Celiac disease associated with antiphospholipid syndrome

INTRODUCTION: celiac disease may be associated with pathologies of immune etiology. We present its association with antiphospholipid syndrome. CASE 1: a 26-year-old female was diagnosed with celiac disease. Six months later she became pregnant, and experienced fetal death. The following year she became pregnant again. IgG anticardiolipin antibodies: 20 GPL U/ml (normal value < 11), and IgM anticardiolipin antibodies: 9 MPL U/ml (n. v. < 10). Hematological tests were otherwise uneventful. Medicated with acetylsalicylic acid she had a normal pregnancy. CASE 2: a 48-year-old female diagnosed with celiac disease presented with thrombosis in her left lower limb and renal infarction. Hematological tests showed no prothrombotic alterations (antiphospholipid antibodies were not measured). A year and a half later she had thrombosis in a finger of her hand. IgG anticardiolipin antibodies: 10 GPL (n. v. < 13), and IgM anticardiolipin antibodies: 35 MPL (n. v. < 12). CASE 3: a 38-year-old female was diagnosed with celiac disease. Some time later she experienced two spontaneous abortions and a transient ischemic cerebral attack. Nowadays, she is in her sixth month of pregnancy. IgM anticardiolipin antibodies: 75 MPL/ml (n. v. up to 20), and IgG anticardiolipin antibodies within normal values. Hematological tests revealed no other prothrombotic alterations. DISCUSSION: antiphospholipid syndrome is characterized by arterial and venous thrombosis, and spontaneous fetal death. Its association with celiac disease has been described in few cases. Celiac disease is associated with spontaneous fetal death; consequently, we hypothesize that antiphospholipid syndrome may be one of the causes for this event.     

Use of omental pedicle for treatment of Buerger's disease affecting the upper extremities. A modified technique

We present a report of a 58-year-old male patient who suffered from Buerger's disease of the upper extremities verified by microscopy and angiography. Despite medical treatment and repeated thoracic sympathectomy he developed gangrene at the tip of his right thumb and index finger. Pedicled omentum was prepared through midline laparatomy, placed through subcostal incision to the subcutaneous space of the forearm and affected fingers. Rest pain disappeared by the fifth day after surgery. The omental pedicle was ligated six weeks post-operatively and the extremity mobilized. The thumb healed well. The index finger required amputation because of continuing infection. Five years postoperatively the patient has a good functioning hand in which the transferred omentum has retained its viability.     

Arterial reconstruction in Buerger's disease: by-pass to disease-free collaterals.

BACKGROUND: Arterial reconstructions for ischemia in patients with Buerger's disease are technically challenging. This retrospective review was conducted to identify the critical factor for a successful outcome in bypass surgery for Buerger's disease. METHODS: Design of study: retrospective review. Setting: University hospital, hospitalized patients. Patients: since 1993, we performed 10 arterial reconstructions in 8 patients with Buerger's disease. There were 8 tibial artery bypasses and 2 collateral artery bypasses. One bypass was performed in the upper extremity. Interventions: bypass surgery with autogenous vein graft. Main outcomes measurements: graft patency. RESULTS: Over a mean follow-up period of 41.8 months, there were 3 graft occlusions. Of these, 2 were of bypasses to a patent but diseased tibial artery. One graft was occluded due to a toe stenosis which had been previously detected. The 2 collateral artery bypasses were patent at the last follow-up. CONCLUSIONS: In Buerger's disease, distal arterial reconstruction is frequently necessary to prevent ischemic limb loss. Collateral artery bypass is an option when the main arteries are affected by the disease. A patent but diseased artery should be avoided as a target for reconstruction.     

Antiphospholipid antibody syndrome in a patient with neurosarcoidosis.

The case of a 56-year-old woman who developed neurosarcoidosis and was discovered to have inferior vena cava and lower extremity thromboses is described. She was found to have anticardiolipin antibodies. This newly described association of antiphospholipid antibody syndrome with sarcoidosis is discussed and the relevant literature reviewed.     

