Tabla I. Características basales de los pacientes. |
|
n = 47 |
|
Edad (años), mediana (DE) |
46 ± 15 |
Sexo masculino, n (%) |
26 (55,3) |
Comorbilidades: |
|
Hipertensión, n (%) |
5 (10,6) |
Diabetes, n (%) |
9 (19,1) |
Enfermedad autoinmunitaria, n (%) |
2 (4,3) |
Tiempo desde el inicio de síntomas hasta el diagnóstico (meses), mediana (RIC) |
6 (2-12) |
Puntuación del MRC en el diagnóstico, mediana (DE) |
29,3 ± 21,9 |
GBS, mediana (RIC) |
3 (3-4) |
Afectación de los nervios craneales, n (%) |
6 (12,8) |
Facial, n (%) |
4 (8,5) |
Bulbar, n (%) |
4 (8,5) |
Variantes típicas de PDIC: |
|
Sensomotora, n (%) |
27 (57,4) |
PDIC de inicio agudo, n (%) |
10 (21,3) |
Variantes atípicas: |
|
De predominio distal, n (%) |
4 (8,5) |
Asimétrica, n (%) |
2 (4,2) |
Ataxia-temblor, n (%) |
2 (4,2) |
Predominio sensitivo (%) |
2 (4,2) |
Disociación albuminocitológica, n (%) |
40/44 (90,9) |
Proteínas (mg/dL), mediana (RIC) |
124 (60-245) |
Tratamiento en el diagnóstico: |
|
Prednisona, n (%) |
14 (29,8) |
Prednisona + pulsos de metilprednisolona, n (%) |
22 (46,9) |
Prednisona + plasmaféresis o IgIV, n (%) |
10 (21,3) |
Agente ahorrador de esteroides (azatioprina, ciclofosfamida), n (%) |
22 (46,8) |
Tiempo al inicio de ahorrador de esteroides, meses (RIC) |
9 (1-18) |
DE: desviación estándar; GBS: Guillain-Barre Disability Score; IgIV: inmunoglobulina intravenosa; MRC: Medical Research Council; PDIC: polineuropatía desmielinizante inflamatoria crónica; RIC: rango intercuartílico. |
Tabla II. Diferencias clínicas en pacientes con buena respuesta al tratamiento frente a mala a los tres meses de seguimiento desde el inicio del tratamiento con esteroides. |
|||
Respuesta favorable |
Respuesta desfavorable |
Valor de p |
|
Edad (años), mediana (DE) |
45,8 ± 16,7 |
45,7 ±1 2,4 |
0,97 |
Edad > 60 años, n (%) |
6 (20,6) |
3 (17,6) |
> 0,99 |
Sexo masculino, n (%) |
18 (62) |
8 (47) |
0,36 |
Diabetes, n (%) |
4 (13,7) |
4 (23,5) |
0,44 |
Tiempo desde el inicio de síntomas hasta el diagnóstico (meses), mediana (RIC) |
4 (1,5-9) |
8 (5,5-18) |
0,005 |
Retraso ≥ 6 meses en el diagnóstico, n (%) |
8 (27,5) |
11 (64,7) |
0,028 |
Puntuación del MRC en el diagnóstico (DE) |
37,6 ± 12,9 |
40,12 ± 11,9 |
0,52 |
Afectación de los nervios craneales, n (%) |
2 (6,8) |
3 (17,6) |
0,34 |
Variantes típicas, n (%) |
27 (93,1) |
9 (52,9) |
0,003 |
Sensitivo motora, n (%) |
18 (62) |
8 (47) |
0,36 |
PDIC de inicio agudo, n (%) |
9 (31) |
1 (5,8) |
0,047 |
Variantes atípicas, n (%) |
2 (6,8) |
8 (47) |
0,003 |
De predominio distal, n (%) |
1 (3,4) |
3 (17,6) |
0,53 |
Asimétrica, n (%) |
0 (0) |
2 (11,7) |
|
Ataxia-temblor, n (%) |
1 (3,4) |
1 (5,8) |
|
De predominio sensitivo n (%) |
0 (0) |
2 (11,7) |
|
Tratamiento en el diagnóstico |
|||
Prednisona, n (%) |
10 (34,4) |
5 (29,4) |
> 0,99 |
Prednisona + pulsos de metilprednisolona, n (%) |
13 (44,8) |
9 (52,9) |
> 0,99 |
Prednisona + plasmaféresis or IgIV, n (%) |
6 (20,6) |
3 (17,6) |
0,73 |
DE: desviación estándar; IgIV: inmunoglobulina intravenosa; MP: pulsos de metilprednisolona (1 gramo intravenosos durante cinco días); MRC: Medical Research Council; PDIC: polineuropatía desmielinizante inflamatoria crónica; RIC: rango intercuartílico. Plasmaféresis (cinco sesiones): IgIV (2 g/kg). |
Figura 1. Cambio de la Guillain-Barre Disability Score en el tiempo en pacientes con terapia con prednisona.
