Israel Medical Association Journal

Abstract

Background: Scleroderma lung disease (ILD-SSc) is treated mainly with cyclophosphamide (CYC). The effectiveness of CYC was judged after 12–24 months in most reports.

Objectives: To analyze the effect of monthly intravenous CYC on pulmonary function tests including forced vital capacity (FVC) and diffusing lung capacity (DLCO), as well as Rodnan skin score (mRSS), during long-term follow-up.

Methods: We retrospectively collected the data on 26 ILD-SSc patients who began CYC treatments before 2007. Changes in FVC, DLCO and mRSS before treatment, and at 1, 4 and 7 years after completion of at least six monthly intravenous CYC treatments for ILD-SSc were analyzed.

Results: Mean cumulative CYC dose was 8.91 ± 3.25 G. More than 30% reduction in FVC (0%, 8%, and 31% of patients), DLCO (15%, 23%, 31%), and mRSS (31%, 54%, 62%) at years 1, 4 and 7 was registered. During the years 0–4 and 4–7, annual changes in FVC, DLCO and mRSS were 3.2 vs. 0.42% (P < 0.040), 4.6 vs. 0.89% (P < 0.001), and 1.8 vs. 0.2 (P = 0.002). The greatest annual FVC and DLCO reduction over the first 4 years correlated with mortality (P = 0.022). There were no differences in the main variables regarding doses of CYC (< 6 G and > 6 G).

Conclusions: In patients with ILD-SSc, CYC stabilized the reduction of FVC during treatment, but this effect was not persistent. The vascular characteristic of ILD-SSc (DLCO) was not affected by CYC treatment. CYC rapidly improved the mRSS. This effect could be achieved with at least 6 G of CYC. Higher rates of annual reduction in FVC and DLCO in the first 4 years indicate the narrow window of opportunity and raise the question regarding ongoing immunosuppression following CYC infusions.

 

Idiopathic pulmonary arterial hypertension (IPAH) is an isolated small-vessel disease comprising vasoconstriction, remodeling and thrombosis of small pulmonary arteries. However, there is evidence that IPAH[1] does not respect anatomic boundaries and might extend into large vessels such as large central thrombi. On the other hand, chronic thromboembolic pulmonary hypertension (CTEPH) represents a distinct category of pulmonary hypertension as it is thought to be due to an occlusion of the major pulmonary arteries following a thromboembolic event. However, it is currently evident that in most patients, there is a concomitant small-vessel disease. The involvement of both small and large vessels in both IPAH and CTEPH[2] together with a high incidence of silent thromboembolic events might create difficulties in identifying the true cause of pulmonary hypertension. An accurate diagnosis of the cause determines the management and prognosis. Patients with CTEPH can potentially be offered curative surgery in the form of pulmonary endarterectomy; however, oxygen, vasodilators, anticoagulation, and lung transplantation are more feasible options for IPAH.

Objectives: To evaluate the outcome of this procedure and to compare the complication rate following circumcisions performed by ritual circumcisers and by physicians.

Methods: In 2001, of the 19,478 males born in four major medical centers in Israel 66 had circumcision-related complications. All the children were circumcised in non‑medical settings within the community. The patients were medically evaluated either urgently due to immediate complications or electively in outpatient clinics later on. Upon the initial assessment a detailed questionnaire was filled to obtain data regarding the procedure, the performer and the subsequent complications.

Results: All the circumcisions were performed during the early neonatal life, usually on day 8 of life (according to Jewish law). In 55 cases (83%) it was part of a ritual ceremony conducted by a ritual circumciser (Mohel), while in 11 babies (17%) physicians were involved. Acute bleeding after circumcision was encountered in 16 cases (24%), which required suturing in 8. In addition, we found two cases of wound infection and one case of partial amputation of glans penis in which the circumcision was performed by a ritual circumciser. Among the late complications, the most common was excess of skin in 38 cases (57%); 5 children (7.5%) had penile torsion and 4 children (6%) had shortages of skin, phimosis and inclusion cyst. The overall estimated complication rate of circumcision was 0.34%.

Conclusions: Complications of circumcision are rare in Israel and in most cases are mild and correctable. There appears to be no significant difference in the type of complications between medical and ritual circumcisions.