Respiratory failure in ANCA-associated vasculitis.

OBJECTIVE: To assess the prevalence, clinical manifestations, and course of respiratory failure in all patients who tested positive for antineutrophil cytoplasmic autoantibodies (ANCA) in our clinics in the period between January 1985 and January 1993. DESIGN: Case-series analysis. SETTING: Three teaching hospitals in the Netherlands. PATIENTS: Two hundred twenty consecutive patients suspected of having vasculitis and/or glomerulonephritis who tested positive for ANCA by indirect immunofluorescence and enzyme-linked immunosorbent assay. RESULTS: Sixty-two patients had pulmonary involvement. Acute respiratory failure developed in nine. Respiratory failure was related to infections in two of them and to ANCA-associated vasculitis in seven. These seven patients uniformly presented with pulmonary hemorrhage and diffuse pulmonary infiltrates. The diagnosis of systemic vasculitis was supported by the presence of a pulmonary-renal syndrome in all patients, and by detection of antibodies to the proteinase 3 or myeloperoxidase antigen in all but one patient. Antiglomerular basement membrane antibodies were absent. The mortality was high due to hypoxic respiratory failure, pulmonary superinfections, and concomitant renal failure. CONCLUSIONS: Acute respiratory failure due to vasculitis developed in one of every nine patients with ANCA-associated pulmonary disease. Patients usually present with pulmonary infiltrates and hemoptysis. A diagnosis of vasculitis may be further supported by analysis of the urinary sediment and determination of the ANCA target antigen. It remains to be proved that early detection of ANCA favorably affects the outcome.

Transfusion-related acute lung injury.

Noncardiogenic pulmonary edema after transfusion therapy is an infrequent but hazardous complication. The occurrence of this entity is linked to the presence of circulating leukoagglutinins. The clinical features are described on the basis of four cases. The hemodynamic changes, underlying mechanisms and therapeutic strategies are discussed.

Autoantibodies against constituents of neutrophils in the diagnosis and treatment of (isolated) subglottic stenosis.

Six patients had a subglottic stenosis either as a presenting symptom or as a manifestation of a systemic disease. All patients had in common the presence of circulating autoantibodies against constituents of neutrophils on indirect immunofluorescence. Cytoplasmic and perinuclear staining patterns were recognized. Such autoantibodies have been reported in Wegener's granulomatosis, microscopic polyarteritis, (idiopathic) glomerulonephritis, and Churg-Strauss syndrome. However, only one of the six patients fulfilled the criteria for these conditions. Because a positive test for autoantibodies against constituents of neutrophils is rare in other conditions and because other diseases had been excluded, we suggest that this places subglottic stenosis within the spectrum of necrotizing (granulomatous) vasculitis. The consequences for therapy are discussed.

The hemodynamic effect of different ultrafiltration rates in patients with cardiac failure and patients without cardiac failure: comparison between isolated ultrafiltration and ultrafiltration with dialysis.

OBJECTIVE: The increasing number of dialysis patients with cardiovascular diseases will lead to an increase in the incidence of intradialytic hypotension. Intradialytic hypotension is determined by changes in plasma volume, changes in vascular reactivity and structural cardiovascular changes. In this study the effect of two different ultrafiltration rates (UF-rate), i. e. 500 and 1000 ml/h, on plasma volume, extracellular volume and arterial blood pressure was studied during different treatments of 2 hours combined ultrafiltration + hemodialysis (UF+HD) and 2 hours isolated ultrafiltration (i-UF). PATIENTS AND METHODS: 15 Patients, 8 patients with cardiac failure, CFpts (NYHA classification III and IV) and 7 patients without cardiac failure (NCFpts) were investigated during a standardized dialysis treatment. RESULTS: The decrease in plasma volume and decrease in extracellular volume was comparable both between i-UF and UF+HD and comparable between CFpts and NCFpts and was only dependent on the UF-rate. i-UF resulted in minor blood pressure changes in both CFpts and NCFpts. In CFpts UF+HD resulted in a significant decrease in systolic blood pressure (SBP) at both UF-rates while in NCFpts SBP decreased significantly only at the higher UF-rate during UF-HD. Although there were no significant differences in hemodynamic stability during the different treatment modalities between CFpts and NCFpts, the decrease in SBP in CFpts at the higher UF-rate during UF+HD was much more pronounced. CONCLUSION: From this clinical study we conclude that differences in hemodynamic stability between i-UF and UF+HD and between CFpts and NCFpts are not related to differences in plasma volume preservation. Other factors like different changes in vascular reactivity and in CFpts structural cardiovascular changes might be responsible for the observed differences.

Acute renal failure in theophylline poisoning.

A 57-yr-old woman with an intentional theophylline overdose complicated by rhabdomyolysis, renal failure and microangiopathic haemolytic anaemia is reported. Severe renal vasoconstriction, myoglobinuria and antagonism of adenosine are considered to be the principal pathophysiological renal mechanisms involved.

Acute pernicious or fulminating beriberi heart disease. A report of 6 patients.

OBJECTIVE: To investigate the incidence and presentation of acute pernicious or fulminating beriberi in a general district hospital. METHODS: All patients with a diagnosis of acute pernicious or fulminating beriberi heart disease made between 1978 and 1994 were identified, and their medical records were retrospectively examined. RESULTS: Six cases of acute pernicious or fulminating beriberi heart disease were recognized. The disease was characterized by circulatory shock and peripheral cyanosis. All patients had severe lactic acidosis, in 5 of them without hypoxaemia. Four patients were alcoholics. The most important diagnostic criterion was the impressive improvement after thiamine administration. CONCLUSIONS: Acute pernicious or fulminating beriberi heart disease can be recognized occasionally. Thiamine should be administered as soon as possible in suspected cases.

