Catastrophic antiphospholipid syndrome presenting as fever of unknown origin

Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE) or "primary" antiphospholipid syndrome (APLS). We report a case of a man with catastrophic antiphospholipid syndrome (CAPS), which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.     

Dissecting aortic aneurysm presenting as pyrexia of unknown origin: Report of a case

(Received for publication on June 5, 1996; accepted on Jan. 7, 1997)     

Intrahepatic cholangiocarcinoma masked as fever of unknown origin

Intrahepatic cholangiocarcinoma is a rare malignancy that often presents in an advanced stage. For many patients, early diagnosis is often delayed, secondary to vague symptoms and a lack of physical findings. Herein, we report an unusual case of fever of unknown origin secondary to intrahepatic cholangiocarcinoma. Presented at the Fourth Americas Congress of the American Hepato-Pancreato-Biliary Association, Miami Beach, Florida, February 27–March 2, 2003.     

Sclerosing mediastinitis of unknown origin: Report of a case

IntroductionWe herein describe a rare case of a sclerosing mediastinitis without IgG4-related disease. This case was clearly excluded from IgG4-related disease, because this patient’s serum IgG4 level was not elevated. Specifically, this patient’s serum IgG4 level was 7.9 mg/dl (4.8–105).Presentation of caseA 61-year-old Japanese female presented at our hospital due to an abnormal chest X-ray that showed a growing shadow in the mediastinum. Chest computed tomography (CT) showed an 80 × 75 × 75 mm tumor, which located in the anterior mediastinum. This large tumor surrounded the thoracic aorta, left brachiocephalic vein and superior vena cava. It was difficult to obtain a definitive diagnosis. We tried to perform three biopsies, and eventually performed a partial resection of the tumor.DiscussionThis case did not fit the criteria for IgG4-related disease, and it was therefore unclear whether steroid therapy should be used for this case. We will continue to carefully follow up this patient’s residual lesion, and there have been no changes in the lesion at present.ConclusionSclerosing mediastinitis and IgG4-related disease should be included in the differential diagnosis of patients presenting with a mediastinal tumor. However, sclerosing mediastinitis is difficult to diagnose, and it is important to obtain a sufficient amount of tissue to ensure an accurate diagnosis.     

Laparoscopic approach to fever of unknown origin

Background: Fever of unknown origin (FUO) is difficult to diagnose. Laparotomy is needed to establish the etiologic diagnosis in some patients. The aim of this study was to analyze the feasibility, safety, and success rate of a protocolized laparoscopy in patients with FUO. Methods: An extensive clinical evaluation was performed before surgery. Laparoscopy included inspection of the abdominal cavity, wedge and tru-cut liver biopsies, lymph node biopsy, splenectomy, and bone marrow biopsy. Histologic analysis, permanent section analysis, and cultures were obtained. Results: The study involved 15 patients with a mean age of 43.6 ± 14.5 years. The mean operative time was 122 ± 60 min. Minor complications occurred in 9% of the patients. One patient bled after surgery and underwent reoperation. There was no operative mortality. An etiologic diagnosis was made in 66% of the patients, and laparoscopy helped to rule out intraabdominal pathology in four additional patients, giving a total success rate of 93%. Conclusion: Protocolized laparoscopy in patients with FUO is safe, feasible, and accurate.     

Tumor necrosis factor-alpha inhibitor-induced lupus-like syndrome presenting as fever of unknown origin in a liver transplant recipient: case report and concise review of the literature

A 44-year-old man was admitted to the hospital with fever and myalgias 11 years after deceased donor liver transplantation for primary sclerosing cholangitis associated with ulcerative colitis. During hospitalization, he developed anemia, thrombocytopenia, and serositis. An extensive series of investigations eliminated infectious, malignant, thrombotic, and metabolic causes of fever. Because the patient had received tumor necrosis factor (TNF)-alpha inhibitor therapy for refractory pouchitis, a diagnosis of TNF-alpha inhibitor-induced lupus-like syndrome was considered. Further evaluation revealed an elevated antinuclear antibody titer of 1:640. Following discontinuation of the TNF-alpha inhibitor and a brief course of systemic corticosteroid therapy, the patient's symptoms resolved. TNF-alpha inhibitor therapy is increasingly used for posttransplantation management of inflammatory bowel disease, and drug-induced lupus is an increasingly recognized complication of such therapy. Because TNF-alpha inhibitor-induced lupus may not be recognized due to its nonspecific symptoms and the potential coexisting illnesses present in transplant recipients, a high index of suspicion is required.     

