Overview.
Benign paroxysmal peritonitis syndrome is also known as familial Mediterranean fever syndrome, hereditary familial amyloidosis, and Seigal-Cattan-Mamon syndrome. The disease is most common in the Mediterranean coastal region, such as the Jews and Arabs of Spain and Portugal, and is more common in adolescents, more often in males than females, and also in children, but less common than in adults.
Etiology
The cause of the disease is unknown. It may be a familial autosomal recessive disorder, a type of amyloidosis.
Symptoms
The main symptoms are paroxysmal or irregular fever, abdominal pain, abdominal distension, which may be diffuse or confined to one area, often accompanied by chest pain, arthralgia, and neurologic dysfunction. The attacks are mostly intermittent and periodic, and the symptoms may be completely relieved between attacks. Female patients often have attacks during menstruation. Abdominal pressure, hepatomegaly, and signs of peritoneal effusion are positive.
Examination
1. The total number of leukocytes is often elevated during an attack, the blood sedimentation rate increases, and the alpha 2 globulin increases. Urine protein is positive, and tubular urine may be present.
2. Liver function may be mildly abnormal in liver involvement.
3. Pleural and abdominal fluid examination is plasma fibrinaceous.
4. Congo red test is performed by injecting 1% Congo red solution 0.22 ml/kg body weight intravenously, and then 10 ml of venous blood is withdrawn at 4 minutes and 1 hour later for colorimetric examination, which shows that the percentage of the dye retained in the serum is significantly reduced.
5. Histopathologic examination of the biopsy showed amyloid deposits.
Diagnosis
The presence of paroxysmal intermittent fever, abdominal pain, chest pain, arthralgia, and multiple manifestations of plasmapheresis in the Mediterranean region should be considered as a possibility of this disease in conjunction with their family history. The Congo red test is feasible due to the rapid absorption of Congo red by the amyloid material. Amyloid deposition in histopathologic examination of biopsy can confirm the diagnosis.
Complications
Accompanied by urticaria, polyneuritis, polyplasmacythemia vera.
Treatment
Colchicine can be applied to reduce the number of attacks and long-term prophylactic treatment (2-3 years). Due to its teratogenic, myelosuppressive, and sperm deficiency side effects, this drug should be used with caution, and it is thought to be used only in patients with significantly limited activity. The application of dimethylafonium has also been reported. Adrenocorticotropic hormones may also be tried for symptomatic relief during an attack, and supportive therapy should be intensified.