Mirtazapine Therapy for a Patient With Weight Loss and Gastroparesis Associated With Limited Systemic Sclerosis.

Objective: Gastroparesis may be present in individuals with systemic sclerosis. In the United States, metoclopramide is the only medication approved for treating gastroparesis. Our case involves using mirtazapine therapy to help with weight loss and gastroparesis associated with systemic sclerosis. Case: A 70-year-old female with limited systemic sclerosis and sicca syndrome began experiencing weight loss, dysphagia, nausea, and abdominal fullness. Neither an esophageal dilation procedure nor six weeks of metoclopramide therapy alleviated her symptoms. However, 15 mg of mirtazapine once daily provided some symptomatic relief. A gastric emptying scan confirmed gastroparesis. The dose of mirtazapine was later increased to 30 mg once daily. With the mirtazapine therapy, the patient experienced both symptomatic improvement and weight gain benefits. Discussion/Conclusion: Mirtazapine therapy has anti depressive, appetite stimulating, anti-emetic, and prokinetic benefits. Consideration of mirtazapine therapy for patients with weight loss and gastroparesis associated with systemic sclerosis may be beneficial.

Disseminated Histoplasmosis Mimicking Macrophage Activation Syndrome in a Patient With Rheumatoid Arthritis.

Infections caused by Histoplasma capsulatum typically do not produce symptoms. However, in individuals who are immunocompromised, progressive disseminated histoplasmosis may occur. A 67-year-old female, with lengthy history of immunosuppression due to management of rheumatoid arthritis, reported a two-month history of fatigue, headaches, and intermittent fevers following hip surgery. Due to the concern for macrophage activation syndrome and hemophagocytic lymphohistiocytosis, a bone marrow biopsy was performed. However, the results indicated the presence of Histoplasma, which was supported by the presence of Histoplasma antigens in the serum and urine, Histoplasma antibodies in the serum, positive (1,3)-beta-D-glucan results, and fungal blood cultures. The patient initially received amphotericin B, but it was switched to itraconazole due to adverse effects. The patient remains on itraconazole therapy and follows as an outpatient with an infectious diseases specialist.

Systemic amyloidosis: unusual presentation mistaken for a recurrent scabies infection.

We report the case of a 63-year-old woman with a history of undifferentiated connective-tissue disease, polyarthritis, and bilateral carpal tunnel syndrome who presented with generalized pruritus and erythematous and excoriated papules on the trunk and extremities. Empiric scabies treatment was unsuccessful. Patch testing and T-cell receptor gene rearrangement studies were unremarkable. The patient was found to have mild interstitial lung disease and hypogammaglobulinemia. Eventually a diagnosis of primary systemic amyloidosis was made after she developed frank lingual hypertrophy despite normal initial serum protein electrophoresis and negative abdominal fat pad aspiration. Diagnosis was confirmed with lingual biopsy. This case demonstrates an unusual presentation of primary systemic amyloidosis consisting of arthritis and intense debilitating pruritus without primary skin lesions for a full year prior to diagnosis of multiple myeloma. The patient responded to treatment with chemotherapy and corticosteroids.

New and improved strategies for the treatment of gout.

The Western world appears to be in the midst of the third great gout epidemic of all time. In this century, gout is increasing in prevalence despite an increased understanding of its risk factors and pathophysiology, and the availability of reasonably effective treatment. The main cultural factors responsible for this appear to be diet, obesity, ethanol use and medications. Excess fructose consumption is a newly recognized modifiable risk factor. The debate has been renewed concerning hyperuricemia as an independent risk factor for renal insufficiency and cardiovascular disease. Prevention is still rooted in lifestyle choices. Existing treatments have proven to be unsatisfactory in many patients with comorbidities. New treatments are available today and on the horizon for tomorrow, which offer a better quality of life for gout sufferers. These include febuxostat, a nonpurine inhibitor of xanthine oxidase with a potentially better combination of efficacy and safety than allopurinol, and investigational inhibitors of URAT-1, an anion exchanger in the proximal tubule that is critical for uric acid homeostasis. New abortive treatments include interleukin-1 antagonists that can cut short the acute attack in 1 to 2 days in persons who cannot take nonsteroidal anti-inflammatory drugs, colchicine or corticosteroids. Lastly, newer formulations of uricase have the ability to dissolve destructive tophi over weeks or months in patients who cannot use currently available hypouricemic agents. Diagnostically, ultrasound and magnetic resonance imaging offer advanced ways to diagnose gout noninvasively, and just as importantly, a way to follow the progress of tophus dissolution. The close association of hyperuricemia with metabolic syndrome, hypertension and renal insufficiency ensures that nephrologists will see increasing numbers of gout-afflicted patients.

Scleromyxedema.

Scleromyxedema is a rare cutaneous mucinous disease characterized by a generalized papular sclerodermoid eruption and systemic manifestations that can lead to significant morbidity and mortality. Although its etiology remains unknown, most theories focus on a pathogenic role by paraproteins; it must be noted, however, that nonparaprotein factors have been suggested to cause fibroblast proliferation and increased mucin production. Several treatment modalities including melphalan, cyclophosphamide, interferon alfa, and plasmapheresis have been suggested; however, further research is needed to prove treatment efficacy.

Metabolic diseases: gout.

