Case Presentation:

A 23–year–old gentleman presented with a 2–week history of cough, epistaxis, hemoptysis and shortness of breath on exertion, loss of appetite, fatigue, nausea and dysgeusia. He denied rash or arthralgias and reported normal urinary output. He was a migrant worker from Mexico without formal access to healthcare, and denied use of illicit drugs. Physical examination revealed mild respiratory distress with tachypnea, tachycardia, mild hypoxemia and hypertension. He had marked pallor, with a hyperdynamic precordium and coarse rales bilaterally. There was no jugular venous distension, pericardial friction rub, stupor, asterixis or peripheral edema. At presentation, his serum creatinine and BUN levels were markedly elevated (41.5 and 218 mg/dl) with hemoglobin of 4.6 g/dl. His urinalysis showed no casts, a 4+ proteinuria, mild hematuria and a urine protein/creatinine ratio of 11.4. Chest X–ray showed symmetric bilateral air space opacities involving the lower lung, and a non–contrast chest CT showed diffuse and somewhat symmetric airspace opacities consistent with a hemorrhagic picture. Ultrasound showed atrophic bilateral kidneys with medical renal disease, and an echocardiogram that was normal. All cultures were negative, and a renal biopsy was consistent with end stage renal disease and a primarily immunologically mediated glomerulonephritis, producing a mesangioproliferative and focally crescentic injury.

Discussion:

The patient received packed red blood cell transfusions and was promptly dialyzed. The Initial working diagnosis was pulmonary renal syndrome (Lupus, Goodpasture’s syndrome, ANCA associated vasculitis, cryoglobulinemia). However, a comprehensive autoimmune work up was inconclusive. The patient’s pulmonary symptoms and alveolar infiltrates completely resolved with repeated dialysis, consistent with the clinical picture of uremic pneumonitis (UP). Described in 1955 by Hopps and Wissler, UP is characterized by uremia–induced increased permeability of pulmonary alveolo–capillary interfaces, leading to interstitial and intra–alveolar edema, alveolar hemorrhage and pulmonary hyaline membrane formation. These changes are further compounded by bleeding diatheses secondary to platelet dysfunction in advanced renal disease. Pulmonary symptoms and radiographic findings in UP are unique in that they are completely reversible with hemodialysis.

Conclusions:

While common in the pre–dialysis era, UP is now rare where there is improved access to healthcare, recognition and care of kidney disease and access to dialysis. This patient had no access to formal healthcare and presented with a now rare and “forgotten” clinical entity. Clinicians need to be alert to this and other severe complications from uremia as they are likely to be encountered more frequently with an increase in the number of uninsured patients, and reduced access to care and dialysis.

Figure 1Bilateral hemorrhagic infiltrates