iloprost and Gangrene

This review addresses the question of what happens long-term to those systemic lupus erythematosus (SLE) patients who develop gangrene. It also seeks to find common clinical and serological features, risk factors and triggers and how best to manage this challenging complication.. We reviewed 850 patients with SLE attending a UK tertiary referral center, followed up over 44 years, assessing their demographics, clinical and serological features, treatment in the acute phase, their long-term outcome and long-term management.. Ten out of 850 patients (1.2%) developed gangrene; the mean age of onset was 17 years (range 12-26 years) Eight out of 10 patients had a single episode of gangrene. One of the other two was not willing to have anticoagulation. The first episode of gangrene ranged from presentation to 32 years after SLE onset, mean duration of SLE at the onset of the gangrene was 18.5 years SD 11.5 years. Anti-phospholipid (PL) antibodies were over-represented in the patients with gangrene. All had active SLE at the time the gangrene developed. All patients were treated with intravenous (IV) iloprost infusions, and the antiphospholipid-antibody positive patients were anti-coagulated, most staying on long term anticoagulation. Underlying possible triggers were treated appropriately. Two patients who did not respond to the initial treatment needed further immunosuppression. All patients suffered digit loss.. Although rare, gangrene is a sinister, potentially late developing complication of SLE, it rarely recurs. It is associated with anti-phospholipid antibodies, active disease, and other possible triggers such as infection and cancer. Anticoagulation therapy, steroids and iloprost, and further immunosuppression may be needed to stop the evolution of gangrene.

Topics: Adolescent; Adult; Antibodies, Antiphospholipid; Anticoagulants; Child; Follow-Up Studies; Gangrene; Humans; Iloprost; Lupus Erythematosus, Systemic; Young Adult

Twelve patients with systemic sclerosis were treated with intravenous infusions of the prostacyclin-stable analogue iloprost 0.5-2.0 ng/kg/min for 6 h from 8 to 13 days. Imminent gangrene was stopped in 2 patients and followed by healing. In 4 of 6 patients iloprost led to complete healing of ischaemic ulcers and in the remaining 2 patients to partial healing. One patient with severe Raynaud's phenomenon discontinued the study after 3 days due to severe headache. The 2 remaining patients with Raynaud's phenomenon as an indication improved, while no improvement was recorded in a patient with vasculitis of the lower leg. Side-effects such as headache, nausea and flushing were the reason that only 5 patients reached the maximum infusion rate. No statistical differences were recorded in digital bloodflow before and after the study or in plasma endothelin in the 9 patients investigated. Three of the 6 patients with healing ulcers, however, showed a pronounced decrease in plasma endothelin. Iloprost appears useful as a treatment of imminent gangrene and ischaemic ulcers in systemic sclerosis. This reparatory capacity could also be of a more general importance in therapy of this disease.

Topics: Adult; Aged; Endothelins; Female; Fingers; Gangrene; Humans; Iloprost; Infusions, Intravenous; Ischemia; Male; Middle Aged; Raynaud Disease; Scleroderma, Systemic; Ulcer; Vasodilator Agents

A man in his 70s, with a recent diagnosis of transitional cell carcinoma of the bladder, reported a 2-month history of discolouration, pain and paraesthesia affecting his fingers. Clinical assessment noted peripheral acrocyanosis with areas of digital ulceration and gangrene. Following further work-up to evaluate potential causes, he was diagnosed with paraneoplastic acrocyanosis. He proceeded to undergo robotic cystoprostatectomy and received adjuvant chemotherapy for the management of his cancer. In parallel to the chemotherapy, vasodilatory therapy was administered as two courses of intravenous synthetic prostacyclin analogue iloprost along with sildenafil. This resulted in a significant improvement in digital pain and gangrene with healing of ulceration.

Topics: Epoprostenol; Gangrene; Humans; Iloprost; Male; Sildenafil Citrate; Vasodilator Agents

Symmetric peripheral gangrene (SPG) is a symmetrical distal ischemic lesion on at least 2 or more extremities in the absence of proximal arterial obstruction and vasculitis. It is a rare and severe clinical entity. The aim of this study was to describe clinical symptoms, etiological agents and the management of SPG through a series of 4 cases.. We included all cases of SPG hospitalized between 2000 and 2014. The inclusion criterion was the presence of distal ischemic damage at two or more sites in the absence of large vessel obstruction.. Four patients (2 men and 2 women) were included. The mean age was 43.2±12 years. Two patients had a history of splenectomy. All patients had blackening of the tips of the fingers and toes. Three patients presented with septic shock. The etiology was bacteremia involving Streptococcus pneumoniae in two cases and a malignant form of Mediterranean spotted fever (MSF). In addition to specific antibiotics, we used a potent vasodilator (iloprost) in two cases and curative heparin therapy in two cases. The outcome was favorable in 3 cases, with regression of necrotic lesions. One case required the amputation of non-perfused necrotic fingers and toes.. SPG can complicate MSF in some rare cases. Thorough and repeated skin examinations are essential to ensure timely diagnosis and treatment of GPS in order to improve the prognosis.