Gout is a disease of antiquity but is increasing once again in prevalence despite availability of reasonably effective treatments. This may be related to a combination of factors, including diet, obesity, and diuretic use. Allergic reactions, noncompliance, drug interactions, and sometimes inefficacy all limit the effective use of current hypouricemic agents. There are new treatments for gout on the horizon, including febuxostat, a nonpurine inhibitor of xanthine oxidase with a potentially better combination of efficacy and side effects than allopurinol. Diagnostic progress is being made in that ultrasound may offer a noninvasive means of diagnosing tophaceous deposits in and around joints. The increasing prevalence of gout means that dermatologists will see more cutaneous manifestations of gout, including tophi, draining sinus tracts, panniculitis, and dystrophic calcifications.

Henoch-Schönlein purpura after etanercept therapy for psoriasis.

Etanercept is a recombinant dimeric fusion protein consisting of a tumor necrosis factor-alpha receptor ligand-binding region linked to the Fc portion of human IgG. It is approved for use in the treatment of rheumatoid arthritis, ankylosing spondylitis, juvenile rheumatoid arthritis, psoriasis, and psoriatic arthritis. Since 1998, there have been reports of vasculitic adverse events, including necrotizing vasculitis and leukocytoclastic vasculitis. In addition, the adverse events reporting system of the U.S. Food and Drug Administration has recorded 35 cases of leukocytoclastic vasculitis, 20 after etanercept therapy and 15 after infliximab. Most cases of cutaneous vasculitis describe development of symptoms within 3 months of etanercept use. In only one case report was direct immunofluorescence performed on tissue and no specific immunoreactivity found. We describe the first case of Henoch-Schönlein purpura with acute renal failure associated with increase in etanercept dose after 11 months of use for treatment of psoriasis. Discontinuation of the drug and treatment with a course of systemic steroids led to the complete resolution of the vasculitis and improvement of renal function. Vasculitis occurring even during chronic use of antitumor necrosis factor agents must be considered as possibly related to the therapy.

Further validation of a questionnaire to identify women likely to have low bone density.

UNLABELLED: A questionnaire instrument called the Simple Calculated Osteoporosis Risk Estimation (SCORE*) stratifies risk for osteoporosis, potentially reducing population-screening costs. SCORE is calculated using race, weight, age, history of estrogen use, fracture history, and presence/absence of rheumatoid arthritis. We tested SCORE in 912 postmenopausal women aged 45 yr or more using a Hologic QDR4500C densitometer for the total hip, femoral neck, and lumbar spine. National Health and Nutrition Examination Survey (NHANES III) norms were used to calculate hip T-scores. Low bone density (T-score < -2.0) was found in 29.6% of patients at the femoral neck, 17.7% at the total hip, 36.1% at the lumbar spine, and 46.1% at one or more sites. The sensitivity/specificity of the SCORE model (using a threshold of 6 points) was 0.97/0.36 for the femoral neck, 0.95/0.30 for the total hip, 0.86/0.35 for the spine, and 0.88/0.41 for any site (total hip, femoral neck, or spine). When used to detect low bone density at any site, SCORE would have deferred 27.6% of women referred for DXA scans, but 20.7% of these (5.7% of the entire population) would have been false-negatives, and thus inappropriately deferred. At a cutpoint of 3 instead of 6, sensitivity/specificity was 0.96/0.16. In those aged 50-60, the group with the greatest need for risk stratification, sensitivity/specificity for low density at any site was 0.72/0.54, and 46.1% would have been deferred, but 18.5% of this group would have been false-negatives. A cutpoint of 1 in this age group yielded sensitivity/specificity of 0.94/0.16. After age 65, few women would be deferred. CONCLUSION: When used to detect low bone density at any site with sufficient sensitivity for clinical practice, SCORE did not have sufficient discriminatory power to be broadly applicable.

A study of musculoskeletal manifestations in 12 patients with SAPHO syndrome.

Synovium and synovial fluid findings in SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome have not been well characterized, and only a few patients have been described in the Americas. We describe clinical, pathologic, and synovial fluid findings in 12 patients with the SAPHO syndrome: hidradenitis suppurativa (7), acne fulminans or conglobata (3), acneiform folliculitis (1) and palmoplantar pustulosis (1). Routine synovial fluid studies were performed in 6 patients, and light and transmission electron microscopic studies were performed in synovium in 2 patients and in bone in 1. The most common musculoskeletal manifestations included erosive or non-erosive oligoarthritis involving metacarpal phalangeal (MCP) and metatarsal phalangeal (MTP) joints as seen in 9 patients, sclerosis of the sacroiliac joints as seen in 5 patients, and osteitis pubis as seen in 1. Three patients had signs of skeletal hyperostosis. The patients with acne fulminans and acneiform folliculitis had chronic aseptic multifocal osteomyelitis. Synovial fluid was sterile in 7, mildly inflammatory in 5, and highly inflammatory in 2. Electron microscopic studies of synovium in 2 patients and of bone in 1 were not useful to detect microorganisms. Three African-American patients with hidradenitis suppurativa presented with pyoderma gangrenosum, and 2 of them had leukocytoclastic vasculitis, and a life threatening course unresponsive to antibiotics, corticosteroids and immunosuppressive therapy. SAPHO in the Americas is most severe in African-Americans with hidradenitis suppurativa, and it presents with heterogeneous musculoskeletal and cutaneous manifestations including erosive polyarthritis or oligoarthritis with nonspecific mild inflammatory fluid. Leukocytoclastic vasculitis and recalcitrant pyoderma gangrenosum were seen in 2 of our patients with the most severe hidradenitis suppurativa. SAPHO syndrome may present with clinical manifestations similar to those seen with seronegative spondyloarthropathies, but it has distinctive cutaneous, radiographic articular, and bone manifestations. Sites of chronic infection need aggressive antibiotic therapy and may need surgical resection.