Streptococcus pyogenes
Peter M. Lydyard, Michael F. Cole, John Holton, William L. Irving, Nino Porakishvili, Pradhib Venkatesan, Katherine N. Ward in Case Studies in Infectious Disease, 2010
Systemic causes of glomerular nephritis: Wegener granulomatosis.Hypersensitivity vasculitis.Cryoglobulinemia.Systemic lupus erythematosus.Polyarteritis nodosa.Henoch-Schönlein purpura.Goodpasture syndrome.Renal diseases.Membranoproliferative glomerulonephritis.
“Primary” Anti-Phospholipid Syndrome
E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson in Phospholipid-Binding Antibodies, 2020
The largest series documented was from Asherson et al. comprising 70 patients and the frequency of clinical and serological abnormalities are shown in the accompanying tables (Tables 1 and 2); 54% suffered from episodes of deep venous thrombosis (DVT). These were accompanied by pulmonary thromboembolism in one third. Two patients suffered from pulmonary hypertension (PHT); one had developed this complication following repeated pulmonary thromboembolism; in two others it resembled the “primary” nonthromboembolic variety. IVC thrombosis and a Budd Chiari syndrome were present in two patients. Arterial occlusions were present in 44%, strokes and TIAs being most frequently encountered (50%). Multi-infarct dementia had followed in four. Myocardial infarctions were seen in five patients; occlusion of the aorta in one, multiple visceral arterial occlusions in one, renal infarction in one and renal artery stenosis in one. Subclavian occlusions were present in two and gangrene of the toes occurred in two. Recurrent fetal loss was present in 34%; 46% were ANA positive and antibodies to mitochondria (type MS) were present in 11 of 40 tested. Thirty-two patients had been thrombocytopenic at some stage of their illness. Menorrhagia was encountered in one patient only in whom the platelet count had fallen to 5.10 x 109/1. A false-positive VDRL was present in 17 of 51 patients tested. Coombs positivity occurred in 10, but hemolytic anemia had developed in only three. Six patients demonstrated cryoglobulinemia.
High-Dose Chemotherapy with Haematopoietic Stem Cell Transplantation in Primary Systemic Vasculitis, Behcet’s Disease and Sjogren’s Syndrome
Richard K. Burt, Alberto M. Marmont in Stem Cell Therapy for Autoimmune Disease, 2019
The international experience with HSCT in autoimmune diseases was presented at an international meeting in October 2000 in Basel, Switzerland and is described in the present book. At the Basel meeting, data on 390 patients from all over the world were reported. This combined international data base included 260 patients from the EBMT/EULAR Basel European/Asian database, 87 from North America (55 from the IBMTR), 39 from Australia and 4 others.1 Experience with PSV remains anecdotal at present. Within this database, 9 cases of systemic vasculitis including Behcet’s syndrome were reported. In 3 cases of Wegener’s granulomatosis, initially complete remissions were observed. Two of them relapsed at 2 and 3 years, respectively. In two of three patients with cryoglobulinemia, with variable vasculitic features, complete responses were reported (Table 5). Three patients with Behcet’s syndrome were treated by HSCT, two of them in our institution. In one case there was a relapse following autologous HSCT, and this patient then received an allogeneic HSCT from her HLA-identical brother.34a For Sjogren’s syndrome, only one patient was reported. However, there are several reports of HSCT in patients with Sjogren’s syndrome and consecutive lymphoma. Unfortunately, in these reports, mainly no response or only short term response of the autoimmune disease, in contrast to the lymphoma, was described.35,36 However, one paper reports a lasting remission of nonerosive polyarthritis in Sjogren’s syndrome after HSCT for lymphoma.37
Managing complications secondary to Waldenström’s macroglobulinemia
Published in Expert Review of Hematology, 2021
Ilias Pessach, Meletios A. Dimopoulos, Efstathios Kastritis
Cryoglobulins are serum proteins or protein complexes that precipitate at low temperatures. In type I cryoglobulinaemia, monoclonal IgM precipitates with 10% to 20% of patients with WM having positive test, but less than 5% being symptomatic because of these cryoglobulins [48]. Symptoms of cryoglobulinemia may include acrocyanosis, Raynaud’s phenomenon, palpable purpura, or glomerulonephritis and in view of these complications one should consider PLEX (through a heated circuit) followed by systemic treatment to achieve long-term control [49]. IgM flare may complicate therapy with anti-CD20 MoAbs and can aggravate symptoms, so that preemptive PLEX may be considered [50]. Ibrutinib may be a reasonable therapeutic option, PIs may also reduce IgM levels before initiation of anti-CD20 [51] and BR is another active 1st line treatment option [46,47].
Treatment of systemic necrotizing vasculitides: recent advances and important clinical considerations
Published in Expert Review of Clinical Immunology, 2019
Christian Pagnoux, Arielle Mendel
The diagnosis of CV is usually made by identifying the presence of serum cryoglobulins in a patient with suggestive small-sized–vessel vasculitic manifestations, mostly affecting the peripheral nerves (sensorimotor polyneuropathy or mononeuritis multiplex), skin (purpura, livedo, gangrene) and kidneys (membranoproliferative glomerulonephritis). Type I cryoglobulinemia is usually due to multiple myeloma, Waldenstrom’s macroglobulinemia, B-cell lymphoma, and/or chronic lymphocytic leukemia. HCV-associated CV is one of the most common causes of type II (90% of type II) or type III (70% of type III) cryoglobulinemia [91–93]. After HCV, the most common causes of mixed (type II or III) cryoglobulinemia are autoimmune diseases (mainly Sjögren’s syndrome), lymphoproliferative disorders, and other infections.
Cutaneous small vessel vasculitis
Published in Postgraduate Medicine, 2023
Cryoglobulinemia is the presence of immunoglobulins that precipitate in the cold and resolubilize with warmer temperatures. There are three types based on immunochemical analysis. Monoclonal or type I, mixed or type II, and polyclonal or type III. Vasculitis is only seen in types II and III. They most commonly present with purpura. Half of the patients have peripheral neuropathy and one third have renal involvement. Mixed is generally secondary to hepatitis C infection, and it is considered the main cause accounting for 80–85% of the cases in multiple studies [20]. Type II also can be associated with autoimmune connective tissue disorders and type III with lymphoproliferative disorders [15].
Related Knowledge Centers
- Antibody
- Cold Agglutinin Disease
- Precancerous Condition
- Purpura
- Autoimmune Disease
- Infection
- Red Blood Cell
- Malignancy
- Cold Sensitive Antibodies
- Gangrene