Faculty Bibliography

Headaches are a common, but under-recognized and understudied symptom in the context of the rheumatic diseases. They can result from intracranial pathology, such as parenchymal and meningeal inflammation, thrombosis, space-occupying lesions, and more. Inflammation, irritation, or degeneration of anatomically related structures such as the eyes, neck, and sinuses can equally cause headaches. In addition, patients with rheumatologic disorders have the same tendencies as the general population to develop primary headaches. While the latter are benign in nature, and generally require only symptomatic relief, the former type of headaches may signal disease manifestation, progression, or complication. Thus, familiarity with common and uncommon headache syndromes related to rheumatologic disorders as well as with their possible underlying disease processes and mechanisms is important. This will help to successfully develop an effective approach toward the evaluation of patients presenting with headaches in a rheumatologic context, and, ultimately, diagnose and treat potentially severe underlying disease.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology in which the normal immune responses are directed against healthy organs and tissues. The disregulated immune system produces antibodies that attack the skin, joints, kidneys, heart, and brain. Some people experience mild rashes and arthritis, others suffer debilitating fever, fatigue, joint pain, and severe organ and/or life-threatening disease. This article provides a medical overview of the epidemiology of SLE, the challenges of diagnosing SLE, the complexity of the clinical manifestations and treatment issues, and the impact of SLE on patients' lives. We also discuss the progress in understanding the disease and its therapy over the last century.

Many drugs can cause myopathies, and such myopathies may range widely from asymptomatic elevations in the serum creatine phosphokinase levels to severe myalgias, cramps, exercise intolerance, muscle weakness, and even rhabdomyolysis. In this article, some of the commonly used drugs that may induce myopathies, as well as the clinical phenotypes, diagnosis, and management of these syndromes are reviewed

The most common drugs currently in use that may cause myopathies were reviewed using the Medline database (U.S. National Library of Medicine, Bethesda, Maryland). Our review included results from epidemiologic and database surveys, clinical trials, and case reports. The clinical spectrum is wide, and presentations range from asymptomatic elevations in serum creatine phosphokinase levels to severe life-threatening rhabdomyolysis. Management of suspected drug-induced myopathy should include immediate discontinuation of the offending agent, as well as supportive care when needed. Earlier diagnosis and drug discontinuation raises the likelihood of resolution and recovery

OBJECTIVES: All pharmacologic agents have the potential for both benefit and toxicity. Among the more interesting and important adverse consequences of drug therapy are a range of joint and connective tissue complaints that may mimic or reproduce primary rheumatologic diseases. In this article, we review the literature on commonly used drugs reported to induce arthritis and/or connective tissue-based diseases. We assess the strength of the reported associations, discuss diagnostic features and treatment implications, and consider possible mechanisms for drug-induced genesis of rheumatic conditions. METHODS: We reviewed the Medline database from 1987 to 2006 to identify drug-induced arthritic and connective-tissue disease syndromes, utilizing 48 search terms. A qualitative review was performed after the articles were abstracted and the relevant information was organized. RESULTS: Three hundred fifty-seven articles of possible relevance were identified. Two hundred eleven publications were included in the final analysis (case series and reports, clinical trials, and reviews). Many drugs were identified as mimicking existing rheumatic conditions, including both well-established small molecules (eg, sulfasalazine) and recently introduced biologic agents (eg, antitumor necrosis factor agents). The most commonly reported drug-induced rheumatic conditions were lupus-like syndromes. Arthritis and vasculitis were also often reported. CONCLUSIONS: Drug-induced rheumatic syndromes are manifold and offer the clinician an opportunity to define an illness that may remit with discontinuation of the offending agent. Early diagnosis and withdrawal of the drug may prevent unnecessary morbidity and disability

Septic arthritis is a commonly reported complication of rheumatoid arthritis (RA). Tumor necrosis factor alpha (TNF-alpha) plays an important role in host defense against infection. Inhibition of its activity could therefore be anticipated to augment the risk of infection. Both opportunistic and bacterial infections have been described in patients with RA treated with anti-TNF-alpha therapy. We describe a patient who experienced 2 episodes of septic arthritis. Both occurred while the patient was on etanercept. Recurrence developed despite prolonged parenteral antibiotic. To our knowledge, this is the first report of relapsing oligoarticular methicillin-sensitive Staphylococcus aureus septic arthritis despite prolonged antibiotic treatment in a patient receiving etanercept therapy. Our case underscores the advisability of discontinuing TNF-alpha blockade in patients with septic arthritis during prolonged antimicrobial therapy

