Hydrocephalus and Diffuse Alveolar Hemorrhage as the Initial Manifestation of Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report.

Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect many systems, such as the central nervous system (CNS), cardiovascular, musculocutaneous, renal, and respiratory systems, etc. SLE can occur alone or in association with other autoimmune diseases, especially antiphospholipid syndrome (APS). Hydrocephalus and diffuse alveolar hemorrhage (DAH) are infrequent manifestations of SLE and APS and increase the risk of mortality and morbidity in these patients. A 48-year-old man was hospitalized due to fever, hemoptysis, and headache, which had not been examined for diagnosis and treatment for a long period of time because of his negligence. After imaging and autoimmune tests were performed, hydrocephalus and DAH were diagnosed as initial manifestations of SLE in association with APS according to the SLE/APS criteria. Additionally, lupus nephritis was confirmed via renal biopsy. The hydrocephalus was due to sigmoid sinus thrombosis in the context of a hypercoagulable state of SLE and APS. The initiation of steroid and immunosuppressive treatments has yielded positive results, negating the need for any invasive procedures. The coexistence of SLE and hydrocephalus, although uncommon, has been documented in the literature. Hydrocephalus, a rare complication of SLE, necessitates timely decision-making regarding treatment based on the patient's clinical symptoms and the degree of ventricular dilation, which may involve either conservative medical management or surgical procedures such as a VP shunt. In patients with multiorgan involvement, rheumatological and autoimmune diseases are among our most important differential diagnoses. Importantly, neurological symptoms, particularly headaches, should not be underestimated, even though they are commonly observed in patients with SLE or APS. A detailed medical history, a comprehensive examination, and strong clinical suspicion are essential when patients present with neurological symptoms, and physicians must consider autoimmune diseases in their differential diagnoses.