Treatment of small vessel vasculitis affecting the kidneys

Summary: Treatment of vasculitis can be divided into two phases: (i) an induction phase to achieve remission, abate destructive inflammation and minimize scarring; and (ii) the maintenance phase to sustain patients in remission with minimal treatment-related side-effects. A combination of corticosteroids and cytotoxic agents is commonly used as induction therapy. the dose and route of administration of corticosteroids have not been studied adequately, but intravenous (i.v.) bolus doses of methyiprednisolone are often administered to patients with severe disease. It has the advantage of fewer side-effects compared to prolonged high dose oral corticosteroids, and the immediate immuno-modulatory effects of the steroid boluses may confer additional therapeutic benefits. It is the general impression that cyclophosphamide is more effective than azathioprine in the acute phase of patients with severe disease. the use of cyclophosphamide by i.v. pulse rather than orally is contentious, and some recent studies have demonstrated its failure to induce sustained remission. Azathioprine with low dose corticosteroids is often employed as long-term maintenance immunosuppression, although low dose cyclophosphamide has also been used for such purpose, which should be withdrawn after 1 year of remission because of its potential side-effects. Clinical and serologic parameters are useful monitors during maintenance therapy. Although serial levels of anti neutrophil cytoplasm antibodies (ANCA) correlate with disease activity, some patients remain well despite positive or increasing levels of ANCA. Consequently, whether immunosuppressive therapy should be esclated based on increasing ANCA Levels along remains controversal.     

Hematospermia as the presenting symptom of metastatic malignant melanoma of unknown primary origin

The presence of blood in the ejaculate is alarming to patients and may arise from many sources in the genitourinary tract. Fortunately, hematospermia is rarely due to underlying malignancy and only necessitates further evaluation with continued symptoms. We report a case of persistent hematospermia in a man with human immunodeficiency virus caused by metastatic malignant melanoma to both seminal vesicles. The evaluation included pelvic magnetic resonance imaging and transrectal ultrasound-guided biopsy. Metastases to the brain were subsequently discovered, but a primary source for the melanoma could not be identified. This is the first reported case of isolated involvement of the seminal vesicles with malignant melanoma and underscores the potential for serious disease presenting as hematospermia.     

Relapse rate and outcome of ANCA-associated small vessel vasculitis after transplantation.

BACKGROUND: Anti-neutrophil cytoplasm antibody-associated systemic vasculitis (AASV) is a rare disease and frequently leads to end-stage renal disease (ESRD). Potentially fatal disease activity can develop after the onset of ESRD or in transplanted patients despite the immunosuppressive effects of uraemia and rejection prophylaxis, respectively, leading to concern that such patients may have greater morbidity and mortality. METHODS: To assess the outcome of patients with AASV following kidney transplantation, a retrospective analysis was performed of nine patients with AASV at our unit who received renal transplants between 1987 and 2000. The renal survival of the patients was compared with a control population who received kidney transplants over the same period for causes other than AASV and diabetes mellitus. RESULTS: Nine patients with the diagnosis of AASV (five patients with Wegener's granulomatosis and four with microscopic polyangiitis) received eight cadaveric grafts and one live-related graft after a mean of 44 months from the start of dialysis. These patients had a mean age of 49.2 years at time of transplantation and they were followed up for a mean of 62 months post-transplantation. Two patients with Wegener's granulomatosis suffered a vasculitic relapse affecting the upper respiratory tract at 40 and 50 months post-transplantation, corresponding to a relapse rate of 0.04 per patient per year. The renal transplant function of vasculitis patients compared with 18 non-diabetic control patients who were transplanted at the same time was better in the vasculitis patients at some time points (P=0.054 at 6 months). CONCLUSIONS: There is a substantial relapse rate in the AASV population, especially affecting the upper respiratory tract and with increasing duration of follow-up. Nonetheless, renal transplantation is a good option for the treatment of vasculitis patients and their outcome compares favourably with that of other non-diabetic patients following transplantation.     

