Florentina Berianu

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.

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.

Systemic Lupus Erythematosus Presenting with Alveolar Hemorrhage

Introduction Diffuse alveolar hemorrhage is a rare presentation of systemic lupus erythematosus. Early diagnosis and appropriate treatment can improve outcome. Case Report An 18-year-old male presented with hemoptysis and respiratory distress requiring orotracheal intubation. Laboratory tests showed positive anti-nuclear antibody and anti-dsDNA and low C3 and C4. Bronchoalveolar lavage became progressively hemorrhagic after each aliquot. He was treated with pulse methylprednisolone, cyclophosphamide, and plasma exchanges. Discussion Alveolar hemorrhage is a rare initial presentation of lupus, with mortality rates reported at about 50%. Lupus should be considered in those presenting with alveolar hemorrhage since delay in therapy may cause a rapid deterioration of the patient. The diagnosis of SLE is illusive when DAH is the presenting symptom. Since early diagnosis and appropriate institution of treatment improve outcome, it is important to keep lupus in mind as an etiology of alveolar hemorrhage. Pulse methylprednisolone, cyclophosphamide, and plasmapheresis therapy resulted in rapid improvement of respiratory function in our patient.

More than meets the eye.

trauma. On physical examination and musculoskeletal ultrasound, there were no signs of synovitis involving her proximal interphalangeal or metacarpophalangeal joint of the affected index finger, but it was extremely painful to palpation and warm to touch. No other findings to suggest an RA flare were noted. No redness or fluid collection was appreciated, but mild swelling was seen compared with the contralateral index (Fig. 1).

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

Destructive Arthritis of the Hand After Diving

Destructive infectious arthritis can result after joint impact, even without obvious trauma. A previously healthy 27-year-old woman woke up with extreme pain, redness, and swelling in her left hand. Redness and swelling had progressed rapidly from the red line on the first day to the blue line on the second day, and extended farther the next day (Figure 1A). She had no fever, but …