**3.5 Abdominal pain**

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].

#### **3.6 Pancreatitis and Raynaud's phenomenon**

Pancreatitis is an excellent example of a disease, which is not part of the listed diagnostic criteria for lupus. Raynaud's phenomenon also not listed in the diagnostic criteria, although approximately 25% of lupus patients suffer from this condition [29, 41]. While either of those may be the presenting feature of systemic lupus, neither are listed as diagnostic criteria which is fine; however, the practitioner should perform a thorough workup to determine if a patient who has pancreatitis, as they may well have lupus. It should be noted however, that alcoholism, gallstone disease and pancreatic divisum, without the atypical sausage pancreas of IgG4-related disease, must be ruled out.

With regard to Raynaud's, the reversible spasm of vessels, usually induced by cold or emotional provocation, typically with triple phase color response from 5 to 60 minutes, is a frequent feature in lupus patients and may well be the initial finding of the disease. The practitioner must look past scleroderma, which has a more ominous prognosis than Raynaud's related to lupus. This is often differentiated with a simple in-office nailfold capillaroscopy, which by in large, is a tremendously underutilized tool [42]. For the well-seasoned rheumatologist, this technique is used more often, but it should be used with regularity. In fact, nailfold capillaroscopy should be used as a baseline in all potential cases of autoimmune patients.

#### **3.7 Heart and lungs**

Attention to the heart and lungs is essential [43]. A patient with recurrent pneumonias is more likely to have lupus pneumonitis or an autoinflammatory disease, rather than the occurrence of infectious pneumonia every three months. After the onset of a second case of pneumonia, a rheumatologist should be consulted, but commonly, this does not occur. Regrettably, the patient who is suffering from an autoimmune disease has now suffered without a proper diagnosis for an unspecified amount of time. At this point, it would be advantageous to the patient to be seen by a rheumatologist without further delay.

Other common heart and lung manifestations of lupus include pleurisy and/ or pericardial effusion [44]. Approximately 12% of lupus patients will develop a pericardial effusion [29]. Alarmingly, in several medical institutions, the treatment of choice for pericardial effusion is a pericardial window. Unfortunately, as in the case of inappropriate splenectomy with abdominal pain in a case of lupus, as mentioned earlier, a pericardial window is carries equal efficacy in a lupus patient presenting with pericardial effusion. As there is no indication for abdominal surgery for a patient with lupus abdominal pain, there is also virtually no indication for pericardial window in a lupus pericarditis patient. The incidence of tamponade is extraordinarily low. Myxomatosis valvular heart disease or so-called Libman-Sacks endocarditis, with or without phospholipid antibodies, is another finding that should be noted, although this is often woefully overlooked.

#### **3.8 Overlooked autoimmunity**

Many lupus patients suffer from autoimmunity that is frequently overlooked and therefore; the percentage of sufferers remains uncalculated [45]. The most common is likely Hashimoto's thyroid disease; however, other conditions include Graves' disease, myasthenia gravis, Addison's disease, primary biliary cirrhosis, and autoimmune hepatitis. Each of these has autoimmune associations that should not be overlooked. Many of the features potentially seen in Sjogren's syndrome, or many lupus-like features such as interstitial lung disease, should never be taken for

**11**

lymphocytes 4mm2

mune diseases with low values [51].

*Don't Miss Lupus*

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

ramifications to expect in the future.

**4. The thought process of a rheumatologist**

granted based on the positive ANA or research criteria, as those patients may well have myositis or scleroderma. As mentioned in Part 2 of this chapter, "A Rheum

There are deep gaps between the thought process and treatment plans of a rheumatologist versus that of a general internist, family practitioner, ophthalmologist,

Rheumatology remains greatly underutilized. This regrettably adds substantial delay to the diagnosis and treatment of a patient. It bears mentioning again that all organ systems may be involved in lupus. Based on this, the all-purpose criteria is preferable to the new SLICC criteria for diagnosis of lupus, as it was far more practical [46]. It also bears mentioning again that no practitioner may diagnose lupus, or any other disease process, based solely on research criteria. Criteria are to be used merely as a guideline. For example, a patient presents to their physician, stating they are "not feeling well". Subsequently, blood studies are ordered that reveal an ANA with a very high titer and upon further perusal, a very high DNA is also discovered, yet the physician fails to recognize that this patient has a forme-fruste of lupus. A rheumatologist would have started the patient on Plaquenil and educated them with regard to their diagnosis, and the physical

Two of the most interesting, but also difficult to treat diseases, a physician may encounter include pulmonary renal syndrome, presenting with alveolar hemorrhage, and glomerular nephritis with ANA, DNA, successfully treated with cyclophosphamide [47]. Another rare, but not uncommon complication of lupus, would be TTP with or without the ADAMTS13 gene and ocular inflammation and orbital pseudotumor. Consider the case of a patient who presented with true renal failure, visual hallucinations and movement disorder. At that point the patient was treated with IV Cytoxan and pulse steroids. Therefore, the patient did not have fever; however the patient was anemic and had schistocytes with an elevated reticulocyte count. Thus, the patient did not fulfill all of the criteria for TTP; therefore, a clinical diagnosis was made of the same. The patient responded almost immediately to with all features of the disease disappearing with plasma exchange. This is a wonderful case to recall, when a hematologist says to a patient, "It cannot be TTP because there is no fever", apparently, this hematologist has lost sight of the fact that the high dose steroids likely blunted the fever. They may argue that there are not enough schistocytes [48] to fulfill the bacteria, however when schistocytes should not exist, and anemia cannot be explained, it can only be rationalized that the use of cyclophosphamides and high dose steroids lowered the schistocytes [49, 50]. This is a fantastic example of

why research criteria alone, should never be used for diagnostic purposes.

It is very important to understand the mechanism of action for each disease feature, as it will impact a patient's treatment. For the purpose of example, thrombocytopenia will be seen in Sjogren's syndrome and hypersplenism, while in lupus platelet antibodies, both conditions can be present with dry eyes and dry mouth. A salivary gland biopsy may not differentiate, as a positive lymphocyte score of 50

an overlap diagnosis, or based on the mechanism of thrombocytopenia, it may also sway the diagnosis. Pneumonitis, while common in lupus, is seen in other autoimmune diseases, including sarcoidosis. All conditions mentioned may have a positive rheumatoid factor or positive ANA. Even CCP antibodies can be seen in autoim-

, may presumably be seen in either condition. This may lead to

or orthopedic surgeon or any other practitioner involved in a patient's care.

with a Different View", lupus should be considered in every disease.

granted based on the positive ANA or research criteria, as those patients may well have myositis or scleroderma. As mentioned in Part 2 of this chapter, "A Rheum with a Different View", lupus should be considered in every disease.
