**3.1 Arthropathies**

Approximately 50% of lupus patients suffer from arthritis [29]. Joint disease, quite often a small joint polyarthritis, typically symmetric, is noted with typical involvement of PIPs, MCPs and wrists, inflammatory in nature; however, this is not erosive, which differentiates it from rheumatoid arthritis [30]. However, the practitioner should keep in mind that the differential diagnosis of IgG4-related disease, lymphomas, Sjogren's, sarcoidosis, or spondyloarthropathies, can also present with a phenotypic appearance of lupus arthritis. The definitive finding of arthritis only seen in lupus would be lupus arthropathy or acute rheumatic fever, which is followed by Jaccoud's arthropathy. Jaccoud's arthropathy is a chronic, non-erosive, reversible (with proper splinting) joint disorder that may occur after repeated bouts of arthritis. This arthropathy is caused by inflammation of the joint capsule and subsequent

fibrotic retraction, causing ulnar deviation of the fingers, through metacarpophalangeal joint subluxation, primarily of the fourth and fifth fingers [31].

The greatest emphasis should be placed on the fact that all joints could be involved in lupus. Arthritis of lupus may be the presenting feature, and therefore, all cases of inflammatory arthritis must be evaluated with x-rays and a thorough history and physical, to exclude other diseases. Treatment would begin with the use of hydroxychloroquine and the addition of methotrexate. If necessary, abatacept (a CTLA4 inhibitor drug), could be added, as well as the newer medication discussed earlier, belimumab. Additionally, low dose steroids are often effective. While some practitioners may view steroids as poison, others feel the patient's quality of life, on Prednisone (5 mg or less), even permanently could be appropriate, if this is necessary for disease control and improvement in the patient's quality of life. The patient should be informed of necessity for vigilance with regard to sleep, lipid and blood pressure monitoring, and the risk of osteoporosis. In the final analysis, the ratio of logic needs to be brought into consideration. As a practicing rheumatologist, with a personal experience of 32 years, experience dictates that 5 mg of Prednisone or less in virtually all the inflammatory patients that cannot be weaned, failed to cause significant steroid side effects. In the minority of patients who do suffer steroid side effects from a 5 mg daily equivalent or less as they begin to age, skin fragility or perhaps early cataracts can be seen; however, this may be difficult to ascertain, unless their ophthalmologist is convinced that any posterior subscapular cataract is the definite consequence of steroid use. Otherwise, this would be difficult to ascertain [32].

#### **3.2 Thrombocytopenia/thrombocytosis**

Approximately 42–46% of patients develop a cytopenia, including leukopenia and anemia [29]. Cytopenias in lupus are typically recognized with anemia, often hemolytic or of chronic disease, thrombocytopenia, or thrombocytosis [33]. Thrombocytosis indicates inflammation, while thrombocytopenia is often autoimmune and antiplatelet antibodies lower platelet counts; however, this should not be taken for granted. As in Sjogren's, the mechanism would be hypersplenism; however, the finding of thrombocytopenia must prompt a probe for lupus. This protocol also stands in the case of a low white blood cell count. A WBC less than 4000 units for all, or lymphocyte of less than 1000, should both prompt an evaluation and workup for lupus. These findings while not specific are quite typical. Please note that one isolated sample needs repeating.

#### **3.3 Lupus nephritis**

Approximately 40% of lupus patients are diagnosed with nephritis [29]. The patient presents with blood or protein in the urine [34]. A renal biopsy is performed. A diagnosis is established - Mesangial proliferative, diffuse or focal proliferation, or pure membranous. The treatments for this vary. The current main stay treatment is mycophenolate mofetil. A new medication, which will be available in the near future, is calcineurin inhibitor, Voclasporin [35]. The data regarding this is promising. Rituximab, anecdotally, and in Pureview Data, indicates that it may also be helpful, although it is not the standard of care. Emphasis should be placed on the actuality that "the standard of care" should supersede the Food and Drug Administration's indications for any drug. Approval for a drug by the Food and Drug Administration is solely based on the drug company's actual "indication application" for that particular drug. While it may be used exclusively for its indication, in some cases it should be noted that the drug may prove more effective for off label use. This unfortunately

**9**

*Don't Miss Lupus*

*DOI: http://dx.doi.org/10.5772/intechopen.96892*

timely establishment of proper treatment.

be determined.

**3.5 Abdominal pain**

**3.4 Central nervous system**

seems to be a matter of "dollars and cents" where the pharmaceutical companies are

Roughly 32% of lupus patients develop lupus that attacks the central nervous system [29]. Lupus involving the central nervous system is both a confusing and interesting aspect of the disease [36, 37]. Virtually any central nervous system or peripheral nervous system problem including, but not limited to, neuropathy, mononeuritis multiplex, seizures, blindness, loss of hearing, cranial nerve palsy, encephalopathy, psychosis and movement disorders, are not uncommon in the lupus population, and may frequently present as an initial feature of the disease. To reemphasize, all symptomatology that has been mentioned in this chapter may be an initial feature of lupus; however, the lack of swift rheumatology involvement often ultimately leads to a delay in diagnosis, which is always detrimental to the patient. Therefore, it is important to perform a comprehensive evaluation, including biopsy, angiogram, or other internal organ imaging, as well as complete serologic testing. Additionally, most patients are not willing to take medication for extended periods of time, unless it can be proven to them by their physician that the medication will indeed benefit them by alleviating the symptoms they are experiencing. This will assist in a more accurate diagnosis of lupus versus another disease process. As in every case involving a possible autoimmune process, emphasis should be placed on the importance of swift initiation of workup, as this will facilitate the

If a patient is acutely ill with psychosis, they will typically be treated in a hospital setting, being initially seen by neurology and psychiatry, as other specialists. Unfortunately, this occurs before a rheumatologist is consulted [38]. An immediate MRI of the brain and lumbar puncture should be ordered, along with autoantibodies and cerebrospinal fluid, to assess the ribosomal P antibody, GAD65 antibody and NMO**.** With these proper evaluations, the likelihood of a CNS lupus diagnosis may

It is quite typical in that lupus patients, including those with renal and central nervous system involvement, in general, do quite well with medical compliance. Published death rates, transplant rates, and dialysis rates for lupus nephritis are decidedly dependent upon the population type that is investigated. A well-educated compliant group of patients has a very low incidence of end stage renal disease while the noncompliant group almost certainly ultimately develop end stage renal disease [39].

Another presentation would be abdominal pain, rather than splenomegaly. This would account for approximately 27% of lupus symptomatology [29]. A patient with severe abdominal pain, who is known to have lupus, after a proper workup for exclusion of perforated viscus or ischemic disease, the treatment would be steroids for what is mesenteric arteritis or serositis. The prognosis would not change, as they are both treated with moderate high dose steroids, oral or IV. Again, this can be a presenting feature of lupus. To the detriment of the patient, they are often are seen by gastroenterologists, who run a plethora of tests, including CTAs and MRIs of various organs, only to ultimately discover a case of hepatosplenomegaly with pain. At that point, to the misfortune of the patient, unnecessary surgery is generally performed for the hepatosplenomegaly, and sadly, the patient passes away as a result. If the patient had been treated properly, their life could have been saved, as they would have been successfully treated with 1 to 2 mg/kg of prednisolone or similar [40].

concerned when determining the indication, they seek from the FDA.

seems to be a matter of "dollars and cents" where the pharmaceutical companies are concerned when determining the indication, they seek from the FDA.
