Eosinophilic fasciitis: clinical characteristics and response to methotrexate.

AIM: To describe our experience with 16 patients with eosinophilic fasciitis (EF) treated in our clinic over 14 years. METHODS: We retrospectively reviewed the charts of all patients with biopsy-proven EF. We collected data regarding demographics, clinical presentations, possible triggers, labs, imaging, treatment and response to therapy on follow-up. RESULTS: Eight women and eight men with a mean age of 52 years were included in the study. Three patients related the onset to prior strenuous exercise and one was exposed to vibratory machinery. Fourteen patients had a gradual onset and presented with induration of the skin. Two other patients presented with acute-onset and significant edema and weight gain. All patients required immunosuppressive therapy. Methotrexate (MTX) was used in all of our patients. The rate of complete remission was ~60%. Although the recurrence rate after stopping MTX was 70%, these patients responded well to re-treatment with MTX. CONCLUSION: We believe that MTX represents an effective treatment option for EF. The rarity of this disease would make a double-blind controlled trial study difficult to perform.

Tocilizumab, an effective treatment for relapsing giant cell arteritis.

OBJECTIVES: Patients with giant cell arteritis (GCA) often respond to corticosteroid (CS) therapy; however, the majority of patients relapse when CS therapy is tapered or withdrawn. The purpose of this study was to assess the efficacy of tocilizumab (TCZ) in patients with relapsing GCA. METHODS: Four patients with relapsing GCA received TCZ monthly (4mg/kg or 8mg/kg). Disease activity and drug tolerability were evaluated clinically and via laboratory test results at the beginning of the study and every 3 months until the publication of this study. All four patients were still receiving TCZ monthly at the time of manuscript submission. RESULTS: All four patients treated with TCZ achieved clinical and laboratory response. No adverse events were detected. CONCLUSIONS: In our small case series, TCZ was efficacious and well tolerated in patients with relapsing GCA. Proper randomised controlled trials are required to achieve confident conclusions regarding the safety and efficacy of TCZ in GCA.

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, Raynaud's 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 Raynaud's 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.

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.

Eosinophilic fasciitis: spectrum of MRI findings.

OBJECTIVE: Our objective was to describe the spectrum of MRI features of eosinophilic fasciitis. CONCLUSION: MRI findings in eosinophilic fasciitis are characteristic and consist of abnormal fascial signal intensity and enhancement, both of which are directly proportional to disease activity. MRI provides a useful aid for diagnosis and a marker for disease activity and response to treatment.

Jaw claudication in primary amyloidosis: unusual presentation of a rare disease.

We describe two patients with temporal artery biopsy-proven amyloidosis presenting with symptoms of jaw claudication, visual disturbance, and proximal muscle stiffness suggestive of giant cell arteritis (GCA) and polymyalgia rheumatica. At the onset of disease, neither patient had other characteristic symptoms to suggest primary amyloid. We point out similarities between GCA and primary amyloid that can lead to confusion in diagnosis.

Temporal artery bruits in a patient with giant cell arteritis.

Giant cell arteritis (GCA) is a disease of unknown etiology characterized by granulomatous inflammation of medium and large arteries. A 69-year-old man presented with right jaw claudication, intermittent scalp tenderness without headache, and visibly swollen temporal arteries. Results of a right temporal artery biopsy were positive for GCA. Auscultation revealed audible bruits of the temporal arteries. We believe this is the first reported example of bruits of the temporal arteries as a manifestation of GCA. The condition resolved with corticosteroid therapy.