Diffuse Alveolar Hemorrhage in IgA Vasculitis with an Atypical Presentation

Ito, Yuhei; Arita, Machiko; Kumagai, Shogo; Takei, Reoto; Noyama, Maki; Tokioka, Fumiaki; Nagamoto, T.; Kawakita, Chieko; Asano, Kenichiro; Okita, Chika; Ishida, Tadashi

doi:10.2169/internalmedicine.8984-17

Cited by 8 publications

(6 citation statements)

References 20 publications

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“…21 The skin, gastrointestinal system, and the joints are frequently involved, while DAH is a rare manifestation. 96 IgAV mainly affects children between the age of 4 and 7 years. It has also been described in adults and in the elderly with DAH.…”

Section: Immunoglobulin a Nephropathymentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Nasser

Cottin

2018

Semin Respir Crit Care Med

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Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue, also excluding infection and other causes of hemoptysis. Although not recommended, lung biopsy is the gold standard for the diagnosis of DAH and pulmonary capillaritis. Pulmonary capillaritis may be primary as in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis or secondary to drugs (especially antithyroid drugs such as propylthiouracil), infections, connective tissue diseases especially systemic lupus erythematosus, or other small vessel vasculitides. Newer toxic causes of drugs of abuse may be difficult to diagnose. Granulomatosis with polyangiitis and microscopic polyangiitis are the most common causes of capillaritis and DAH, whereas DAH is extremely rare in eosinophilic granulomatosis with polyangiitis. When pulmonary capillaritis is not secondary to underlying systemic vasculitis, idiopathic pauci-immune pulmonary capillaritis may be considered, with or without ANCA. Supportive treatment strategy is mandatory in all cases of DAH. Mechanical ventilation and extracorporeal membrane oxygenation may be used in severe cases. Early identification and removal of the putative drug is crucial in drug-induced vasculitis/DAH and may obviate the need for immunosuppressive therapy. High-dose corticosteroids, intravenous cyclophosphamide, and recently rituximab are the mainstay of treatment in vasculitis. Plasma exchange is recommended in anti–glomerular basement membrane disease and in severe DAH associated with systemic lupus erythematosus and is used in selected cases in ANCA-associated vasculitis.

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Section: Immunoglobulin a Nephropathymentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Nasser

Cottin

2018

Semin Respir Crit Care Med

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“…The renal symptoms in IgA nephropathy and IgA vasculitis are virtually indistinguishable [ 8 ]. The main differences are that IgA vasculitis typically also presents with skin manifestations (purpura), GI manifestations (abdominal pain), and arthralgias [ 2 ], none of which were present in this patient. In IgA vasculitis, it is rare to have pulmonary involvement, although DAH is the most common form [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Immunoglobulin A (IgA) nephropathy is a condition in which immune complexes deposit in the small blood vessels of the kidney [ 1 ]. IgA vasculitis (formerly known as Henoch-Schonlein purpura) typically causes IgA nephropathy and commonly affects the skin, gastrointestinal tract, and joints [ 2 ]. It is rare to have pulmonary symptoms, although diffuse alveolar hemorrhage (DAH) has been associated with the disease [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

A Case Report of Diffuse Alveolar Hemorrhage Coexisting With Immunoglobulin A (IgA) Nephropathy

Kolman¹,

Song²,

Han³

et al. 2022

Cureus

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Immunoglobulin A (IgA) nephropathy is the most common cause of primary glomerulonephritis worldwide. IgA vasculitis (formerly known as Henoch-Schonlein purpura) typically presents with IgA nephropathy on renal biopsy in addition to extrarenal symptoms like purpura, abdominal pain, and arthritis. Diffuse alveolar hemorrhage (DAH) is the most common pulmonary complication, but this is rarely seen. In this case report, we describe a 35-year-old male with chronic untreated hepatitis B infection who presented with pulmonaryrenal syndrome. He was found to have clinical findings of DAH and concomitant IgA nephropathy on renal biopsy, without having any other typical manifestations of IgA vasculitis. This shows that IgA nephropathy should be considered in the differential diagnosis of DAH and emphasizes the importance of a renal biopsy in patients presenting with pulmonary-renal syndrome.

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“…Burada yaygın purpurik döküntü, gastrointestinal kanama ve plevral efüzyonla seyreden HSP'nin akciğer ve gastrointestinal tutulumu olan bir vaka sunuldu. Solunum sistemi tutulumu nadir olmakla birlikte mortalite ve morbiditesinin yüksek olması nedeniyle yakın takip edilmeli ve yoğun tedavi verilmelidir (3). Kliniği ağır seyreden hastalarda lökositoklastik vaskulitlerden hipersensitivite anjiti, mixed kriyoglobulinemi, antinötrofil sitoplazmik antikor ilişkili vaskulitler (ANCA), sistemik lupus eritematozus gibi kollajen doku hastalıklarının ayırıcı tanısı yapılmalıdır (4).…”

Section: Pulmoner Tutulumu Olan Bir Henoch-schönlein Olgusuunclassified

“…Fizik muayenesinde vücut ısısı:39°C, kan basıncı:70/40 mmHg, solunum sayısı:40/ dakika, nabız sayısı:120/dakika, oksijen saturasyonu:%95 olan, orofarinks, gövde, üst-alt ekstremitelerde yaygın peteşi, purpura, ekimozlar ve ense sertliği saptanan hasta meningokok septisemisi ön tanısıyla çocuk yoğun bakım servisine yatırıldı. Laboratuvar tetkiklerinde hemoglobin 13.6 mg/dL, beyaz küre sayısı 24.400/mm 3 , trombosit sayısı 360000/mm 3…”

Section: Olgu Sunumuunclassified

Pulmoner tutulumu Olan Bir Henoch-Schonlein Olgusu

Kurt¹,

Başaran

Aydin

et al. 2019

Turkish Journal of Pediatric Disease

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Henoch-Schönlein purpurası (HSP), artrit / artralji, gastrointestinal ve genitoüriner sistem tutulumunun eşlik ettiği nontrombositopenik purpura ile karakterize, çocukluk çağının en sık görülen vaskülitidir. Gerçekte solunum sistemi tutulumu nadir olmakla birlikte, morbidite ve mortalitesinin yüksek olması nedeniyle önemlidir. Bu yazıda atipik cilt tutulumu, gastrointestinal ve solunum sistemi bulguları olan HSP vakası sunulmuş ve tedavisi tartışılmıştır. Tedavide yüksek doz metilprednisolon uygulandıktan sonra klinik düzelme sağlanmıştır. Solunum sistemi tutulumu olan HSP vakalarında, mortalite ve morbiditesinin yüksek olması nedeniyle tedavinin hızlı ve yoğun olması gerektiği vurgulanmıştır.

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Diffuse Alveolar Hemorrhage in IgA Vasculitis with an Atypical Presentation

Cited by 8 publications

References 20 publications

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

A Case Report of Diffuse Alveolar Hemorrhage Coexisting With Immunoglobulin A (IgA) Nephropathy

Pulmoner tutulumu Olan Bir Henoch-Schonlein Olgusu

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