OBJECTIVE: To describe a case of postirradiation morphea and subcutaneous polyarteritis nodosa occurring simultaneously in a patient and to review the literature on postirradiation autoimmune phenomenon and the potential pathogenesis of such changes. METHODS: A 75-year-old woman with breast cancer treated with chemotherapy and radiation who developed postirradiation morphea and subcutaneous polyarteritis nodosa, both inside and outside of the field of radiation, is described. Literature searches were performed on postirradiation morphea and other radiation-related inflammatory cutaneous conditions and the potential pathogenic mechanisms involved. RESULTS: Twenty-five cases of postirradiation morphea and 8 cases of postirradiation panniculitis were reported in the literature. Only 3 cases of morphea with distant vasculitis occurring in the same patient have been reported and each of these patients had features suggestive of an underlying connective tissue disease. This is the first case of morphea and subcutaneous polyarteritis nodosa occurring in the same location both inside and outside the field of radiation. CONCLUSIONS: Postirradiation morphea is an uncommon condition but is being increasingly recognized. Related phenomena following radiation include postirradiation panniculitis and now postirradiation subcutaneous polyarteritis nodosa. Radiation may be responsible for inducing some of the pathogenic changes seen in scleroderma and other autoimmune diseases. Rheumatologists should be aware of these potential complications of radiation treatment

BACKGROUND: Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater and has been associated with rheumatoid arthritis, syphilis, Wegener's granulomatosis, tuberculosis, and cancer. Few series of the idiopathic variety have been described, particularly with respect to MRI correlation to clinical outcome and treatment. OBJECTIVE: To investigate the clinical and laboratory evaluation, course, and treatment of patients with idiopathic hypertrophic pachymeningitis (IHP), to correlate the MRI findings with the clinical course, and to review the literature on IHP. METHODS: Retrospective case series of 12 patients (9 men, 3 women), with a mean age of 55 years (range 39 to 88 years), who had IHP by imaging studies, meningeal or orbital biopsy, or both. The clinical features, laboratory evaluation, contrast-enhanced MRI, treatment, and clinical outcome were documented for each case. The mean duration of follow-up was 3.5 years (range 3 months to 16 years). RESULTS: The main clinical features at presentation were headache (11 cases), loss of vision (7 cases), diplopia (4 cases), papilledema (2 cases), other cranial nerve involvement (3 cases), ataxia (2 cases), and seizures (1 case). On the initial MRI, the location of abnormal enhancement of the dura mater correlated with the clinical findings and the sphenoid wing area was affected in all patients. The sedimentation rate was elevated in five cases. The CSF had increased protein in six cases and lymphocytosis in four cases. Biopsy of the dura mater in five cases and the orbital soft tissue in one case showed infiltrates of small mature lymphocytes, plasma cells, and epithelioid histiocytes, but no neoplasia, vasculitis, or infectious agents. Cultures of the CSF and biopsy material remained sterile. Corticosteroid therapy improved the vision in 7 of 8 cases and controlled headache in 10 of 11 cases. Five cases had partial improvement of other neurologic symptoms and signs. Recurrence developed with steroid tapering in six cases. One case had progressive deterioration and died. In four cases methotrexate or azathioprine was added with reduction of the steroid dose. Follow-up MRI performed in 11 patients correlated 80% with the clinical state (p = 0.01). CONCLUSION: IHP can be suspected on MRI and defined pathologically on biopsy. Untreated, the clinical course is usually marked by severe headache and progressive neurologic deterioration and vision loss. Although initially steroid-responsive, clinical manifestations frequently recur with corticosteroid taper, requiring the addition of immunosuppressive agents in some cases

The objective of this study was to define clinical and imaging characteristics of periaortitis prior to and after therapy with immunosuppressive drugs. Four consecutive patients with periaortitis (two secondary to atherosclerosis and two with rheumatic diseases) were studied with contrast-enhanced CT and magnetic resonance angiography (MRA), rheumatologic serologies, and acute-phase reactants. All were treated with corticosteroids and two patients received immunosuppressive agents. Patients were followed with serial MRA scans, CT scans, and clinical exams. Prior to treatment, all patients demonstrated a rind of periaortic tissue, which was enhanced with both contrast-enhanced CT as well as gadolinium-enhanced MRA. Clinical symptoms resolved and rind contracture occurred in all cases following therapy. Enhancement of the rind persisted despite the clinical improvement in all patients. No patient developed an aortic aneurysm or retroperitoneal fibrosis during the follow-up period. Corticosteroid/immunosuppressive treatment was continued for an average of 41 months. At 62 months of total follow-up, there has been no recurrence of periaortitis by clinical and/or radiologic exam. Treatment of periaortitis with corticosteroids and immunosuppression therapy leads to resolution of clinical symptoms and radiologic contracture of the periaortic rind. Patients responded to therapy without developing progressive fibrosis or aneurysm. MRA allows safe and repetitive imaging of periaortitis and provides excellent definition of lumenal abnormalities including plaque rupture