IgG4-related disease with multiorgan involvement: a case-based review.

IgG4-related disease (IgG4-RD) is an immune-mediated multi-organ inflammatory disorder caused by tissue infiltration of lymphocytes with IgG4-secreting plasma cells. Herein, we discuss a case of a patient with IgG4-RD who had involvement of multiple organs: the kidneys, lymph nodes, bone marrow (biopsy performed), lungs, liver, and small intestine (imaging abnormalities). Although several case reports and series of IgG4-RD involving different organ involvement are in the literature, our patient has extensive simultaneous multi-organ involvement. We utilized the four domains (serologic, pathologic, radiologic, and pathologic) as discussed in the new 2019 ACR/EULAR classification criteria to provide a useful framework in considering an alternative tool for IgG4-RD in multi-organ involvement, where biopsy is more invasive and not always accessible. We highlight the findings of each organ involved that increase the likelihood that the patient has IgG4-RD. In our patient, the IgG4-RD classification criteria was fulfilled with total points adding up to 48. Our case meets the classification criteria for IgG4-RD, since at least one organ is involved to meet entry criteria (biopsy proven), no exclusion criteria are present, and the total points are ≥ 20. When such extensive involvement of IgG4-RD occurs, early diagnosis and treatment are recommended to avoid irreversible organ damage and better outcomes.

Trauma-induced concomitant psoriatic arthritis and complex regional pain syndrome.

To report the simultaneous occurrence of psoriatic arthritis (PsA) and chronic regional pain syndrome type I (CRPS I) both triggered by intense walking in a male golfer with a history of scalp psoriasis. Sequential existence of these two conditions have been reported in the literature; however, to our knowledge, this is the first report of a simultaneous occurrence of PsA and CRPS I. This case illustrates the complex interplay between genetic predisposition and environmental risk factors with the central nervous and immune systems. As the pathogenesis of PsA has been better understood in recent years, we propose a mechanism that explains how the release of pro-inflammatory cytokines and neuropeptides following a traumatic event elicits a vicious cycle that is a common ground for the development of both PsA and CRPS I. Even unperceived trauma, such as intense walking, when directed to the synovio-entheseal complex, can precipitate the development of PsA and CRPS I in predisposed individuals.

Limb-Girdle Muscular Dystrophy 2B and Miyoshi Presentations of Dysferlinopathy.

We report the following 2 subtypes of progressive limb-girdle dystrophy type 2B: limb-girdle muscular dystrophy type 2B (LGMD2B) and Miyoshi. The first patient described had weakness in the anterior thigh muscles (LGMD2B) and the second patient had calf muscle weakness and atrophy (Miyoshi). Literature review was performed and LGMD2B was compared and distinguished from other myopathies of similar nature. Genetic testing with polymerase chain reaction analysis of the DYSF gene confirmed the diagnosis in both patients. Additional findings of histopathology, specific stain for sarcolemmal membrane protein, Western blot analysis and clinical presentation clinched the diagnosis further of dysferlinopathy (LGMD2B) in both our patients. Currently, there is no definitive treatment on the horizon and immunosuppressive therapy is not recommended for this condition. Gene therapy may have a future role, but at present, muscle-strengthening exercises and patient awareness are the mainstays.

Vasculopathy, hematological, and immune abnormalities associated with levamisole-contaminated cocaine use.

OBJECTIVES: To report 4 cases of cocaine-related purpura and to review previously reported cases of levamisole, levamisole-contaminated cocaine, and cocaine-induced vasculopathy. METHODS: We describe 4 patients suspected of vasculopathy associated with levamisole-tainted cocaine use. A retrospective review of the literature was performed using the PubMed, PubJet, MD consult, and Cochrane review databases. RESULTS: Four cases (2 females and 2 males), 46 to 55 years of age, presented with cocaine-related purpura, mainly affecting the ears, neutropenia, and autoantibodies. Skin biopsies revealed a mixed pattern of leukocytoclastic vasculitis and microvascular thrombosis in 2 cases, and pure thrombosis in the third case. The mixed vasculopathic pattern in association with neutropenia, both known adverse effects of levamisole, and levamisole positivity in 2 cases point to this compound as the true etiologic agent in our patients. Eleven cases of levamisole-contaminated cocaine-induced vasculopathy have been described in the English literature. Among these, 10 were females. Age range was 22 to 57 years. Urine levamisole positivity was tested and confirmed in 3 of the 11 cases. The clinical characteristics, laboratory features, histology, treatment, and recovery rates were compared for the published cases of levamisole, levamisole-contaminated cocaine, and cocaine-induced vasculopathy. CONCLUSIONS: Adulterated cocaine abuse is an increasingly recognized phenomenon in North America. Levamisole is among the many contaminants that have been detected in seized cocaine throughout North America and Europe. Recent reports described an association between levamisole-tainted cocaine and purpuric skin rash, neutropenia, and the presence of autoantibodies.

HIV infection and clinical spectrum of associated vasculitides.

Various infections have been causative in the pathogenesis of systemic vasculitides, and HIV infection is not spared. In an immunocompromised host, cytomegalovirus, Epstein-Barr virus, varicella zoster virus, herpes simplex virus, hepatitis B and hepatitis C virus, and mycobacteria, along with HIV infection can cause vasculitis. Herein we emphasize the spectrum of vasculitides, their pathogenesis, presentation, course, and therapy in the HIV-infected population. Every spectrum and size of the blood vessel involvement have been seen in HIV-associated vasculitides. We review each spectrum in detail and describe our experience with polyarteritis nodosa, the most common presentation occurring in HIV-infected patients. We also discuss the differences in HIV, hepatitis B, and hepatitis C- related polyarteritis nodosa in detail.

Familial Mediterranean fever presenting as anti-cyclic citrullinated peptide antibody negative palindromic rheumatism.

A 64-year-old Egyptian man who resides in the United States presented to the rheumatology clinic with 6 months history of episodic recurrent pauci--arthritis along with constitutional symptoms. His Mediterranean ancestry, anti-cyclic citrullinated peptide negativity, and cyclical palindromic rheumatism prompted an investigation for familial Mediterranean fever gene mutation. He was found to have heterozygous 694I gene mutation during MEFV analysis. He also met Liveneh 1 major and 1 minor criteria for the diagnosis of familial Mediterranean fever.

HIV infection and rheumatic diseases: the changing spectrum of clinical enigma.

In this article, the authors discuss the occurrence and prevalence of rheumatic syndromes before and after highly active antiretroviral therapy became the usual mode of treatment. The immunologic, environmental, and genetic factors behind the combination of HIV infection and rheumatic manifestation contribute to the complexity of these diseases. Miscellaneous case reports are discussed in relation to HIV infection. The authors conclude that geriatric care of HIV patients is on the horizon as more people have access to newer, more effective therapy and mortality is on the decline. Younger HIV patients will be committed to a lifetime of therapy to address bone disease and other chronic problems. In the future, newer agents may steer the clinical scenario in unforeseen directions.