Buerger's disease with intestinal ischemic involvement

Buerger's disease is uncommon arterial disease that affects mainly young people with heavy smoking history. Ischemic symptoms of upper and lower extremities are clearly defined as the most common kind of presentation. Visceral arteries are rarely affected. We report a case of a 44 year old young female with Buerger's disease and mesenteric ischemic involvement, and a revision of the literature about Buerger's disease with visceral affection. Due to the extreme rarity of the intestinal involvement of Buerger's disease, the early diagnosis is difficult, for this reason all patients affected by Buerger's disease who present intestinal symptoms should be carefully evaluated. Aortography and early laparoscopic revision are very important to limit ischemic intestinal injury and later complications. If hemodynamic instability is present emergency laparotomy at the operating theatre is recommended.     

Vasculitis in the antiphospholipid syndrome. A cause of ischemia responding to corticosteroids

A 63-year-old woman with atherosclerotic peripheral vascular disease and the lupus anticoagulant developed ischemia of the right lower extremity, requiring progressive amputations. Pathologic specimens revealed inflammatory vasculitis in multiple arteries. Her serum showed anticardiolipin antibodies in high titer. Treatment with high-dose corticosteroids reversed the ischemic process. In patients with antiphospholipid antibodies, thrombosis is the most common pathologic finding associated with cutaneous lesions and/or gangrene. Vasculitis, although uncommon, is known to occur and may respond to corticosteroid therapy.     

A young man with nonhealing venous ulcers

A 35-year-old man presented with nonhealing ulcers at an atypical location on his left foot, caused by a combination of venous insufficiency (after deep venous thrombosis) and arterial insufficiency. The underlying cause was Buerger's disease.     

Vasculitis in the antiphospholipid syndrome. A cause of ischemia responding to corticosteroids.

A 63-year-old woman with atherosclerotic peripheral vascular disease and the lupus anticoagulant developed ischemia of the right lower extremity, requiring progressive amputations. Pathologic specimens revealed inflammatory vasculitis in multiple arteries. Her serum showed anticardiolipin antibodies in high titer. Treatment with high-dose corticosteroids reversed the ischemic process. In patients with antiphospholipid antibodies, thrombosis is the most common pathologic finding associated with cutaneous lesions and/or gangrene. Vasculitis, although uncommon, is known to occur and may respond to corticosteroid therapy.     

Buerger's disease or thromboangiitis obliterans revealed by an enteric ischemia. Case report and literature review

Thromboangiitis obliterans or Buerger's disease is a rare arteritis affecting mainly young male smokers. Distal limb symptoms are well-known. Digestive manifestations, especially when inaugural, are less known and sometimes misdiagnosed. We report the case of a 37-year-old man who developed a large mesenteric infarct with diffuse ischemic colitis and ischemia in the hepatic artery field. Histopathological findings were consistent with thromboangiitis obliterans. Unexplained abdominal pain, even if inaugural, in young male smokers, should evoke the possibility of Buerger's disease.     

Factors affecting the long-term outcome of Buerger's disease (thromboangiitis obliterans).

BACKGROUND: Although the age at onset in patients with Buerger's disease is relatively young, the life expectancy has been seldom reported in detail. The aim of this study is to study long-term results of Buerger's disease and factors affecting the ultimate outcome. METHODS: From 1965 to 1980, 682 patients with Buerger's disease were treated in our outpatient department. We studied their long-term status, including concomitant diseases, and the disease progression by mail. RESULTS: Of the 287 mail responders, 266 were male and 21 were female, with a mean age of 60 years. One hundred and fifty-five of these patients are currently suffering from clinical symptoms. Forty-eight patients underwent minor amputation and 30 and major amputation. Forty-six patients underwent sympathectomy, and only 17 bypass reconstruction. Although there was no significant difference in the continuation of symptoms between current smokers and ex-smokers, the amputation rate was higher in current smokers and continuous smoking is closely related to both minor and major amputations after sympathectomy and to minor amputations after drug therapy. Arteriosclerotic diseases were recognized in 57 patients, and gastroduodenal ulcer in 44. Thirty-three patients had died. Among 14 who died of neoplasm, three died of esophageal cancer and lung cancer, respectively, which were closely related to smoking. CONCLUSIONS: The natural history of the limbs in patients with Buerger's disease is not completely discouraging, and in order to obtain a favourable outcome for patients with Buerger's disease we recommend complete smoking cessation with drug-therapy and surveillance for neoplasm, especially of the upper gastrointestinal tract and lung.     

