What clinical features help delineate prognosis in Relapsing Polychondritis

What clinical features help delineate prognosis in Relapsing Polychondritis?

• Nasal chondritis is associated with airway involvement and should prompt additional evaluation of the respiratory tree with CT or MRI scan imaging regardless of pulmonary symptoms.

• Airway involvement is associated with higher rates of infection as well as ICU admissions.

• Renal involvement has traditionally been associated with a worse prognosis, but prior studies may have included patients with missed diagnoses of ANCA-associated vasculitis (see Question 16).

• Vasculitis is associated with a higher mortality.

• Male sex is associated with a higher mortality (independent from other risk factors).

• Cardiac involvement is associated with a higher mortality.

• MDS (and other hematologic malignancies) are associated with higher mortality. In a large series of 200 patients with RPC, over 10% had concurrent MDS. Patients are more commonly men, typically over the age of 60 years, and have higher rates of cutaneous involvement than RPC patients without MDS (75% versus 25%). Neutrophilic dermatoses, including Sweet’s syndrome, may be especially common in patients with RPC and myelodysplasia.

In 1976, McAdam et al. reported that the 5- and 10-year survival rates of 112 patients with RPC were 74% and 55%, respectively. Infection and systemic vasculitis were the major causes of death. More recent cohorts have demonstrated improved 10-year survival rates of >90%, although among patients with concurrent MDS, survival may be as low as 50%. A recent series of 142 patients with RPC (followed for an average of 13 years) identified three distinct phenotypes based on clinical features and prognosis:

1) Hematologic phenotype (MDS & less common hematologic malignancies): worst prognosis with 58% 10-year mortality and over 50% of patients with a serious infection as well as an ICU admission during the study period. A large number of clinical manifestations of RPC (80% of patients with eight or more manifestations) was seen in this phenotype grouping, with the exception of tracheobronchial involvement.

2) Respiratory phenotype (characterized by laryngotracheal or bronchial tree involvement): moderate rates of serious infection (35%) and ICU admission (27%), but mortality similar to the “mild phenotype.”

3) Mild phenotype (absence of hematologic disorder and respiratory tree involvement): low rates of serious infection (6%), ICU admission (2%), and death (4%).

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