Recurrent ANCA-associated small vessel vasculitis after transplantation: A pooled analysis.

BACKGROUND: Recurrent antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis (ANCA-SVV) after renal transplantation has been described in case series. However, general information regarding the frequency, character, and predictors of recurrent disease after transplantation is currently lacking. We considered the rate of relapse, whether a positive ANCA at the time of transplantation predicted relapse, and whether cyclosporine A prevented recurrent disease. METHODS: We performed a pooled analysis of published data, added to the experience at the Universities of North Carolina (14 patients) and Lund, Sweden (11 patients). To avoid reporting bias, only case series were included for analysis. Subgroup analysis was performed by disease category (Wegener's granulomatosis, microscopic polyangiitis, or necrotizing crescentic glomerulonephritis) and ANCA staining pattern. RESULTS: ANCA-SVV recurred in 17.3% of all patients (N = 127), in 20% of cyclosporine A-treated patients (N = 85), and in 25.6% of patients with circulating ANCA at the time of transplantation (N = 39). There was no statistically significant difference in the relapse rate between patients treated and those not treated with cyclosporine A (P = 0.45), between those with and without circulating ANCA at the time of transplant (P = 0.75), or between patients with Wegener's granulomatosis and those with microscopic polyangiitis or necrotizing crescentic glomerulonephritis alone (P = 0.62). CONCLUSION: There is a substantial relapse rate in the ANCA-SVV population. Therapy with cyclosporine A does not protect against recurrent ANCA-SVV, and the presence of a positive ANCA at the time of transplantation does not preclude transplantation. These conclusions must be substantiated with a prospective study of renal transplantation in patients with ANCA-SVV so as to optimize their management.     

No association of G-463A myeloperoxidase gene polymorphism with MPO-ANCA-associated vasculitis.

BACKGROUND: The activation of neutrophils and monocytes by ANCA, resulting in the release of reactive oxygen species and proteases like myeloperoxidase (MPO), is essential to the pathogenesis of ANCA-associated vasculitis. As the A allele of the G-463A MPO gene polymorphism is associated with diminished activity of MPO, it is conceivable that the presence of this allele protects against MPO-ANCA-associated vasculitis. METHODS: Allelic frequencies of the G-463A polymorphism were studied in 119 ANCA-associated vasculitis patients, 48 with MPO-ANCA and 71 with proteinase 3 (PR3)-ANCA. RESULTS: Allelic frequencies of MPO G-463A promoter polymorphism did not differ between MPO-ANCA- and PR3-ANCA-associated vasculitis patients. Moreover, allelic distribution was similar to that of the normal population. CONCLUSIONS: The data suggest that G-463A polymorphism does not seem to contribute to either MPO-ANCA- or PR3-ANCA-associated vasculitis formation.     

Diagnostic laparotomy for fever or abdominal pain of unknown origin

Diagnostic laparotomy performed on twenty-four patients with FUO and twenty-seven patients with obscure abdominal pain resulted in a positive yield of 87 and 82 per cent, respectively. No deaths occurred in either group and the complication rate was minimal. These findings indicate that it is appropriate to include laparotomy in the armamentarium for diagnosis of the cause of FUO and abdominal pain.     

Typhoid fever presenting as histiocytic medullary reticulosis: a case report

Because of a pancytopenia, moderately cellular bone marrow with hypoplastic erythropoiesis but with active granulo- and thrombopoiesis and a conspicuous increase in histiocytic cells, a patient was provisionally diagnosed as having histiocytic medullary reticulosis. However, Salmonella typhi was cultured from blood, urine and stool specimens. After 3 weeks the Widal reaction showed a titre of 1/320 for the H-antigen and 1/1,280 for the O-antigen. The patient was treated with chloramphenicol for 10 days and the fever, enlarged spleen and enlarged lymph nodes returned to normal.