A case of Buerger's disease causing ischemic colitis with perforation in a young male.

A case of Bueger's disease causing ischemic colitis with perforation in a 32-year-old male is presented. Abdominal angiography demonstrated occlusion of the vasculature supplying the involved transverse colon. The association of Buerger's disease with ischemic colitis is emphasized.     

Catastrophic antiphospholipid syndrome: atypical presentation in the setting of chronic graft versus host disease: case report and review of the literature

A 44-year-old female developed a catastrophic thrombotic syndrome following allogeneic stem cell transplantation for acute lymphoblastic leukemia. In the context of chronic graft versus host disease, she developed a lethal multi-system thrombotic state as evidenced by stroke, renal failure and cardiac thrombi in association with elevated anticardiolipin antibody. The case is discussed in the framework of the existing literature and derives clinical practice recommendations for this rare but clinically devastating entity.     

A SERIAL STUDY OF ANTICARDIOLIPIN ANTIBODY AND ANTIMITOCHONDRIAL ANTIBODY TYPE M5 IN A PATIENT WITH POLYARTHRITIS AND POLYMYOSITIS

A 22-year-old female presented with polyarthritis and subsequentpolymyositis. Initially, she had moderately high levels of anticardiolipin(aCl) and antimitochondrial antibody (AMA) type M5. Followingcorticosteroid therapy, the anticardiolipin antibody rapidlyfell to background levels but reappeared with the onset of twosignificant clinical events. KEY WORDS: Anticardiolipin antibody, Antimitochondrial antibody, Polymyositis     

Graves' disease associated with anticardiolipin antibody positivity and acquired protein S deficiency

A 33-year-old woman had experienced recurrent pregnancy loss. She had positive anticardiolipin antibody and protein S deficiency. Her pregnancy was managed with anticoagulant therapy and she delivered a healthy infant. Three years after delivery, she reported progressive sweating, tremor, tachycardia, and a 4-kg weight loss. She was diagnosed with Graves' disease. This is a rare case of combined anticardiolipin antibody positivity, acquired protein S deficiency, and Graves' disease.     

Chagas disease and anticardiolipin antibodies in older adults

Infectious agents have been implicated in the induction of antiphospholipid antibodies and the development of antiphospholipid antibody syndrome. The objective of this study was to evaluate the association of anticardiolipin antibodies with Chagas' disease antibodies. A total of 45 patients, who were positive for Chagas' disease (American Trypanosomiasis) antibodies and had elevated levels of anticardiolipin antibodies, were investigated in a case-control study. Twenty-four of the patients were male and 21 female with ages ranging from 60 to 81 years and with a mean age of 68.3 years. Twenty-three female and 11 male individuals from a senior citizen support group who were apparently healthy formed a control group. Their ages varied from 62 to 80 years with a mean of 68 years. The measurement of anticardiolipin antibodies was performed by means of enzyme-linked immunoabsorbent assay (ELISA) for quantitative measurement of IgG and IgM antibodies against cardiolipins in serum and evaluation of Chagas' disease was confirmed by the Machado Guerreiro test. Statistical analysis was made using Fisher's exact test with a confidence interval of 95% and a p-value <0.05. Elevated levels of anticardiolipin antibodies were detected in 48.8% of the patients and in 26.4% of the control group giving a p-value <0.038 using the Fisher's exact test. Thus, an association between Chagas' disease antibodies and anticardiolipin antibodies was evidenced in this series of patients.     

