William W. Ginsburg

Transient Regional Osteoporosis: A Study of 56 Cases and Review of the Literature

We describe the clinical features and course of disease in 56 patients (33 men and 23 women) with transient regional osteoporosis. The median age at hospitalization was 48 years. All patients had monoarticular or oligoarticular joint pain that was aggravated by weight bearing. Eighteen also had pain at rest in the involved joints and 6 had nocturnal pain. The joints of the lower extremities were affected in 54 patients; the hip was the most commonly affected joint. There was little evidence of synovitis. Osteoporosis was diagnosed on the basis of characteristic clinical findings and the results of roentgenograms and bone scans. The osteoporosis resolved spontaneously over several months. Treatment consisted of joint protection, initiation of gradual ambulation, and administration of nonsteroidal anti-inflammatory agents. Although its cause is unknown, osteoporosis appears to be a regional rather than a systemic process.

Osteoid osteoma: 95 cases and a review of the literature

Osteoid osteoma is a benign bone tumor occurring primarily in patients under the age of 30 yr. Bone pain at night and relief by aspirin or other nonsteroidal antiinflammatory agents is a common symptom complex. The proximal femur and spine are frequent sites of involvement, but almost any bone can be involved. If plain roentgenograms do not demonstrate the lesion, tomography or a bone scan may be helpful. Complete surgical excision is the therapy of choice with a low recurrence rate. Osteoid osteoma may present initially with symptoms suggestive of inflammatory arthritis, degenerative joint disease, neoplasm, or infection. This lesion can therefore be a difficult diagnostic problem, especially if routine roentgenograms are normal. A high index of suspicion is necessary to make the diagnosis.

Eosinophilic fasciitis: clinical characteristics and response to methotrexate

To describe our experience with 16 patients with eosinophilic fasciitis (EF) treated in our clinic over 14 years.

Visual recovery following Mycobacterium chelonae endophthalmitis.

Following uncomplicated cataract surgery, a patient receiving etanercept for psoriatic arthritis developed Mycobacterium chelonae endophthalmitis. Vitrectomy, capsulectomy, and intraocular lens removal was followed by intravitreal amikacin, topical gatifloxacin, intravenous imipenem, and oral clarithromycin for six months. The patient achieved a final corrected visual acuity of 20/20. Etanercept has been implicated in the development of numerous, severe granulomatous infections, though not previously with M. chelonae. This represents the first reported case of visual recovery following M. chelonae endophthalmitis.

Gaucher's disease and glioblastoma multiforme in two siblings: a clinicopathologic study.

The clinical and pathologic features of two brothers with the adult form of Gauchers disease, both of whom developed cerebral glioblastoma multiforme, are presented. Neither patient had a long-standing neurologic disorder, and morphologic evidence of nervous system glucocerebroside storage was not seen. Despite the known increased incidence of malignancy in Gauchers disease, a review of the literature and of sixty-two Mayo Clinic cases of adult onset Gauchers disease revealed only one other instance of the association with glioma. The significance of the relationship is unknown.

Late-onset peripheral joint disease in ankylosing spondylitis

We reviewed the records of 150 patients with definite ankylosing spondylitis who had significant peripheral arthritis and were seronegative and found 7 patients who had the onset of peripheral arthritis after their spinal disease became inactive. This late-onset peripheral arthritis may lead to significant joint deformity, and aggressive therapy may be warranted.

Tarsal coalition: An unusual cause of foot pain—Clinical spectrum and treatment in 129 patients

Abstract Tarsal coalition is an unusual cause of foot symptoms. The abnormal fusion of tarsal bones is caused by a congenital failure of mesenchyme to differentiate and form joints in the majority of cases. Symptoms from tarsal coalition generally present in the second decade of life when the congenital coalition ossifies and becomes immobile. Patients describe pain and stiffness of the foot and reduced subtalar motion, local tenderness, and pes planus may be found on examination. In the adolescent, symptoms from tarsal coalition are readily recognized, but the diagnosis is frequently delayed in older patients. This is possibly due to physician unawareness that coalition causes foot symptoms in older patients and to overlooking the coalition on plain film radiography. Tarsal coalition has an age-related distribution. The diagnosis should be considered in the patient with chronic foot pain, subtalar stiffness with or without a pes planus deformity.

Healing of erosive disease in multicentric reticulohistiocytosis.

To the Editor: Multicentric reticulohistiocytosis (MRH) is a rare multisystem disease reported to respond to various regimens including prednisone, methotrexate (MTX), cyclophosphamide, and other immunosuppressants. We previously described a 55-year-old man with MRH followed at our institution for 9 years. He responded to treatment with etanercept, leflunomide, and prednisone1. Over the past 5 years since that report, the patient has remained in remission, and followup radiographs demonstrated significant healing of erosions in his hands. Prior to the initial visit at our institution he had been treated with MTX, hydroxychloroquine, prednisone, and chlorambucil with prednisone, with no improvement after 4 months. He had also been treated with alendronate 70 mg/week for osteoporosis prophylaxis. At his initial evaluation, he was taking 40 mg/day prednisone and 25 mg/week MTX. He had active polyarthritis and multiple skin lesions. Cyclophosphamide was added at 2 mg/kg. He had gradual improvement of skin lesions and arthritis over 8 months, and prednisone was reduced to 20 mg/day. Cyclophosphamide was discontinued due to gross hematuria. Etanercept 25 mg …

Cryoglobulinemic vasculitis in a patient with CREST syndrome

Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynauds syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient.

AB0696 Hypereosinophilia and Disease Flare in Eosinophilic Fasciitis

Background Hypereosinophilia was found at the disease onset in up to 85% of patient with eosinophilic fasciitis. Objectives To describe our experience in patients with eosinophilic fasciitis (EF) and hypereosinophilia. To identify if hypereosinophilia is found with EF flare. Methods We retrospectively reviewed the charts of the patients with biopsy proven EF seen in our clinic. We identified patients with hypereosinophilia at the onset of disease and follow the eosinophils counts over 3 years (January 2012 to December 2014). Results 10 patients with biopsy proven EF and hypereosinophilia at the onset were found. Hypereosinophilia value ranged between: 980-4800 cells/mcl (Normal less 350 cells/mcl). The eosinophil count responded quickly to prednisone which was initiated in all 10 patients. Over 3 years of follow up 5 patients had 7 flares. Hypereosinophilia was identified in 3 patients with 4 flares. None of the patients were on Prednisone at the time of flares. Conclusions Hypereosinophilia can be associated with disease flare in some of EF patients found with high eosinophils count at the disease onset. References Shulman LE. Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians. 1975;88:70-86. Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB. Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases. Semin Arthritis Rheum. May 1988;17(4):221-231. Lebeaux D, Frances C, Barete S, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients. Rheumatology (Oxford). Mar 2012;51(3):557-561. Disclosure of Interest None declared