Friday, 15 Feb 2019

You are here

Scleroderma Expert Treatment Preferences

Frustration may be the word that best characterizes many scleroderma management plans - owing to a lack of trials, lack of agreement and lack of clear guidance on management.

A panel of concerned experts  on systemic sclerosis (SSc) set out to develop consensus on SSc treatment algorithms.

Experts were from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (n = 170). Consensus was reached in several areas:

  1. scleroderma renal crisis: 81% of experts agreed  that first‐, second‐, and third‐line treatments were angiotensin‐converting enzyme inhibitors, then adding calcium‐channel blockers [CCBs], then adding angiotensin receptor blockers [ARBs], respectively).
  2. pulmonary arterial hypertension (PAH),:81% of experts agreed (for mild PAH, treatments were phosphodiesterase 5 [PDE5] inhibitors, then endothelin receptor antagonists plus PDE5 inhibitors, then prostanoids, respectively; for severe PAH, prostanoids were first‐line treatment).
  3. mild Raynaud's phenomenon: 79% of experts agreed (treatments were CCBs, then adding PDE5 inhibitors, then ARBs or switching to another CCB, respectively;
  4. Severe Raynauds: first‐ through fourth‐line treatments were CCBs, then adding PDE5 inhibitors or prostanoids, then adding PDE5 inhibitors (if not added as second‐line treatment) or prostanoids (if not added as second‐line treatment), then switching to another CCB, respectively. For active treatment of digital ulcers, 66% of experts agreed (first‐ and second‐line treatments were CCBs and PDE5 inhibitors, respectively).
  5. Interstitial lung disease: 69% of experts agreed (for induction therapy, treatments were mycophenolate mofetil [MMF], intravenous cyclophosphamide [IV CYC], and rituximab, respectively; for maintenance, first‐line treatment was MMF).
  6. Skin involvementt: 71% of experts agreed (for a modified Rodnan skin thickness score [MRSS] of 24, first‐ and second‐line treatments were methotrexate [MTX] and MMF, respectively; for an MRSS of 32, first‐ through fourth‐line treatments were MMF, MTX, IV CYC, and hematopoietic stem cell transplantation, respectively).
  7. Inflammatory arthritis:79% of experts agreed (first‐ through fourth‐line treatments were MTX, low‐dose glucocorticoids, hydroxychloroquine, and rituximab or tocilizumab, respectively). 
Disclosures: 
The author has no conflicts of interest to disclose related to this subject

Add new comment

More Like This

Mortality and Hospitalization with Pulmonary Hypertension in Systemic Sclerosis

PHAROS is a prospective cohort studying the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). They have shown that risk factors for poor outcomes in this cohort included male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. 

Noninfectious Proximal Aortitis Needs Serial Follow-ups

A Cleveland Clinic review of patients undergoing thoracic aortic surgery (1996‐2012) identified 196 patients with histopathology‐proven non‐infectious aortitis. The majority of these patients had a recognized systemic disorder, and serial assessments and imaging is crucial to an accurate diagnosis.

Ten Contemporary Challenges Facing Lupus Treatment

A group of French researchers have pooled their interests to identify the challenges ahead in the management of patients with systemic lupus erythematosus (SLE). 

Elevated Fracture Risk in Lupus

A Medicaid study of lupus (SLE) patients finds that fracture risks are elevated in low‐income SLE and lupus nephritis patients compared to those without SLE.

A cohort study matched Medicaid SLE patients and age‐ and sex‐matched non‐SLE comparators to study the primary outcome of fracture of the pelvis, wrist, hip, or humerus.

Improved Survival with Renal Transplant in SLE

The Annals of Internal Medicine has published a cohort study of lupus nephritis patients with end-stage renal disease showing that renal transplantation is associated with improved survival benefit, and a reduction in death from cardiovascular disease and infection.