Department of Pulmonary, Critical Care and Sleep Medicine, VMMC and Safdarjung Hospital, New Delhi, India.
Perm J. 2020 Nov;24:1-3. doi: 10.7812/TPP/20.004.
The systemic manifestations of immunoglobulin A (IgA) nephropathy with lung involvement include diffuse alveolar hemorrhage due to monoclonal IgA disorders, IgA-variant Good pasture's syndrome, and Henoch-Schoenlein purpura. However, pneumonitis due to IgA immune complex has rarely been reported as the pulmonary manifestations of IgA nephropathy.
A 35-year-old woman presented with 2 years of progressive shortness of breath, dry cough, low-grade fever along with progressive loss of appetite, and loss of weight. She underwent renal, duodenal, and lung biopsies. She was diagnosed with a rare combination of IgA-mediated nephropathy, IgA-associated celiac disease, and IgA-mediated immune complex cavitary lung disease.
Secretory IgA may be acting as an immune complex or proinflammatory agent to provoke the signs and symptoms in this case. Thus, the respiratory process may incite renal disease or vice-versa. Further research is needed to analyze the possibility of such associations.
免疫球蛋白 A(IgA)肾病伴肺受累的全身表现包括单克隆 IgA 疾病、IgA 变体 Good pasture 综合征和过敏性紫癜引起的弥漫性肺泡出血。然而,IgA 免疫复合物引起的肺炎作为 IgA 肾病的肺部表现很少有报道。
一名 35 岁女性因进行性呼吸困难、干咳、低度发热以及逐渐食欲不振和体重减轻而就诊。她接受了肾脏、十二指肠和肺部活检。她被诊断为 IgA 介导的肾病、IgA 相关乳糜泻和 IgA 介导的免疫复合物空洞性肺病的罕见组合。
分泌型 IgA 可能作为免疫复合物或促炎剂在这种情况下引起症状和体征。因此,呼吸过程可能会引发肾脏疾病,反之亦然。需要进一步研究来分析这种关联的可能性。