By Howard R. Smith, MD, and Mehrnaz Hojjati, MD
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A 48-year-old man presented to his primary care physician reporting the following symptoms over the prior month: fatigue, low-grade fevers, 15-pound weight loss, new-onset shortness of breath, numbness/tingling over the left foot, and polyarthralgia involving the metacarpophalangeal and proximal interphalangeal joints, wrists, shoulders and knees. His history was notable for hypertension and tobacco chewing for 20 years, and his family history was notable for lupus (systemic lupus erythematosus; SLE) in an aunt. Preliminary lab evaluation by his primary care physician showed an elevated ESR (87 mm/hr) and positive anti-nuclear antibody (ANA) findings (1:640 titer) (Figure 1).
Figure 1. Anti-nuclear antibody indirect immunofluorescence staining showing a nucleolar pattern.
He was referred to Cleveland Clinic’s Center for Vasculitis Care and Research, where further testing revealed elevated anti-dsDNA antibodies, leukopenia and thrombocytopenia, a low C3 level, proteinuria and anti-cardiolipin IgM antibodies. Serology for antineutrophil cytoplasmic antibodies, cryoglobulins, hepatitis and HIV was negative, as was serum protein electrophoresis. Chest CT revealed pulmonary emboli (Figure 2), and EMG showed moderate sensory axonal neuropathy with a mononeuritis multiplex pattern.
Figure 2. Chest CT showing saddle pulmonary emboli.
Anticoagulation was initiated, along with methylprednisolone, and the patient had same-day appointments at Cleveland Clinic to see a rheumatologist with lupus expertise at the Lupus Clinic, a nephrologist for a renal biopsy, and a neurologist and an infectious disease specialist to rule out lupus mimics. Lupus nephritis and lupus vasculitis were confirmed on further evaluation and renal biopsy. A coordinated course of therapy that included pulse corticosteroids and additional immunosuppressive therapy with monthly IV cyclophosphamide was initiated.
The above vignette represents the complexity of symptoms with which some SLE patients present, underscoring the importance of a multidisciplinary approach to address all medical aspects of these patients’ cases and fully optimize their care. This case exemplifies the type of patient evaluated and managed at Cleveland Clinic’s new multidisciplinary Lupus Clinic, which was established by the Department of Rheumatic and Immunologic Diseases to integrate the management of patients with SLE and provide comprehensive, leading-edge care.
For patients with complex SLE cases, the Lupus Clinic provides access to the department’s team of rheumatologists specializing in SLE along with streamlined access to subspecialists in other disciplines, including nephrologists, dermatologists, neurologists and preventive cardiologists. The clinic offers both consultative services and long-term management, including treatments directed at all of SLE’s diverse facets, such as arthritis, dermatitis, nephritis and cerebritis.
Same-day access to other specialist providers with expertise in SLE involving other organs, such as the kidneys and the skin, is a cornerstone of the Lupus Clinic. It serves to ensure both maximum convenience for patients and care that addresses all aspects of their condition, whether it be SLE or related connective tissue diseases such as overlap syndromes.
The Lupus Clinic provides care that goes beyond the diagnosis, focusing on routine follow-up appointments that are tailored to patients’ needs and disease complexity. For instance, patients who are at higher risk for frequent disease flares or end-organ involvement (e.g., renal complications, which are often clinically asymptomatic) are monitored via simple urine and blood tests at routine follow-up visits. Lupus patients evaluated in the clinic also are offered screening for modifiable cardiovascular risk factors, through Cleveland Clinic’s state-of-the-art Preventive Cardiology Program, as well as lifestyle modification counseling and therapeutic guidance based on their risk stratification.
Additionally, the Lupus Clinic offers interested patients the opportunity to enroll in Cleveland Clinic’s lupus registry, which is directed by Mehrnaz Hojjati, MD, in the Department of Rheumatic and Immunologic Diseases. The clinic gives patients easy access to investigational clinical trials in SLE and other challenging autoimmune diseases, facilitated by on-site clinical research coordinators. As the Lupus Clinic’s cumulative patient base grows, we look forward to sharing research insights that emerge from these trials.