Topics: Adult; Amputation, Surgical; Anti-Bacterial Agents; Boutonneuse Fever; Female; Fibrinolytic Agents; Fingers; Gangrene; Heparin; Humans; Iloprost; Male; Pneumococcal Infections; Retrospective Studies; Shock, Septic; Toes; Vasodilator Agents

A patient with digital ischaemia and gangrene was treated with iloprost and antiplatelets for two weeks. His vasculitic screen was negative except for a positive HIV test. His vasculitis improved three weeks after treatment with antiretroviral medications. Though vasculitis is well known to be associated with HIV infection, very few cases of HIV present as vasculitis.

Topics: Adult; Aged; Anti-Retroviral Agents; Female; Fingers; Gangrene; HIV Infections; Humans; Iloprost; Male; Treatment Outcome; Vasculitis

Raynaud's phenomena is a common disorder which may be primary or secondary to some connective tissue disorders such as systemic sclerosis and systemic lupus erythematosus. Jellyfish sting is a rare but life-threatening cause of Raynaud's phenomena. Digital gangrene is reported in 3% of children with secondary Raynaud's phenomena but does not occur in children with primary Raynaud's phenomena. We report a case of a 4-year-old boy who initially presented with episodes of pain and bluish to blackish discolouration and necrosis affecting the fingers on both hands after a jellyfish sting without any sign of connective tissue disorder.

Topics: Animals; Bites and Stings; Child, Preschool; Fingers; Gangrene; Humans; Iloprost; Male; Raynaud Disease; Scyphozoa; Vasodilator Agents

The objective of this study was to evaluate the incidence of the most severe vascular complications, such as pulmonary arterial hypertension, scleroderma renal crisis, and digital necrosis requiring amputation, in a monocentric group of systemic sclerosis (SSc) patients cyclically treated with intravenous iloprost. We reviewed the record-charts of 115 patients affected by SSc (18 men and 97 women, mean age 58.9.1 ± 14.2 years) regularly receiving iloprost for at least 3 years; the mean duration of the treatment was 98.8 ± 37.5 months (a total of 946.8 years of therapy). Demographic and clinical features were recorded. None of the patients died of SSc-associated vascular complications. After iloprost administration digital gangrene requiring amputation developed in 2 patients who had concomitant peripheral arterial disease (a total of 3 episodes; annual incidence of 0.31 for 100 years of iloprost therapy). Four patients were diagnosed with pulmonary arterial hypertension during iloprost treatment (annual incidence of 0.42 for 100 years of drug therapy); in none of the cases did the complication show a progressive course. No cases of scleroderma renal crisis were observed. With the limits of an observational study and in the absence of a control group, our experience suggests that prolonged cyclic iloprost therapy may limit the incidence/progression of severe digital and visceral SSc-vasculopathy.

Topics: Adult; Aged; Amputation, Surgical; Familial Primary Pulmonary Hypertension; Female; Finger Injuries; Gangrene; Humans; Hypertension, Pulmonary; Iloprost; Male; Middle Aged; Peripheral Arterial Disease; Scleroderma, Systemic; Severity of Illness Index; Toes; Vasodilator Agents

Topics: Angiogenesis Inducing Agents; Child, Preschool; Combined Modality Therapy; Cross Infection; Debridement; Fatal Outcome; Fingers; Gangrene; Humans; Iloprost; Infant; Ischemia; Male; Meningitis, Meningococcal; Multiple Organ Failure; Nitroglycerin; Platelet Aggregation Inhibitors; Shock, Septic; Staphylococcal Infections; Toes; Tuberous Sclerosis; Vasodilator Agents

A 4 year-old boy was admitted to our clinic with symptoms of pain and ecchymosis in his right leg and foot after injection of benzathine penicilline. There was a localized gangrenous area at the femoral injection site. Doppler ultrasonography showed no arterial flow in the femoral artery and clear evidence of acute thrombosis of the superficial femoral and popliteal veins. Femoral arterial and venous thrombectomy and fasciotomy were performed immediately. After surgery the boy was treated by Iloprost infusion and enoxaparine. One week later necrotic changes had regressed, fasciotomies were closed and only the distal phalanx of the third toe needed amputation. Early surgical intervention and standard management combined with Iloprost infusion may help in healing the lesions by increasing extremity perfusion and may prevent extremity loss.

