Diagnostic Criteria Drafted For Juvenile Systemic Sclerosis

VERSAILLES, FRANCE — The presence of sclerosis/induration is the cornerstone criterion for confirming the diagnosis of juvenile systemic sclerosis, according to conclusions drawn from an international consensus conference.

In addition, Francesco Zulian, M.D., explained at the 12th European Pediatric Rheumatology Congress, that the diagnosis also requires that patients have at least two of the following minor criteria:

Skin: Sclerodactyly.

Vascular: Raynaud's phenomenon, digital ulcers, nailfold capillary changes.

Gastrointestinal: Gastrointestinal reflux, dysphasia.

Respiratory: Lung fibrosis, pulmonary hypertension, diffuse lung capacity for carbon monoxide.

Renal: Renal crisis, new-onset hypertension.

Cardiac: Heart failure, arrhythmias.

Neurologic: Carpal tunnel syndrome, peripheral neuropathy.

Musculoskeletal: Arthritis, myositis, tendon friction rubs.

Serology: Antinuclear antibodies, systemic sclerosis-selective autoantibodies.

Dr. Zulian of the University of Padua (Italy) emphasized that these criteria are in the draft stage. The next step will be to validate the classification with trials in adult and juvenile patients. Although the system is based on childhood cases, he suggested it might also improve the rate of diagnosing the disease accurately in adults.

An added benefit of the project is that “we [now] have an international database we are going to open up and use for future research,” he told this newspaper in an interview. “This is a very rare disease, and we need cooperation between different countries. It is a devastating disease, and the mortality is high.”

Fifty-five centers in 24 countries contributed data on 205 patients. The cases included 153 patients with systemic sclerosis, 26 patients with overlap syndrome, and another 26 patients with mixed connective tissue disease.

The project, which began in 2002, initially identified 86 preliminary classification criteria, including the three major criteria: sclerosis/induration, Raynaud's phenomenon, and sclerodactyly.

A group of 16 adult and pediatric rheumatologist experts then used the criteria to diagnose 160 patients from records presented at a consensus conference. The case files included children with systemic sclerosis and the confounding diseases.

The experts were able to reach consensus on 127 patients (79%): 70 with juvenile systemic sclerosis and 57 without the condition. They were unable to reach a consensus on 33 patients (21%), Dr. Zulian reported.

As a result of that concensus process, the 86 possible diagnostic factors were narrowed down to a short list. The objectives were “to propose classification criteria that mimic as much as possible the evaluation of physicians attending the consensus conference,” and to evaluate how well the criteria distinguish patients who have juvenile systemic sclerosis from those who do not, he said.

VERSAILLES, FRANCE — The presence of sclerosis/induration is the cornerstone criterion for confirming the diagnosis of juvenile systemic sclerosis, according to conclusions drawn from an international consensus conference.

In addition, Francesco Zulian, M.D., explained at the 12th European Pediatric Rheumatology Congress, that the diagnosis also requires that patients have at least two of the following minor criteria:

Skin: Sclerodactyly.

Vascular: Raynaud's phenomenon, digital ulcers, nailfold capillary changes.

Gastrointestinal: Gastrointestinal reflux, dysphasia.

Respiratory: Lung fibrosis, pulmonary hypertension, diffuse lung capacity for carbon monoxide.

Renal: Renal crisis, new-onset hypertension.

Cardiac: Heart failure, arrhythmias.

Neurologic: Carpal tunnel syndrome, peripheral neuropathy.

Musculoskeletal: Arthritis, myositis, tendon friction rubs.

Serology: Antinuclear antibodies, systemic sclerosis-selective autoantibodies.

Dr. Zulian of the University of Padua (Italy) emphasized that these criteria are in the draft stage. The next step will be to validate the classification with trials in adult and juvenile patients. Although the system is based on childhood cases, he suggested it might also improve the rate of diagnosing the disease accurately in adults.

An added benefit of the project is that “we [now] have an international database we are going to open up and use for future research,” he told this newspaper in an interview. “This is a very rare disease, and we need cooperation between different countries. It is a devastating disease, and the mortality is high.”

Fifty-five centers in 24 countries contributed data on 205 patients. The cases included 153 patients with systemic sclerosis, 26 patients with overlap syndrome, and another 26 patients with mixed connective tissue disease.

The project, which began in 2002, initially identified 86 preliminary classification criteria, including the three major criteria: sclerosis/induration, Raynaud's phenomenon, and sclerodactyly.

A group of 16 adult and pediatric rheumatologist experts then used the criteria to diagnose 160 patients from records presented at a consensus conference. The case files included children with systemic sclerosis and the confounding diseases.

The experts were able to reach consensus on 127 patients (79%): 70 with juvenile systemic sclerosis and 57 without the condition. They were unable to reach a consensus on 33 patients (21%), Dr. Zulian reported.

As a result of that concensus process, the 86 possible diagnostic factors were narrowed down to a short list. The objectives were “to propose classification criteria that mimic as much as possible the evaluation of physicians attending the consensus conference,” and to evaluate how well the criteria distinguish patients who have juvenile systemic sclerosis from those who do not, he said.

VERSAILLES, FRANCE — The presence of sclerosis/induration is the cornerstone criterion for confirming the diagnosis of juvenile systemic sclerosis, according to conclusions drawn from an international consensus conference.

In addition, Francesco Zulian, M.D., explained at the 12th European Pediatric Rheumatology Congress, that the diagnosis also requires that patients have at least two of the following minor criteria:

Skin: Sclerodactyly.

Vascular: Raynaud's phenomenon, digital ulcers, nailfold capillary changes.

Gastrointestinal: Gastrointestinal reflux, dysphasia.

Respiratory: Lung fibrosis, pulmonary hypertension, diffuse lung capacity for carbon monoxide.

Renal: Renal crisis, new-onset hypertension.

Cardiac: Heart failure, arrhythmias.

Neurologic: Carpal tunnel syndrome, peripheral neuropathy.

Musculoskeletal: Arthritis, myositis, tendon friction rubs.

Serology: Antinuclear antibodies, systemic sclerosis-selective autoantibodies.

Dr. Zulian of the University of Padua (Italy) emphasized that these criteria are in the draft stage. The next step will be to validate the classification with trials in adult and juvenile patients. Although the system is based on childhood cases, he suggested it might also improve the rate of diagnosing the disease accurately in adults.

An added benefit of the project is that “we [now] have an international database we are going to open up and use for future research,” he told this newspaper in an interview. “This is a very rare disease, and we need cooperation between different countries. It is a devastating disease, and the mortality is high.”

Fifty-five centers in 24 countries contributed data on 205 patients. The cases included 153 patients with systemic sclerosis, 26 patients with overlap syndrome, and another 26 patients with mixed connective tissue disease.

The project, which began in 2002, initially identified 86 preliminary classification criteria, including the three major criteria: sclerosis/induration, Raynaud's phenomenon, and sclerodactyly.

A group of 16 adult and pediatric rheumatologist experts then used the criteria to diagnose 160 patients from records presented at a consensus conference. The case files included children with systemic sclerosis and the confounding diseases.

The experts were able to reach consensus on 127 patients (79%): 70 with juvenile systemic sclerosis and 57 without the condition. They were unable to reach a consensus on 33 patients (21%), Dr. Zulian reported.

As a result of that concensus process, the 86 possible diagnostic factors were narrowed down to a short list. The objectives were “to propose classification criteria that mimic as much as possible the evaluation of physicians attending the consensus conference,” and to evaluate how well the criteria distinguish patients who have juvenile systemic sclerosis from those who do not, he said.