral occipital lobe infarctions, the other multiple cotton wool spots and an attenuated retinal vascular system. Of the two patients with documented SLE prior to the onset of visual problems, one presented with a coincidental retinal tear and the other with retinal phlebitis.

Systemic lupus erythematosus presenting with lingual infarction.

A 73-year-old woman developed a lingual infarction, Raynaud's phenomenon, cutaneous lesions, pancytopenia, and pleuritis. Serologic studies supported a diagnosis of systemic lupus erythematosus (SLE), and anticardiolipin antibodies were detected in significant titers. This is the first reported case of SLE presenting with an infarction of the tongue.

Aggressive lesion in osseous sarcoidosis.

We describe an unusual aggressive lytic bone lesion with "sunburst/hair-on-end" periosteal reaction in a patient with surgically proven osseous sarcoid and review the pertinent literature.

Lymphocytotoxic antibodies.

LCTA are a heterogenous group of antibodies found in many disease states. The recurring observation is that they are able to modulate immune function in vitro. The exact mechanism of this modulation is unknown, however, alteration of cell surface antigens appears to be important. Recent advances in molecular biological techniques have made the surface antigen, against which LCTA is directed, more accessible. Once the surface antigen is identified, the exact role of LCTA may then be elucidated. Endothelial cell damage is a recurring pathologic finding in many of the diseases discussed. Again, identification of the antigen and its similarity to antigens on various cell types may help explain the immunopathogenesis of the different disease processes.

Rheumatology training at internal medicine and family practice residency programs.

The Medical Research Education Subcommittee of the American Rheumatism Association surveyed a random selection of large and small programs in internal medicine and family practice residency programs in order to evaluate their rheumatology training. Formal rheumatology training is offered in 90% of these residency programs, but many available positions are not being filled. A full-time staff rheumatologist was present at 69% of large internal medicine programs, 32% of small internal medicine programs, and 11% of family practice programs. The methods of rheumatology training are similar in most programs, although small internal medicine programs and family practice programs more often utilize physicians' offices or outside medical centers for the rheumatology elective training. A majority of the directors of these residency programs thought that many basic skills and techniques were not taught adequately and that the training of their rheumatology residents was not equal to that of residents in cardiology or gastroenterology.

Medically reversible quadriparesis in tophaceous gout.

This case report presents a successful nonoperative outcome of a type II odontoid fracture secondary to severe tophaceous gout. The patient presented with spastic quadriparesis and tophaceous involvement of the odontoid process with fracture and C1-C2 instability. He refused surgery. However, his odontoid process stabilized with the used of a collar, and he regained almost normal strength plus independence in self-care and ambulation, demonstrating that an unstable type II odontoid fracture secondary to tophaceous gout can stabilize with conservative management.

Presence of complement-fixing anti-endothelial cell antibodies in systemic lupus erythematosus.

Vasculitis in systemic lupus erythematosus (SLE) is associated with the deposition of IgG and complement in blood vessel walls. However, it is not known whether immune injury to endothelial cells is a part of this process. Therefore, we used a solid phase radioimmunoassay to study the ability of IgG from normal human sera and sera from patients with SLE to bind to endothelial cells. In this assay, cultured human umbilical venous endothelial cells were sequentially incubated with normal or SLE sera, goat anti-human IgG, and 125I-labeled staphylococcal protein A (*SPA). After exposure to normal sera, 2.5 +/- 0.5% (mean +/- SD) of the added *SPA bound to the cells, whereas after exposure to SLE sera 13.8 +/- 7.6% of the added *SPA bound to these cells. This difference in binding was highly significant (P less than 0.001). Binding was partially reduced when SLE sera were preincubated with B-lymphocytes or monocytes, but not after exposure to erythrocytes, platelets, or T lymphocytes. Incubation of endothelial cells with the 7S fraction of SLE sera or with the F(ab')2 fragment of SLE-IgG resulted in the deposition of greater than 80% as much IgG as was deposited on endothelial cells by whole serum. However, since higher molecular weight fractions (greater than 7S) of SLE sera were also active, we tested the capacity of endothelial cells to bind IgG complexes. Endothelial cells bound heat-aggregated IgG (HA-IgG) in a saturable manner at one log concentration below the binding of normal monomeric IgG. Binding of HA-IgG to endothelial cells was markedly enhanced by preincubation with a serum source of complement. Both HA-IgG and SLE-IgG also bound to freshly obtained endothelial cells in suspension, as detected by automated fluorescence flow cytometry. Binding of SLE-IgG and HA-IgG to endothelium initiated complement activation, deposition of the third component of complement, and disruption of the monolayer. In addition, SLE-IgG and HA-IgG caused endothelial cells to secrete prostacyclin and caused the adherence of platelets, confirmed by scanning electron microscopy. These studies demonstrate that IgG anti-endothelial antibodies are present in the sera of patients with active SLE. These sera may also contain IgG complexes that are capable of binding to endothelial cells. The association of IgG and complement with endothelial cells may initiate vascular injury in SLE and other human disorders.

Cellular immune function in rheumatic disease.

Investigation of the cellular immune function in patients with rheumatic diseases is important in elucidating the pathogenesis of the disease processes and in determining the associated abnormalities of recognition and regulation exerted by the immune system. However, because of the lack of specificity and the variations noted from laboratory to laboratory, tests of cellular immune function are, at present, of little value in the laboratory diagnosis of these diseases. The abnormalities found in the rheumatic diseases occur with many autoimmune diseases and other inflammatory states. The common pathway of immune abnormalities appears to be influenced by several factors. They include several genetic loci, possible environmental factors, and immunologic mechanisms, which appear to interact in an intimate way to induce various autoimmune diseases.

Lymphocyte subsets in patients with systemic lupus erythematosus.

The role of abnormal numbers and function of lymphocyte subsets in SLE remains, to date, unclear. Evidence exists for both functional T lymphocyte and B lymphocyte defects although the data for T lymphocyte defects appear more compelling. The role of autoantibodies on these cellular defects is also unclear. They may take on an important pathophysiologic role in depletion of certain lymphocyte subsets with subsequent alteration of function or they may, in fact, be interesting epiphenomena since an inverse relationship of depressed T lymphocyte numbers and function to increased levels of LCTA are frequent. Many other factors clearly influence lymphocyte function and disease expression including genetics and endocrine factors. As methodologies improve, a clearer understanding of the pathogenesis of SLE will emerge.