Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature

Naniwa, Taio; Banno, Shogo; Sugiura, Yoshiki; Yokota, Kaori; Oosawa, Tomoyo; Maeda, Shinji; Hayami, Yoshihito; Takahashi, Nobuyuki; Ueda, Ryuzo; Matsumoto, Yoshihisa

doi:10.1007/s10165-006-0540-0

Cited by 14 publications

(13 citation statements)

References 21 publications

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“…72 DAH has been reported in systemic sclerosis as a part of pulmonary-renal syndrome. 73,74 It has also been linked to the coexistence of p-ANCA and antimyeloperoxidase ANCA in patients with confirmed systemic sclerosis. [75][76][77] DAH was also observed in a 57-year-old man with fulminant systemic sclerosis/dermatomyositis overlap.…”

Section: Systemic Lupus Erythematosusmentioning

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: Systemic Lupus Erythematosusmentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Nasser

Cottin

2018

Semin Respir Crit Care Med

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“…It is thought that endothelial cell injury is involved in these conditions. There have been reports of pulmonary alveolar hemorrhage with hemolytic uremic syndrome [9,10], thrombotic thrombocytopenic purpura [11], microangiopathy [12], and systemic sclerosis [13]. Therefore, a pulmonary alveolar hemorrhage with malignant-phase hypertension may be caused by a mechanism that is similar to that underlying these diseases.…”

Section: Discussionmentioning

confidence: 95%

A rare case of malignant-phase hypertension with pulmonary alveolar hemorrhage

et al. 2011

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Malignant-phase hypertension is characterized clinically by severe accelerating hypertension with neuroretinopathy or papilledema and by evidence of renal damage. A Japanese male in his early thirties presented with hemoptysis and general fatigue. He had a 5-year history of hypertension, but had not received any treatment. His blood pressure was 290/150 mmHg and his serum creatinine level was 8.24 mg/dL. Chest X-rays and computed tomography scans of the chest revealed a pulmonary alveolar hemorrhage. He was suspected of having vasculitis syndrome or Goodpasture's syndrome, but his renal biopsy specimen showed malignant nephrosclerosis. Myeloperoxidase antineutrophil cytoplasmic antibody (ANCA), proteinase-3 ANCA and antiglomerular basement membrane antibody were negative. He was treated with a calcium antagonist and a β-blocker, followed by an angiotensin-converting enzyme inhibitor. After the administration of the β-blocker, his blood pressure decreased and his renal function gradually improved. This is a rare case of malignant-phase hypertension with pulmonary alveolar hemorrhage; this condition should be considered in the differential diagnosis in order to avoid unnecessary treatment such as immunosuppressive therapy.

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“…We reviewed the reported cases of normotensive SRC or SSc complicated by TMA in Japan, including those from Japanese language journals (13)(14)(15)(16)(17) (Table). Hypertensive SRC, ANCA-positive SRC and SSc complicated by TTP were excluded from our review.…”

Section: Discussionmentioning

confidence: 99%

Glucocorticoid-induced Normotensive Scleroderma Renal Crisis: A Report on Two Cases and a Review of the Literature in Japan

et al. 2013

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We herein report the findings of 2 cases of normotensive scleroderma renal crisis (SRC) that developed soon after the commencement of a glucocorticoid therapy. We also review 8 cases of normotensive SRC reported in Japan, including our cases. The common characteristics of these 8 cases are as follows: the recent onset of systemic sclerosis, the presence of diffuse skin sclerosis, the presence of myositis and/or serositis, a high titer of antinuclear antibody and positivity for anti-Scl-70 antibody. In 7 of the 8 patients, thrombotic microangiopathy developed within one month of starting the glucocorticoid treatment. We should be careful with the use of glucocorticoids in systemic sclerosis patients exhibiting these features in order to avoid cases of normotensive SRC.

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Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature

Cited by 14 publications

References 21 publications

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

A rare case of malignant-phase hypertension with pulmonary alveolar hemorrhage

Glucocorticoid-induced Normotensive Scleroderma Renal Crisis: A Report on Two Cases and a Review of the Literature in Japan

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