Our study found that high serum CRP level (>26.8 mg/L) was a risk factor for the survival of patients with iMCD; and we built the CRP-A nomogram model based on serum CRP level (>26.8 mg/L), age > 60 years, hepatomegaly and/or splenomegaly, HGB ≤80 g/L, and PC type, which can better quantify survival risk of the 1-year, 3-year, and 5-year iMCD patients, providing a tool for risk stratification in iMCD based on CRP-associated biomarkers. Here, CRP is linked to pachyonychia congenita.