Figura 2. Cambio de la puntuación del Medical Research Council en el tiempo en pacientes con terapia con prednisona.
Tabla III. Respuesta clínica en el seguimiento. |
|||||||
Inicial |
3 meses |
6 meses |
12 meses |
18 meses |
24 meses |
Valor de p |
|
GBS, mediana (RIC) |
3 (2,25-4) |
2 (1,25-3) |
2 (1-2) |
1,5 (1-2) |
1 (1-2) |
1 (0-2) |
< 0,001 |
MRC, media, DE |
39,5 ± 12 |
47,7 ± 9,5 |
50,5 ± 11,5 |
54,9 ± 5,1 |
55,1 ± 5,7 |
56,3 ± 5,1 |
< 0,001 |
Marcha independiente (%) |
8 (17%) |
27 (60%) |
34 (75,6%) |
39 (92,9%) |
37 (90,2%) |
30 (93,8%) |
< 0,001 |
Dosis de prednisona (mg), mediana (RIC) |
50 (32,5-50) |
40 (20-40) |
30 (20-40) |
10 (5-20) |
5 (0-10) |
1 (0-5) |
< 0,001 |
DE: desviación estándar; GBS: Guillain-Barre Disability Score; MRC: Medical Research Council; RIC: rango intercuartílico. |
Efficacy of long-term prednisone therapy in patients with chronic inflammatory demyelinating polyneuropathy (CIDP): a retrospective cohort study Introduction. Patients with CIDP respond adequately to steroid therapy and intravenous immunoglobulin (IVIG). However, few patients have access to IVIG in developing countries. Little information exists about the clinical response to steroid therapy in Latin American countries. Objective. to describe the long-term functional clinical response (24 months) to prednisone therapy in CIDP patients. Material and methods. A retrospective cohort was conducted. Selection included patients with definitive CIDP diagnosis according to European criteria from the Neuromuscular Diseases clinic of the National Institute of Neurology and Neurosurgery between January 2016 and December 2020. Good response to steroid therapy was defined as with improvement in at least one point on the GBS disability score. Poor response to steroid therapy was defined as patients who did not show improvement in at least one point on the GBS disability score. Patients were evaluated at 3, 6, 12, 18 and 24 months. Results. Forty-seven patients with CIDP were included. Half of them were male and mean age was 46±15 years. Mean time since symptom onset to diagnosis was 6 (IQR 2-12) months. The most common clinical variant was sensory-motor 57.4%, followed by acute-onset CIDP 21.3% and atypical variants 21.2%. At diagnosis our patients presented: mean GBS disability score of 3 (2.25-4) points, MRC score 39.5 ± 12 points, independent gait in 17%, mean prednisone dose of 50 mg (32.5-50). Twenty-four months after prednisone therapy, a less mean GBS disability score –1(0-2) points–, mean MRC score 56.3 ± 5.1 points, independent gait 93% and prednisone dose 1 (0-5) mg. Patients with poor three-month functional clinical response had a delay in diagnosis > 6 months (64.7% vs 27.5%) and atypical clinical variants (47% vs 6.8%). Conclusion. CIDP patients treated with prednisone have good long-term functional clinical response. Delay in diagnosis and atypical variant are common clinical characteristics for poor functional clinical response in treatment with prednisone. Key words. Chronic. CIDP. Neuropathy. Prednisone. Prognosis. Treatment. |