Diffuse alveolar hemorrhage (DAH) is a pulmonary condition that can be caused by autoimmune disorders such as lupus, small vessel vasculitis, and antiphospholipid syndrome. Sarcoidosis as a cause of ...DAH has been reported; however, the literature remains limited. We performed a chart review for patients with a diagnosis of both sarcoidosis and DAH. Seven patients met inclusion criteria. Mean (range) patient age was 54 years (39–72), and 3 patients had a history of tobacco use. Diagnosis of DAH and sarcoidosis were concurrent for 3 patients. Corticosteroids were used for treatment of DAH in all patients; 2 (including 1 with refractory DAH) were successfully treated with rituximab. We believe sarcoidosis-associated DAH is more common than previously reported. It is essential to consider sarcoidosis in the differential diagnosis of immune-mediated DAH.
Key Points
• Sarcoidosis can cause diffuse alveolar hemorrhage (DAH); more extensive studies are needed to estimate this condition’s prevalence.
• BMI of 25 or higher appears to be a risk factor for the development of sarcoidosis-associated DAH.
A 53-year-old male with rheumatoid arthritis presented with recurrent headaches, seizures and right-sided lower extremity paralysis while on antiepileptic medications. Work up revealed pachymeningeal ...and leptomeningeal enhancement on brain MRI. Differential diagnosis included a variety of infections, neoplasm and vasculitis. Histopathology showed findings consistent with rheumatoid meningitis (RM). Ultimately based on symptoms, MRI findings and tissue pathology, he was diagnosed with RM. Intravenous pulse dose steroids were initiated followed by rituximab every 6 months, resulting in significant improvement of the brain MRI findings. Patient has remained seizure free.
Antiphospholipid syndrome (APS) is a systemic autoimmune disease clinically associated with thrombotic and obstetric events. Additional manifestations have been associated with APS, like diffuse ...alveolar hemorrhage (DAH). We aimed to summarize all the evidence available to describe the presenting clinical features, their prognostic factors, and short- and long-term outcomes.
We performed a mixed-method approach combining a multicenter cohort with a systematic literature review (SLR) of patients with incident APS-associated DAH. We described their clinical features, treatments, prognostic factors, and outcomes (relapse, mortality, and requirement of mechanical ventilation MV). Kaplan-Meier methods were used to estimate relapse and mortality rates, and Cox and logistic regression models were used to assess the factors associated as appropriate.
We included 219 patients with incident APS-associated DAH (61 from Mayo Clinic and 158 from SLR). The median age was 39.5 years, 51% were female, 29% had systemic lupus erythematosus, and 34% presented with catastrophic APS (CAPS). 74% of patients had a history of thrombotic events, and 26% of women had a history of pregnancy morbidity; half of the patients had a history of thrombocytopenia, and a third had valvulopathy. Before DAH, 55% of the patients were anticoagulated. At DAH onset, 65% of patients presented hemoptysis. The relapse rate was 47% at six months and 52% at one year. Triple positivity (HR 4.22, 95% CI 1.14–15.59) was associated with relapse at six months. The estimated mortality at one and five years was 30.3% and 45.8%. Factors associated with mortality were severe thrombocytopenia (< 50 K/μL) (HR 3.10, 95% CI 1.39–6.92), valve vegetations (HR 3.22, 95% CI 1.14–9.07), CAPS (HR 3.80, 95% CI 1.84–7.87), and requirement of MV (HR 2.22, 95% CI 1.03–4.80). Forty-two percent of patients required MV on the incident DAH episode. Patients presenting with severe thrombocytopenia (OR 6.42, 95% CI 1.77–23.30) or CAPS (OR 4.30, 95% CI 1.65–11.16) were more likely to require MV.
APS-associated DAH is associated with high morbidity and mortality, particularly when presenting with triple positivity, thrombocytopenia, valvular involvement, and CAPS.
•Antiphospholipid syndrome (APS) can manifest as diffuse alveolar hemorrhage (DAH).•Forty-seven percent of the patients with APS-associated DAH relapsed within six months.•Mortality rates were 30.3% and 45.8% at one and five years, respectively.•Triple positivity, thrombocytopenia, valvular involvement, and Catastrophic APS were associated with worse outcomes.
IS IT LIBMAN-SACKS ENDOCARDITIS? Azeem, Amir; Alexander-Nickens, Myrna E; Majithia, Vikas ...
Journal of the American College of Cardiology,
04/2016, Letnik:
67, Številka:
13
Journal Article
Recenzirano
Odprti dostop
A transesophageal echocardiogram (TEE) revealed a 0.5 x 0.9 cm heterogeneous echodensity on the posterior mitral leaflet with mild mitral regurgitation.
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic’s syndrome, are a group of inflammatory disorders of the central nervous system (CNS) ...characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone −1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders.
Reply Smith, Benjamin J.; Bolster, Marcy B.; Slusher, Barbara ...
Arthritis care & research (2010),
October 2019, Letnik:
71, Številka:
10
Journal Article
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