Lupus, which means wolf in Latin, is as strange, unpredictable, and mysterious as its name. It is not a name for one single disease, but by lupus, it could mean four diseases that are systemic lupus, cutaneous lupus, drug-induced lupus and neonatal lupus. Out of them, systemic lupus is the most common (more than 70% of cases), and the involvement of multiple organs is characteristic of it. Lots have been researched, written and yet very little is known and understood about the causes of this mysterious ailment. Like many autoimmune disorders, its numbers are rising in developed nations, but again no one can say for sure the reason behind it.
Symptoms of lupus can also vary greatly from person to person. Some of the symptoms like skin rashes and joint aches are common in lupus, but again those symptoms are common in many other disorders. In early stages of lupus fever, fatigue, and weight loss are found in the majority of cases. Other common symptoms are a pain in smaller joints along with fatigue and muscular aches. Perhaps the only symptom that is very characteristic of this disease is a malar rash, which is redness of the face that resembles a butterfly.
It is not rare for lupus to have atypical manifestations. As these symptoms are not very characteristic of lupus, it is not uncommon that the diagnosis of lupus is missed even by the best of medical professionals.
Lesser known symptoms of lupus
Some people may present painful or non-painful chronic oral ulcers. They may be present even in the nasal cavity, upper palate, and in rare cases a person may also have ulcers on genitals. There is little relationship between the ulcers and disease activation. Some people may also have impaired taste, poor dental health. In absence of other typical symptoms of lupus, it may be really confusing and difficult to find the true reason of these ulcers. Further, ulcers related to lupus are difficult to treat(1).
Non-scarring patchy alopecia may be presented in many cases of lupus and may be confused with alopecia areata (AA). But unlike AA in lupus, it is less common to have complete hair loss in a patch, further in lupus the patches may have more varied shapes.One may also see other changes in scalp like red dots, angiotelectasia. Broken hairs, thinning of hairs are also quite common in lupus. Hair loss associated with lupus is reversible. Unlike AA relapse of alopecia is less common in lupus(2).
Urticarial Vasculitis and other atypical skin rashes
Some people may have urticarial like eruption in lupus, but unlike urticarial rashes, they are usually painful and non-pruritic. It is a type 3 hypersensitivity reaction occurring due to deposition of immune complexes in small blood vessels of the skin. People with urticarial lupus would generally have other symptoms like fever, malaise, and fatigue.
Livedo Reticularis is another uncommon eruption in lupus, it is lace-like patterns of discoloration more often found on the skin of the lower limbs.
Bullous systemic lupus erythematosus (BSLE) is characterized by blisters and vesicles, that form due to autoantibodies against sub-epidermis. Blisters usually occur on the exposed part of the skin (1).
Severe muscular pains and inflammation
Myositis, severe inflammation of muscles is a rare complication of lupus, it is characterized by severe pain in muscles, weakness, tenderness. Lupus myositis affects the multiple muscles. In lupus, myositis is commonly associated with alopecia, mouth ulcers, joint pains, and disease of lungs. Myositis is aserious complication of lupus and it increases the risk of early death(3).
It is well known that people suffering from lupus are much more prone to psycho-social problems like anxiety and depression, some of the people may even develop psychosis and seizures, though very little is understood about the underlying mechanism.
Gastrointestinal problems in lupus
Though problems of gastrointestinal system are common in lupus, they can be difficult to diagnose due to the diverse nature of their presentation. Some people may have severe abdominal pain and chronic diarrhea, while others may present with symptoms of pseudo intestinal obstruction.
Gastrointestinal symptoms in lupus are caused due to mesenteric vasculitis (inflammation of blood vessels of intestine), thus there would be pain, malnutrition and weight loss.
Intestinal pseudo-obstruction is well-recognized, serious complication of lupus. It is caused due to dysfunction of enteric nerves and muscles. There can be severe abdominal distention, the absence of peristalsis, without identifiable organic obstruction. Early diagnosis is very important in such condition, as it is characterized by a high mortality rate(4).
Pancreatitis is a rare but life-threatening complication of lupus. Though the pathological mechanism is not known, vascular damage in the pancreas is considered to be behind it. Occlusion of arteries and arterioles leads to local necrosis (death of cells)(4).
Inflammatory bowel disease and celiac disease often coexist with lupus.
Lung problems is are quite common in lupus. One of the most common presentation is pleuritis, characterized by pain while inhaling or exhaling and accumulation of fluid in lung cavity. Most problems of lungs are caused due to inflammation of blood vessels. Timely diagnosis is important, else there is a high risk of infectious complications(5).
Though all the three layers (outer-pericardia, middle muscular-myocardia, inner-endocardia) of the heart may get diseased in lupus, inflammation of outer covering is most commonly diagnosed in the clinical settings. It is called pericarditis. Pericarditis is characterized by acute chest pain and can be treated.
Disease of the inner layer that is endocardia may often lead to disease of cardiac valves.
People suffering from lupus are also more prone to various types and degrees of cardiac arrhythmias.
As lupus affects the blood vessels, it means that a person suffering from lupus is at much higher risk of developing cardiovascular disorders, which may manifest itself in form of angina pectoralis, or myocardial infarction(6).
Thus we see that lupus has a very diverse presentation. In most cases, these lesser-known manifestations would be found along with more classical signs of lupus-like malar rash and joint pain. But it is not rare to find these signs as the primary or initial symptom of lupus. When such rare kind of manifestations are present as a primary or main symptom of the disease it may be difficult to diagnose even by the best of the specialist in the field of lupus.
It is important to be aware of these lesser-known manifestations, as some of them are associated with high level of mortality if they remain undiagnosed for long because the treatment of lupus is very different from many other disorders with similar presentations.
In complications of lupus, immunosuppression with corticosteroid or other drugs would be the treatment of choice. While such drugs are contraindicated in most other disorders with similar manifestations.
Uva L, Miguel D, Pinheiro C, Freitas JP, Pedro O, Marques Gomes M, et al. Cutaneous Manifestations of Systemic Lupus Erythematosus. Autoimmune Dis. 2012;2012:15.
Ye Y, Zhao Y, Gong Y, Zhang X, Caulloo S, Zhang B, et al. Non-scarring patchy alopecia in patients with systemic lupus erythematosus differs from that of alopecia areata. Lupus. 2013 Dec;22(14):1439–45.
Tian X-P, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: Insight into pathogenesis, diagnosis and treatment. World J Gastroenterol WJG. 2010 Jun 28;16(24):2971–7.
Kamen DL, Strange C. Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med. 2010 Sep;31(3):479–88.
Ansari A, Larson PH. Heart Disease in Systemic Lupus Erythematosus: Diagnosis and Management. Tex Heart Inst J. 1985 Mar;12(1):9–21.