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Objective.To describe the Israeli experience of treating adult-onset Still’s disease (AOSD) with tocilizumab (TCZ).Methods.Israeli rheumatologists who treated AOSD with TCZ filled in questionnaires on symptoms, number of tender and swollen joints, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and dosage of prednisone at initial TCZ administration, after 6 months, and at the end of followup.Results.Nine male and 6 female patients, aged 33 ± 12 years, mean disease duration 9 years (range: 1–25) were identified. They had used a mean of 3.6 disease-modifying drugs, including 10 patients with tumor necrosis factor blockers. Intravenous TCZ 8 mg/kg was administered every 4 weeks (12 patients) or every 2 weeks (3 patients). All patients completed at least 6 months of treatment. The mean followup period was 15.7 ± 9 months. At the onset of therapy, despite the use of prednisone (27.6 ± 26.3 mg/d), all patients reported joint pain. Fever was reported in 9 patients, rash in 7, pleuritis in 3, and hepatitis in 2 before TCZ use, with mean ESR and CRP levels of 60 ± 28 mm/h and 11.6 ± 15 mg/dl, respectively. After 6 months of treatment and at the end of followup, the number of tender and swollen joints, the ESR and CRP levels, and the prednisone dosage decreased significantly. Only 2 patients still complained of mild arthralgias, and none reported systemic symptoms at the end of followup.Conclusion.TCZ 8 mg/kg was extremely efficacious in treating adult patients with refractory Still’s disease. Both TCZ and interleukin 1 blockade should be considered in the treatment algorithm of AOSD. Randomized controlled studies are needed to validate these findings.

Objective.To characterize the clinical manifestations of patients with antiphospholipid antibody syndrome (APS) and pulmonary hemorrhage (PH).Methods.We performed a retrospective, single-center analysis of patients with APS who were followed up from 1980 to 2011. Of these patients, only those who fulfilled the Sydney criteria for APS were included. Patients with APS that manifested with PH were called the PHAPS group. The rest of the patients with APS served as controls. Clinical manifestations were compared between the PHAPS group and controls.Results.Sixty-three patients fulfilled the criteria for APS. Thirteen experienced PH and were included in the PHAPS group. Seventy-five percent of the patients with PHAPS and 22% of the controls had mitral valve disease (p = 0.001). Central nervous system (CNS) involvement (cerebrovascular accident, seizures) was present in 61% and 16% of the patients with PHAPS and controls, respectively (p = 0.001). Skin involvement (livedo reticularis, chronic leg ulcers) was present in 54% and 8% of the patients with PHAPS and controls (p = 0.001). Pregnancy morbidity occurred in 87.5% and 32.5% of the patients with PHAPS and controls (p = 0.005). Ninety-two percent and 83% of the patients with PHAPS had high-titer immunoglobulin γ (IgG) anticardiolipin and β2-glycoprotein I IgG antibodies compared to 43% and 30% of the controls (p = 0.002, p < 0.001, respectively).Conclusion.Patients with PHAPS were more likely than controls to have mitral valve disease, skin disease, CNS involvement, and pregnancy morbidity as well as high-titer APS. PHAPS seems to be a unique subgroup of all patients with APS.

Diffuse alveolar hemorrhage (DAH) is a rare manifestation of primary antiphospholipid antibody syndrome (APS). We describe a patient with primary APS and refractory recurrent episodes of DAH. The patient was admitted 15 times due to recurrent episodes of DAH in a period of 18 months. Multiple immunosuppressive drugs did not improve his condition. Two years after his presentation, he was treated with rituximab (two doses of 1 g, 2 weeks apart). Six months later, the attacks of DAH have gradually disappeared. In a follow-up of more than 2 years after he received rituximab, the patient has had no further admissions due to DAH. Levels of antiphospholipid antibodies were measured during follow-up of 4 years. Anti-β2 glycoprotein IgG titer decreased to normal 6 months after therapy but anticardiolipin (aCL) antibody titer increased. We conclude that rituximab caused a dramatic clinical response in this patient. Anti-β2 glycoprotein IgG correlated better with the clinical response in this patient than aCL.

S U N D A Y 410 Successful Omalizumab Treatment of a 6-Year Old Child with Severe Solar Urticaria Yuval Tal, MD, PhD, Zvi Dranitzki, MD, Meir Shalit, MD, FAAAAI, David Claes Enk, Assi Levi; Allergy and Clinical Immunology Unit, Department of Medicine, Hadassah Hebrew University Medical Center, Jerusalem, Israel, Department of Dermatology, Hadassah Hebrew University Medical Center, Jerusalem, Israel, Photodermatosis Clinic, Laser unit, Department of Dermatology, Rabin Medical Center., Petah-Tikva, Israel. RATIONALE: Solar urticaria is a rare form of physical urticaria usually occurring minutes after sun exposure. Angioedema might accompany the urticaria in severe cases. Treatment is difficult and often unsatisfactory. Here, we present a 6-year old child with severe solar urticaria and angioedema following short exposures to light despite continued combination therapy with high dose anti-histamines and leukotriene-receptor antagonists. As the child faced living in darkness unless an effective therapeutic remedy was found, we initiated omalizumab treatment with cautious dose elevation. METHODS: Action spectrum and minimal urticaria dose (MUD) were established by UVB (290-320 nm), UVA1 (340-400 nm) and visible light (400-760 nm) photoprovocation tests. Omalizumab treatment was initiated, with dose elevation every two weeks up to a complete remission dose of 300 mg, repeated every four weeks as maintenance. RESULTS: The action spectrum was established within the visible light spectrum with a MUD of 30 J/cm (unchanged, preceding and during nonbiological therapy). Omalizumab treatment was added, beginning with a dose of 75 mg. Partial response was noted at a dose of 150 mg, with some recurrence prior to the next dosing. At a maintenance dose of 300 mg every four weeks, the child remains in remission one year after initiation of treatment, with no urticaria episodes even following prolonged exposure to mid-day summer sun. Repeated photoprovocation with visible light was negative, validating the clinical remission. CONCLUSIONS: Solar urticaria and angioedema are both agonizing and life-threatening conditions. Treatment with omalizumab should be considered when high dose antihistamines and leukotriene-receptor antagonists fail.

Two siblings suffering from mental retardation, progressive bronchiectasis, extensive warts, and persistent hepatitis B are described. The propositus also had an unusual physiognomy and non-specific colitis. Both patients had a marked decrease in the population of CD4+ helper T cells.