Endocrine organ failure due to systemic AA-amyloidosis.

We describe a patient with panhypopituitarism and adrenal insufficiency associated with systemic AA-amyloidosis caused by tuberculosis. This case demonstrates the ongoing process of amyloidosis, despite a presumed cure for the tuberculosis more than 30 years previously. Difficulties in recognizing clinical symptoms and interpreting laboratory data in a patient on regular haemodialysis are discussed.

Acquired angio-oedema caused by IgA paraprotein.

The syndrome of acquired angio-oedema is characterized by late onset of recurrent bouts of angio-oedema or abdominal pain and may be caused by an acquired deficiency of C1-inhibitor (C1-INH), the inhibitor of the first component of complement. Acquired C1-INH deficiency has been described in approximately 50 patients and is strongly associated with malignant B-cell proliferations. We describe a patient with an 8-year history of recurrent abdominal symptoms and angio-oedema with acquired C1-INH deficiency, caused by the presence of IgA-kappa antibodies that inactivate C1-INH. Analysis of the bone marrow revealed an IgA-kappa monoclonal population of plasma cells, without evidence of overt myeloma. Angio-oedema caused by an autoantibody of the IgA isotype is extremely rare and has never been described in a Dutch patient. Recognition of angio-oedema, both hereditary and acquired, is important because of the therapeutic consequences, as will be discussed.

Hydralazine-induced constrictive pericarditis.

A 59-year-old man was diagnosed as having constrictive pericarditis 17 months after a typical hydralazine-induced autoimmune syndrome. This late complication of hydralazine has been reported only once. Ten years later the patient was found to have anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase.

The inflammatory abdominal aortic aneurysm. Prevalence, clinical features and diagnostic evaluation.

Eight patients with an inflammatory abdominal aortic aneurysm are described. They constituted 3.1% of all aortic aneurysms operated in the period 1981-1990. All presented with abdominal complaints and/or backache, and an elevated erythrocyte sedimentation rate (ESR); 3 had significant weight loss. The discovery of the aneurysm was considered a non-related finding except for 2 patients for whom the correct diagnosis was suggested by CT scan. Except for the aortic dilatation, the clinical and histopathological features of the inflammatory aneurysm resembled those of idiopathic retroperitoneal fibrosis with obstruction of the ureters and involvement of the duodenum by extensive perianeurysmal inflammation with infiltrates of mononuclear leukocytes and varying degrees of fibrosis. Although the triad of abdominal complaints and/or backache, an elevated ESR and weight loss has been proposed to suggest the diagnosis, the positive predictive value of these parameters was at best 22%. Additional studies, including CT scan, have to be performed to exclude, among other factors, vasculitis. Treatment should aim at repair of the aneurysm. Resolution of the inflammatory process after operation is frequently observed. Immunosuppressive therapy should only be used with caution, considering the possible increased risk of rupture.

[Diagnosis and prognosis of polymyalgia rheumatica and giant cell arteritis]. / Diagnostiek en prognose van polymyalgia rheumatica en reuzencel-arteriitis.

Retrospectively the data were examined of 69 patients with polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA), of whom 62 were treated with corticosteroids. The clinical and laboratory data, and the outcome of temporal artery biopsy were compared. In addition the relation between the course of the disease and survival was investigated. PMR and GCA are closely related syndromes with a multiform clinical presentation. There even is a group of patients with merely systemic symptoms, without specific signs of PMR or GCA. Concerning the course of the disease patients can be divided into groups with a shorter and longer period of disease activity; patients with a smooth stable remission and a chronic disease. The former group shows a significantly better five-year survival. At the time of diagnosis no reliable prediction of the course of the disease is possible.

[Myositis in the presence of slightly elevated creatine kinase values]. / Myositis bij een normale of licht verhoogde creatinekinasewaarde.

The clinical picture with, among other things, muscular swelling, fever and nocturnal sweating in three males aged 30, 37 and 52 years, suggested a neoplasm and sepsis. Ultimately, they were found to suffer from focal myositis, localized nodular myositis and polymyositis, respectively. The ESR and leukocyte counts were increased, the serum creatinine kinase (CK) activity was normal or slightly increased. The symptoms decreased after prednisone treatment. Both localized and generalized inflammatory muscular diseases may be present without the serum CK activity being raised.

Dutch guideline for the management of electrolyte disorders--2012 revision.

Electrolyte disorders are common and often challenging in terms of differential diagnosis and appropriate treatment. To facilitate this, the first Dutch guideline was developed in 2005, which focused on hypernatraemia, hyponatraemia, hyperkalaemia, and hypokalaemia. This guideline was recently revised. Here, we summarise the key points of the revised guideline, including the major complications of each electrolyte disorder, differential diagnosis and recommended treatment. In addition to summarising the guideline, the aim of this review is also to provide a practical guide for the clinician and to harmonise the management of these disorders based on available evidence and physiological principles.

Renal abnormalities in a family with Alagille syndrome.

Alagille syndrome is largely unknown to the general internist because the diagnosis is usually made by a paediatrician. Nevertheless, it is important to be aware of this syndrome because it sometimes manifests later in life with a great variability in clinical presentation and important consequences for the individual patient. We therefore discuss this syndrome using a patient with the usual characteristics of this syndrome.