Favourable outcome of planned pregnancies in systemic sclerosis patients during stable disease.

OBJECTIVE: Studies evaluating pregnancy outcomes in systemic sclerosis (SSc) are limited. SSc is associated with maternal complications and adverse neonatal outcomes. This study investigated the impact of disease stage (stable vs active) on the maternal and neonatal outcomes of pregnancies of patients followed at an Israeli medical centre. METHOD: The charts of 354 SSc female patients followed during 2003-2020 were reviewed. Data on clinical and laboratory features, number of pregnancies close to SSc diagnosis, and maternal and neonatal outcomes were analysed. Patients were divided into a stable disease and an active/early disease group. RESULTS: The active/early disease group included 26 patients [19 diffuse SSc (dSSc)], with 38 pregnancies. Median disease duration was 1 year, except for four patients who were first diagnosed during pregnancy. SSc was exacerbated in all patients during pregnancy or shortly after delivery [skin, lung, and heart involvement in dSSc; severe vasculopathy in limited SSc patients]. Six patients had hypertensive disorders of pregnancy; four pregnancies ended with foetal death. Thirty-three children were born, 60% with intrauterine growth retardation (IUGR)/low birthweight (LBW). The stable disease group included 19 patients, including seven with previously active disease, now stabilized (five dSSc), and 32 pregnancies. All pregnancies were planned and monitored closely. Disease remained stable in all patients. Four patients had hypertensive disorders of pregnancy; 12/29 newborns had LBW (41%). CONCLUSION: Active maternal disease during pregnancy poses an increased risk of SSc aggravation. The maternal and neonatal outcomes in planned pregnancy during stable disease are favourable. IUGR/LBW is common among neonates, even in stable disease.

Herpes simplex hepatitis after liver transplantation: case report and literature review.

Herpes simplex virus (HSV) hepatitis is an uncommon cause of liver failure, but may have a dramatic outcome. We herein present a case report of a liver graft infection by HSV-1 associated with liver failure and encephalitis. A complete hospital chart review of the case and a literature search were undertaken. Literature review suggests that herpes simplex acute liver failure is rare and associated with a poor prognosis, even with early treatment. Novel diagnostic and preventive approaches need to be instituted.

Vitamin D level: is it related to disease activity in inflammatory joint disease?

The objectives of this study are to assess the vitamin D status in patients (pts) with inflammatory joint diseases (IJD), and its correlation with disease activity. 121 consecutive pts (85 rheumatoid arthritis (RA), 22 psoriatic arthritis (PSA), 14 ankylosing spondylitis (AS)) underwent clinical and laboratory evaluation which included kidney and liver function tests, serum calcium and phosphor levels, 25(OH)D and parathyroid hormone (PTH). Disease activity was assessed by DAS 28 in RA and PSA pts and by BASDAI in AS pts, sedimentation rate (ESR) and CRP. According to activity indexes, pts were divided into subgroups with low (DAS28 < 3.2 and BASDAI < 4), and moderate-to-high disease activity (DAS28 > 3.2 and BASDAI > 4). Associations between serum levels of 25(OH)D and age, gender, ethnicity, type and disease duration, treatment, (anti-tumor necrosis factorα (TNFα) agents or DMARDs), seasonal variations, and disease activity were assessed. Vitamin D deficiency was found in 51 pts (42.1%). The incidence was higher among Arab pts (76.7%) compared to Jews (23%). The difference of 25(OH)D levels between Arabs (mean 9.4 ± 4.2 ng/ml) and Jews (mean 17.8 ± 8.4 ng/ml) was statistically significant (p < 0.0001). We did not find correlation between vitamin D levels and the other evaluated factors. A surprisingly high incidence of vitamin D deficiency was found in IJD patients in a sunny Mediterranean country. This finding justifies the inclusion of vitamin D in the routine lab work-up of pts with IJD. The only statistical significant correlation was found between vitamin D level and ethnic origin. Further studies are needed to look for genetic polymorphism of vitamin D receptors.

Seronegative polyarthritis as severe systemic disease.

BACKGROUND: Severe extra-articular disease is associated with high levels of rheumatoid factor (RF ) in patients with seropositive rheumatoid arthritis (RA ) and a poor prognosis. It is said that patients with seronegative rheumatoid arthritis have a more benign course and less destructive disease. We observed several patients with seronegative non-rheumatoid polyarthritis, with aggressive extra-articular systemic disease. OBJECTIVES: Review of seronegative systemic polyarthritis with clinical presentation of typical cases. METHODS: Medline search for systemic manifestations of seronegative polyarthritis. CLINICAL PRESENTATIONS: 1. A 56-year-old woman was admitted to the cardiac intensive care unit with stabbing presternal chest pain aggravated by breathing and progressive dyspnoea, which gradually developed over a period of two weeks with one episode of fever at 38.0 degrees C. She had suffered chronic pain in her buttocks for three years with polyarthralgia and evanescent palmar-plantar rash. Imaging showed bilateral sacroiliitis (HLA B27 negative) and a large pericardial effusion. Extra-articular manifestations of SAPHO syndrome were proposed and she was successfully treated with combined therapy: pulse methylprednisolone, azathioprine, colchicine and prednisone. 2. A 47-year-old woman with psoriatic arthropathy developed high fever with leucocytosis and thrombocytosis and lung infiltrates during exacerbation of her joint disease . She was treated with pulse methylprednisolone followed by corticosteroid tapering, anti-TNF (infliximab) and methotrexate with complete resolution. 3. A 19-year-old man with inflammatory bowel disease developed acute pericarditis with response to 6-mercaptopurine, salazopyrine and prednisone. RESULTS: We discuss a range of seronegative arthritis diseases with possible systemic manifestations including the main procedures for early diagnosis. Infection, malignancy, hypersensitivity, granulomatous disease and other collagen diseases such as systemic lupus erythematosus should be excluded, but investigations for an underlying disease should not delay early corticosteroid and immunosuppressive therapy. CONCLUSION: A high level of suspicion of extra-articular disease should always be maintained when treating active seronegative polyarthritis.

Efficacy of a targeted, oral penicillin-based yaws control program among children living in rural South America.

Yaws is endemic in rural Guyana. An observational study was conducted to determine the efficacy of oral penicillin V therapy in treating skin lesions of yaws in children. In 1999, inhabitants of 7 rural villages near Bartica, Guyana, were screened for skin lesions of yaws. Cases were confirmed by serological testing. A control program was implemented in 2000: children < or =14 years old were screened, and those with active lesions were treated with oral penicillin V for 7-10 days. In 2001, children were rescreened and active cases were treated. Prevalence of yaws skin lesions fell from 5.1% (52 of 1020 children screened in 2000) to 1.6% (8 of 516 in 2001), a 71% drop. Sixteen (94%) of 17 children treated in 2000 and reassessed in 2001 had complete resolution of lesions. A targeted, oral penicillin-based treatment regimen can successfully treat dermatologic yaws in individual children and can decrease the prevalence of skin yaws in a community in which it is endemic. This information may aid in the implementation of additional control efforts.