RESUMEN
Background and Aims: Behçet's disease is a chronic, multisystemic, and relapsing inflammatory disorder. It lacks a permanent cure, the focus of treatment is on mitigating symptoms, decreasing the frequency and severity of relapses, and preventing life-threatening complications. This study aims to report the experience of a single center in managing patients with Behçet's disease and discuss the treatment outcomes. Methods: This study was a retrospective case series conducted over 2 years. All cases were clinically diagnosed according to the International Criteria for Behçet's Disease. The extracted data were demographics, family history, clinical findings, criteria scores, treatment, and outcomes. Results: A total of 31 patients were included, consisting of 13 males (42%) and 18 females (58%). Most cases were over the age of 30, and both genders were nearly equally distributed among age groups. The most commonly affected site was the oral cavity, observed in 96.77% of cases. Genital, cutaneous, and vascular involvements were more common in males, while females were more likely to have oral, ocular, and musculoskeletal involvements. For various treatment regimens, oral, cutaneous, vascular, and musculoskeletal involvements showed complete response in all cases. Among cases with genital involvement, complete response was achieved in seven cases (41.2%), while four cases (23.5%) showed only partial response, and six cases (35.3%) experienced recurrence. In cases with ocular involvement, only partial responses were observed. Conclusion: Oral, cutaneous, vascular, and musculoskeletal involvements may have a higher likelihood of a complete response to treatment regimens. However, genital involvement may be the most recurrent manifestation, followed by ocular involvement.
RESUMEN
Amyloidosis is a rare disorder characterized by the deposition of abnormal proteins in extracellular tissues, resulting in the dysfunction of vital organs and, eventually, death. The occurrence of amyloidosis due to primary Sjogren's syndrome (pSS) is a rare finding. This study describes a rare case of pSS complicated by amyloid-associated amyloidosis. Case presentation: A 35-year-old male was diagnosed with nephrotic syndrome and secondary amyloidosis caused by pSS. He had microscopic hematuria, a creatinine level of 6.59 mg/dl, and an elevated erythrocyte sedimentation rate of 107 mm/hrs. Furthermore, investigations of antinuclear antibodies, antimitochondrial antibodies, SSA, SSA native, and Ro-52 recombinant as well as rheumatoid factor showed positive results. After establishing the diagnosis of pSS through clinical, physical, and laboratory assessments, a renal biopsy was performed, which revealed the occurrence of secondary amyloidosis. Clinical discussion: The risk of developing secondary amyloidosis depends on the extent of elevated serum amyloid levels as well as persistent subclinical inflammation. The definitive diagnosis of amyloidosis requires histological confirmation of amyloid fibril deposition in tissue. Conclusion: Secondary renal amyloidosis is an unusual condition in patients with pSS. Still, it should be regarded in the differential diagnosis of patients with proteinuria and/or renal failure, and a renal biopsy should be performed.