313. FULMINANT IGA VASCULITIS AS A CATASTROPHIC ANCA-NEGATIVE SMALL VESSEL VASCULITIS

Small vessel vasculitis is vasculitis affecting predominately small intraparenchymal arteries, arterioles, capillaries, and venules. There are two main types: antineutrophil cytoplasmic antibody associated and immune complex mediated. The ANCA associated vasculitides are discussed in chapter 19.3 IgA vasculitis (IgAV) was formerly known as Henoch Schönlein purpura. The revised nomenclature reflects the importance of IgA vasculitis in pathogenesis. The Chapel Hill Consensus Conference defined IgA vasculitis as ‘vasculitis with IgA1-dominant immune deposits, affecting small vessels (predominantly capillaries, venules, or arterioles)’. IgA vasculitis often involves skin and gut, and frequently causes arthritis. Glomerulonephritis indistinguishable from IgA nephropathy may occur. Its aetiology is unknown, but it frequently occurs after an infection several days to weeks before. The most frequently isolated organism is beta-haemolytic streptococcus. Drugs such as a penicillin, ampicillin, erythromycin, and non-steroidal anti-inflammatory drugs have been reported as precipitating agents. There is an association with HLA-DRB1*01 in Caucasians and there appears to be a familial association.

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Heparin-induced thrombocytopenia during IgA vasculitis: a case report

Oxford Medical Case Reports ◽

10.1093/omcr/omab002 ◽

2021 ◽

Vol 2021 (3) ◽

Author(s):

Kaisei Yamasaki ◽

Moe Kyotani ◽

Yasuyo Urase ◽

Yoichi Goto ◽

Tsuneaki Kenzaka

Keyword(s):

Platelet Count ◽

Case Report ◽

Renal Disease ◽

Immune Complexes ◽

Immunoglobulin A ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Iga Vasculitis ◽

Heparin Induced Thrombocytopenia ◽

Leg Edema

ABSTRACT Immunoglobulin A (IgA) vasculitis is characterized by small vessel vasculitis involving immune complexes and IgA deposition. The development of heparin-induced thrombocytopenia (HIT) during IgA vasculitis is extremely rare. An 87-year-old man presented with general fatigue, leg edema, purpura, arthritis and renal disease. He was diagnosed with IgA vasculitis and was admitted to our hospital. Hemodialysis with heparin was initiated thrice a week on post-admission Day 11. On Day 21, during hemodialysis, the pressure in the dialysis circuit increased and the dialysis was interrupted. On Day 24, the platelet count rapidly decreased to 18 000/μl. The patient was diagnosed with HIT after testing positive for HIT antibodies; heparin was discontinued at this time. Precautions must be taken against the onset of HIT when initiating hemodialysis in patients with IgA vasculitis.

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Don't Discount Severity of Small-Vessel Vasculitis

Rheumatology News ◽

10.1016/s1541-9800(07)70598-7 ◽

2007 ◽

Vol 6 (11) ◽

pp. 9

Author(s):

DIANA MAHONEY

Keyword(s):

Small Vessel ◽

Small Vessel Vasculitis

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CLIPPERS With Chronic Small Vessel Damage: More Overlap With Small Vessel Vasculitis?

Journal of Neuropathology & Experimental Neurology ◽

10.1097/nen.0000000000000050 ◽

2014 ◽

Vol 73 (3) ◽

pp. 262-267 ◽

Cited By ~ 13

Author(s):

B.K. Kleinschmidt-DeMasters ◽

Matthew West

Keyword(s):

Small Vessel ◽

Small Vessel Vasculitis ◽

Vessel Damage

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Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽

10.3390/pathogens10010031 ◽

2021 ◽

Vol 10 (1) ◽

pp. 31

Author(s):

Céline Betti ◽

Pietro Camozzi ◽

Viola Gennaro ◽

Mario G. Bianchetti ◽

Martin Scoglio ◽

...

Keyword(s):

Mycoplasma Pneumoniae ◽

Legionella Pneumophila ◽

Leukocytoclastic Vasculitis ◽

Systemic Vasculitis ◽

Bacterial Pathogen ◽

Small Vessel ◽

Small Vessel Vasculitis ◽

Systemic Involvement ◽

Symptomatic Disease ◽

Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

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