Topics: Amputation, Surgical; Anti-Bacterial Agents; Anticoagulants; Cardiovascular Agents; Child, Preschool; Combined Modality Therapy; Drug Eruptions; Enoxaparin; Fasciotomy; Gangrene; Humans; Iloprost; Infusions, Intravenous; Injections; Leg; Male; Penicillin G Benzathine; Thrombectomy; Toes; Treatment Outcome; Venous Thrombosis

We report one new case of hemolytic-uremic syndrome (HUS) and one case of digital necrosis after treatment with gemcitabine (Gemzar). Case 1, a 34-year-old man, was given first-line metastatic treatment with gemcitabine for a adenocarcinoma of the pancreas. After a cumulative dose of 10 000 mg/m2 gemcitabine, the onset of subacute renal failure associated with hemolytic anemia of mechanical origin was observed. A diagnosis of probable gemcitabine-induced thrombotic microangiopathy was arrived at. Symptoms resolved after stopping the chemotherapy, in spite of the progression of the disease. Case 2, a 61-year-old man, was administered a combination of gemcitabine and a platinum salt as first-line metastatic treatment for carcinoma of the bladder urothelium. Following a cumulative dose of 10 000 mg/m2 of gemcitabine, the patient suffered from bilateral peripheral vascular disease of somewhat acute onset with hemorrhagic lesions of the finger pads that became necrotic. The work-up was negative and a causal relationship was attributed to gemcitabine. The patient made good progress when given an i.v. infusion of Ilomedine (iloprost trometamol) and chemotherapy was withdrawn. We conclude that gemcitabine must be added to the list of drugs that cause HUS and necrotizing vasculitis.

Topics: Adenocarcinoma; Adult; Antimetabolites, Antineoplastic; Carcinoma, Transitional Cell; Deoxycytidine; Fingers; Gangrene; Gemcitabine; Hemolytic-Uremic Syndrome; Humans; Iloprost; Male; Middle Aged; Pancreatic Neoplasms; Urinary Bladder Neoplasms; Vasodilator Agents

Topics: Aged; Female; Fingers; Follow-Up Studies; Gangrene; Humans; Iloprost; Raynaud Disease; Treatment Outcome; Vasodilator Agents

Systemic necrotizing vasculitis is uncommon in children and may be rarely associated with gangrene. We describe a 3-yr-old girl with parvovirus B19-induced necrotizing vasculitis whose digital gangrene was successfully treated with iloprost, a prostacyclin analogue.

Topics: Antibodies, Viral; Child, Preschool; Female; Fingers; Gangrene; Humans; Iloprost; Parvoviridae Infections; Parvovirus B19, Human; Platelet Aggregation Inhibitors; Polyarteritis Nodosa

Topics: Arterial Occlusive Diseases; Chronic Disease; Gangrene; Humans; Iloprost; Infusions, Intra-Arterial; Leg; Male; Middle Aged

Platelet sensitivity to PGI2 and platelet PGI2 receptors were investigated in eight subjects with peripheral artery disease (stage IV according to Fontaine) treated for 14 consecutive days with six hour iv infusion of Iloprost (Schering, FRG) 2 ng/kg/min. Platelet studies were performed on the 1st, the 2nd, the 7th and the 14th day of therapy, immediately before infusion (between 8.00 and 9.00 a.m.), at the end and 6 and 18 hours (the following morning) after the end of the infusion. Platelet sensitivity to PGI2 was assessed by determining the PGI2 inhibitory dose 50(ID 50) on platelet aggregation induced by 5 microM ADP. PGI2 platelet receptors were investigated by a direct radioligand binding assay. PGI2 ID 50 after the infusion was significantly higher than that at baseline(p less than 0.01) and six hours later the baseline sensitivity was restored. After the six hour Iloprost iv infusion a significant reduction in the number of high affinity PGI2 platelet receptor (HAR) was observed (p less than 0.005) without any change in their affinity for the ligand. Six hours after the end of the infusion the number of the HAR was still significantly reduced (p less than 0.05). The following morning the receptor number of HAR was restored. The baseline values of PGI2 HAR, when reassessed after seven and fourteen days of treatment, were not significantly different from those recorded on the first day of therapy. These data indicate that the reduction of platelet PGI2 sensitivity following short-term Iloprost infusion is rapidly reversible and is related to a contemporary down-regulation of PGI2 platelet receptors.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Blood Platelets; Epoprostenol; Gangrene; Humans; Iloprost; Platelet Aggregation; Receptors, Epoprostenol; Receptors, Prostaglandin