MD News and Information

Pneumocystis jiroveci pneumonia is a rare but serious complication for patients with rheumatic diseases

Author: Erika Darrah
Date Published: March-2017
Source: Johns Hopkins Medicine

A research team from the Johns Hopkins Division of Rheumatology lead by Christopher Mecoli, M.D., M.H.S, studied medical records of patients with rheumatic diseases who were admitted to the Johns Hopkins Hospital for pneumocystis jiroveci pneumonia (PJP) infection over a 20-year period (1996-2015). PJP is an uncommon but severe lung infection that can occur in patients with rheumatic disease, in particular in those who are taking medications that suppress the immune system.

 

Pros and cons of echocardiography in the screening, diagnosis and follow-up of patients with systemic sclerosis pulmonary arterial hypertension – a rheumatologist’s perspective

Author: Murray Baron
Date Published: February-2017
Source: JSRD

Purpose: Our objective was to review the evidence regarding echocardiography in patients with systemic sclerosis (SSc) and possible pulmonary hypertension (PH).

Methods: The literature was reviewed. In addition, a survey was conducted of 315 rheumatologists/internists with a self-declared interest in SSc, to determine their preferred use of echocardiograms.

Results: The most relevant literature findings come from two studies, the DETECT study and one from the Australian Scleroderma Interest Group. In both these studies, it appears that the use of non-echocardiographic variables such as pulmonary function tests (PFTs) and values of serum N-terminal pro-brain natriuretic peptide (NT-proBNP), are adequate on their own in suggesting which patients are at high risk of PH. Echocardiograms added very little information and in fact may confuse the picture by appearing to be normal when in fact underlying PH is present.

 

 

Digital ulcers in SSc treated with oral treprostinil: a randomized, double-blind, placebo-controlled study with open-label follow-up

Author: J. Seibold, F. Wigley, E. Schiopu, C. Denton, et al
Date Published: February-2017
Source: JSRD

Methods: The literature was reviewed. In addition, a survey was conducted of 315 rheumatologists/internists with a self-declared interest in SSc, to determine their preferred use of echocardiograms.

Background: Prostacyclins are routinely used to treat vascular features of systemic sclerosis (SSc, scleroderma) but require parenteral infusion or inhalation. This study evaluated the safety and efficacy of oral treprostinil in digital ulcers secondary to SSc. Methods: This was a randomized (1:1) placebo-controlled, multicenter study in adults with SSc and at least one active digital ulcer at entry. Oral treprostinil was administered twice daily and titrated to maximum tolerated dose with clinical assessments at Weeks 5, 10, 15 and 20. The primary endpoint was change in net digital ulcer burden. Secondary outcomes included ulcer healing and prevention, measures of hand function, quality of life, Raynaud phenomenon and global assessments. Simplified data were gathered during open-label follow up.

 

Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS)

Author: A. Herrick, X. Pan, S. Peytrignet, M. Lunt, R. Hesselstrand, L. Mouthon, A. Silman, E. Brown, L. Czirják, J. Distler, O. Distler, et al
Date Published: February-2017
Source: Annals of the Rheumatic Diseases

Objectives The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches.

Methods This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or ‘no immunosuppressant’. Patients were assessed three-monthly for up to 24 months. The primary outcome was the change in modified Rodnan skin score (mRSS). Confounding by indication at baseline was accounted for using inverse probability of treatment (IPT) weights. As a secondary outcome, an IPT-weighted Cox model was used to test for differences in survival.

 

Immunosuppressants Only of ‘Weak’ Benefit to Diffuse Scleroderma Patients, Study Reports

Author: Magdalena Kegel
Date Published: February-2017
Source: Scleroderma News

A large study following patients in the early stages of diffuse cutaneous scleroderma showed that immunosuppressants offered only a negligible benefit for those who took them, compared to those who didn’t. Since immunosuppressants produced numerically larger reductions in skin symptoms, the findings may support patients using such drugs from the onset of their disease. But since differences between treated and untreated patients were not statistically significant, the study may also point to the need for better treatments.

 

Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS)

Author: A. Herrick1, X. Pan, S. Peytrignet, M. Lunt, R. Hesselstrand, et al
Date Published: February-2017
Source: Annals of the Rheumatic Diseases

Objectives The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches. Methods This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or ‘no immunosuppressant’. Patients were assessed three-monthly for up to 24 months. The primary outcome was the change in modified Rodnan skin score (mRSS). Confounding by indication at baseline was accounted for using inverse probability of treatment (IPT) weights. As a secondary outcome, an IPT-weighted Cox model was used to test for differences in survival.

 
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