Successful intravenous regional sympathetic blockade (Bier's Block) with guanethidine and lidocaine in a patient with advanced Buerger's Disease (thromboangiitis obliterans)--a case report

A 65-year-old man, a heavy smoker with Buerger's disease (thromboangiitis obliterans), presented to this department with persistent severe ischemic rest pain at the fingers of his right hand, not responding to oral treatment with vasodilators and analgesics. Critical blood flow was discovered in the middle, ring, and little finger, with ischemic ulcerations apparent in the fingertips of these 3 fingers. The distal phalanx of the little finger had been amputated 6 months before because of gangrenous necrosis. In an attempt to avoid further disabling amputations, the patient received 3 series of Bier's block sessions with guanethidine and lidocaine according to a specific protocol. Marked increase in finger blood flow was induced even after the first series, and complete disappearance of both fingertip ulcerations and ischemic rest pain was achieved. No side effects were observed. The above-described method in a patient with advanced Buerger's disease resulted in excellent pain relief and full restoration of both blood flow and function of the affected fingers.     

Buerger's disease in a young woman

Buerger's disease or thromboangiitis obliterans is characterized by peripheral arterial occlusions in young male cigarette smokers. It is rarely considered in the differential diagnosis of vascular disease in women, although there have been several well-documented cases in the literature. This report presents a young woman with both angiographic and histopathologic evidence for Buerger's disease who was initially treated with daily corticosteroids for presumed vasculitis. This case emphasizes the fact that Buerger's disease can present in a fashion similar to both vasculitis and collagen vascular disease.     

The prostacyclin analog, treprostinil sodium, provides symptom relief in severe Buerger's disease--a case report and review of literature

Buerger's disease (thromboangiitis obliterans or TAO) is a clinical syndrome characterized by the development of segmental thrombotic occlusions of the medium and small arteries. The primary treatment for Buerger's disease is cessation of cigarette smoking. In patients whose disease progresses despite smoking cessation, therapeutic options are limited. Revascularization is rarely indicated and usually not successful because of the diffuse and distal distribution of the disease. Prostacyclin, or PGI2, and its analogs have been proposed as pharmacotherapy for Buerger's disease and studied in Europe. The authors review the prostacyclin literature in the treatment of Buerger's disease and present a case report of a patient with progressive Buerger's disease and the use of treprostinil sodium (Remodulin). This case report experience suggests hat subcutaneous treprostinil therapy could be clinically useful in Buerger's disease that does not improve with smoking cessation, particularly in the presence of critical limb ischemia where other therapeutic options have failed.     

Large vessel occlusion, cerebral infarction and thrombocytopenia in the "primary" antiphospholipid syndrome. Response to anticoagulation

A 65-year-old white female without lupus developed concurrent thrombocytopenia and disturbed arterial circulation to the brain and lower leg (a minor stroke and lower leg gangrene, necessitating amputation). Laboratory studies disclosed high levels of anticardiolipin antibodies. Anticoagulant treatment restored circulation in the remaining leg and also normalized platelet levels. This case emphasizes the importance of searching for anticardiolipin antibodies in unexplained thrombotic events.     

Acute ischemia of the lower extremity in an adolescent with Crohn disease]

The authors report a case with a rare form of peripheral ischaemia. A 25-year-old man with Crohn's disease suffers from sudden ischaemia of his right leg. There is no evidence of entrapment of the popliteal artery, of arterial embolism or Buerger's disease. Thrombocytosis in combination with hypercoagulability as described previously in patients with Crohn's disease seems to be the most probable cause. - In this case percutaneous transluminal thrombectomy and thrombolysis were more successful than surgical thrombectomy using balloon catheters.